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Solitary fibrous tumor of the orbit: a clinicopathologic study of six cases with review of the literature
SURVEY OF OPHTHALMOLOGY
VOLUME 48 • NUMBER 5 • SEPTEMBER–OCTOBER 2003
CLINICAL PATHOLOGIC REVIEW MILTON BONIUK AND STEFAN SEREGARD, EDITORS
Solitary Fibrous Tumor of the Orbit: A Clinicopathologic Study of Six Cases With Review of the Literature Subramanian Krishnakumar, MD,1 Nirmala Subramanian, MS, Mch,1 E. Ravindra Mohan, MD, FRCS Ed,1 Lakshmi Mahesh, DNB,1 Jyotirmay Biswas, MS,1 and Narsing A. Rao, MD2 1
Medical and Vision Research Foundations, Sankara Nethralaya, Chennai, India; and 2Doheny Eye Institute, Keck School Of Medicine, University Of Southern California, Los Angeles, California, USA
Abstract. Solitary fibrous tumor of the orbit is a rare spindle cell neoplasm. There are 42 cases of solitary fibrous tumor of the orbit available in the literature. We present six more cases of orbital solitary fibrous tumors, which presented to our institute between 1999 and 2001. We highlight the need for clinical recognition of these tumors as a distinct entity and inclusion of this tumor in the etiological differential diagnosis of well-circumscribed orbital lesions presenting as unilateral proptosis in both children and in adults. The diagnosis may be suspected based on radiological features supported by histopathologic and immunohistochemical study. The strong CD34 immunoreactivity of this tumor supports its diagnosis. Complete surgical resection is the most important prognostic factor of this tumor. (Surv Ophthalmol 48:544–554, 2003. 쑖 2003 Elsevier Inc. All rights reserved.) Key words. CD34
•
orbit
•
solitary fibrous tumor
Solitary fibrous tumor (SFT) is the name given to a distinct and rare spindle cell neoplasm, which most frequently arises from the pleura.4,11,16,17,35 Although initially described as a pleural mesothelial tumor,48,61 this tumor has also been reported at extra pleural sites.23 Solitary fibrous tumors arising in the orbit are relatively rare. However, in the last few years there have been several case reports and short series of orbital SFT (Table 1). A review of literature shows 42 reported cases of SFT occurring in the soft tissues of orbit.1,6,8,12,15,18–22,27,29–31,34,36–39,42–45,53,56,59 We report herein six cases of orbital SFT, and discuss the clinical presentation, previous diagnoses,
•
spindle cell tumor
radiological characteristics, histopathologic and immunohistochemical features, treatment, and prognosis of this not so uncommon tumor with a review of the literature.
Case Studies CASE 1
A 30-year-old man was seen in January 1999 with a gradual proptosis of the right eye of over 1 year duration. Ophthalmic examination revealed a visual acuity of 20/20 in both eyes. On the right eye there was an upward and outward proptosis of more than 15 mm 544
쑖 2003 by Elsevier Inc. All rights reserved.
0039-6257/03/$–see front matter doi:10.1016/S0039-6257(03)00087-0
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SOLITARY FIBROUS TUMOR OF THE ORBIT TABLE 1
List of Solitary Fibrous Tumors of the Orbit in the World Literature Author (year) Westra
56
(1994)
Dorfman15 (1994)
Fukunaga20 (1995) Lucas37 (1995) Sciot44 (1996) Scott45 (1996) Ruska43 (1996) De Backer12 (1996) Ramdial42 (1996) Mc Elvanney39 (1996) Suster50 (1995) Cho8 (1998) Ing31 (1998) Lanuza36 (1998) Zamenik61 (1998) De Sant Aubain13 (1999) Festa19 (1999) Kim34 (1999) Woo59 (1999) Alexandrakis1 (2000) Havlik27 (2000) Fenton18 (2001) Carrera6 (2001) Gigantelli21 (2001)
Takamura H et al53 (2001) Lucci LM et al38 (2001) Giuffre I et al22 (2001) Hayashi S et al29 (2001) Krishnakumar et al (2001)
Age/sex
No
60/Male 63/Female 44/Male 65/Female 69/Female
2
NG
Orbit
NG (not given)
3
NG
Orbit
21/Male 40/Female 20/Female 19/Female 23/Male 76/Male 48/Female 75/Female 43/Female 23/Female NG NG 62/Male
1 3
Orbit Orbit
1 1 1 1 1 1 1 1 1
4 months 1 month NG 9 months NG NG 2 year 8 months 5 years 2 years NG NG NG
35/Female 46/Female NG 61/Male 24/Male 20/Female 40/Female 23/Male 34/Female 14/Female
1 1 1 1 3
NG NG NG 3 year NG
2
NG
Orbit Orbit Orbit Orbit Lac gland Lac gland Orbit Lacrimal sac
18 months 12 years 10 years (2 recurrences one at 4th yr and other at 6th yr) 22 months 18 months 23 months 6 years 9 months 1 year NG NG NG 2 years NG NG Post enucleation followed by exenteration 20 years later 2 years NG NG NG 21 months 5 months 22 months NG
1
5 months
Orbit
38/Male NG NG 24/Female 69/Male 72/Male 45/Female 31/Male 18/Female 62/Male 38/Female 10/Male 40/Female 54/Male 30/Male
1 1 1
12 months NG NG NG
Orbit Orbit Orbit
42/Female 26/Male 30/Male 44/Male 9/Male
Duration
Location
Orbit Lac gland Orbit Orbit Orbit Lacrimal sac Orbit Lacrimal sac Orbit
7 Orbit
1 1 1 1
3 60 months 48 months 7 months 36 months
Orbit Orbit Orbit Orbit & extraorbit
6
84 months 36 months 12 months 6 months 6 months
Orbit
Follow-up
Recurrence after 4 months NG NG 8-year follow-up 30 months 12 months 12 months 6 months 6 months 18 months 12 months 15 months NS Not given Recurrence twice 14 months (recurrence at 3 months) 18 months 21 months Lost to follow-up 10 months 3 months
NG ⫽ not given.
with global limitation of movements. An ill-defined non-tender mass was felt along the inferomedial orbit with poorly defined margins. The anterior segment and fundus examination was normal in both eyes.
Computed tomography (CT) scan of the orbit study revealed a well-circumscribed extraconal mass lesion measuring 36 mm × 23 mm × 32 mm in the nasal orbit with well corticated erosion of the medial
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Fig. 1. Computerized tomography of the orbit showing a well-circumscribed peripherally enhancing lesion with cystic areas (case 2).
orbital wall. An ultrasound study showed low to moderate internal reflectivity with fairly homogenous structure without evidence of compressibility or vascularity. No cystic areas were seen. An initial fine needle biopsy showed clusters of small to mediumsized spindle cells arranged in a storiform pattern suggestive of a spindle cell tumor. A medial orbitotomy was performed and the tissue was submitted for histopathological study. The patient was lost to follow-up.
KRISHNAKUMAR ET AL
Fig. 2. Ultrasound of the orbit showing a well-defined lesion in the supero-temporal quadrant with multiple cystic spaces without infiltration of the lateral or superior rectus (case 2).
CASE 3
A 42-year-old woman was seen in October 1999 with a proptosis of the right eye of 7 years duration. She had undergone an incisional biopsy earlier, and labeled to have fibrous histiocytoma or a low-grade spindle cell sarcoma of the orbit. Ophthalmic examination revealed a visual acuity of 20/80 in the right eye was. There was a 12-mm proptosis with a superior displacement of the globe, fullness of the superior orbit, and a firm multinodular mobile tumor along the inferolateral orbit (Fig. 3). There was global
CASE 2
A 26-year-old man was seen in June 1999 with complaints of swelling of the outer part of left upper lid of 3 years duration. He had undergone an open biopsy elsewhere 3 years earlier and labeled to have inflammatory pseudotumor of the orbit. Ophthalmic examination showed his visual acuity was 20/20 in both eyes. There was mild left upper eyelid ptosis with a prominent palpebral lobe of the lacrimal gland. There was a down and in proptosis of 4 mm with global restriction of ocular movements. The anterior segment and fundus examination was normal. The right eye and systemic examinations were normal. CT of the orbit revealed a well-circumscribed peripherally enhancing lesion with central cystic areas significantly increased in size from the previous studies (Fig. 1). An ultrasonogram of the orbit revealed a well-defined lesion in the supero-temporal quadrant with multiple cystic spaces without infiltration of the lateral or superior rectus (Fig. 2). The patient underwent a repeat lateral orbitotomy. At 2-year follow-up, the patient remained asymptomatic without recurrence of the tumor.
Fig. 3. External photograph showing proptosis with a superior displacement of the globe (case 3).
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SOLITARY FIBROUS TUMOR OF THE ORBIT
Fig. 4. Computerized tomography of the orbit revealed an intensely enhancing well-circumscribed large orbital mass lesion measuring 5 × 4.8 × 4.5 cm (case 3).
restriction of all ocular movements with exposure keratopathy. Fundus revealed venous dilatation, tortuosity, and pallor of the optic nerve head. Examination of the left eye was normal. Regional and systemic examinations were normal. CT of the orbit revealed an intensely enhancing well-circumscribed large orbital mass lesion measuring 5 cm × 4.8 cm × 4.5 cm causing proptosis with stretching of the optic nerve (Fig. 4). An ultrasound study revealed a multilobulated mass with variable echolucency surrounding the optic nerve without indentation of the globe. The lateral orbital rim appeared thin without erosion. The patient underwent an inferolateral orbitotomy. At 18-month follow-up, visual acuity was 20/20 with full extraocular movements and no afferent defect. Repeat imaging ruled out residual tumor or recurrence of the tumor. CASE 4
A 30-year-old man was seen in February 2000 with a 3-year history of insidious prominence and decreased vision. A CT scan of the brain and orbit revealed an intensely enhancing mass lesion of the inferomedial orbit for which he had an incisional biopsy elsewhere and diagnosed as either a neurofibroma or schwannoma. Ophthalmic examination revealed perception of hand movements on the left eye. There was a relative afferent pupillary defect with gross supero-temporal displacement of the globe with a firm lobulated mass felt along the inferonasal orbit and conjunctiva, with scarring of the cornea (Fig. 5). Fundus examination revealed disk edema with venous dilatation and tortuosity on the left side. Right eye was normal. With a diagnosis of a benign soft tissue tumor, he underwent an anterior orbitotomy. Postoperatively, the patient continued to have severe globe
Fig. 5. External photograph showing a supero-temporal displacement of the globe with a firm lobulated mass felt along the infero-nasal orbit and conjunctiva, with scarring of the cornea (case 4).
malposition and a recurrence of firm lesions along the inferior orbit 3 months later. Vision was stable. An exploration with biopsy revealed findings consistent with the previous pathology. CASE 5
A 44-year-old man was seen in March 2000 with a 6-month history of gradual progressive visual loss with displacement of the right eye. Ophthalmic examination revealed a best corrected visual acuity of 20/200 in the right and 20/80 in the left. Examination of the left eye was unremarkable while on the right there was a gross superior and lateral displacement of the eye. There was a dense relative afferent pupillary defect. The inferior cornea was scarred with vascularization. A firm to hard mass was felt along the infero-medial orbit extending superiorly and laterally the posterior aspect of which could not be made out. Fundus examination revealed a hyperemic edematous disk with choroidal folds. CT scan of the orbits revealed a brilliantly enhancing well-circumscribed lesion along the medial orbit displacing the medial rectus and optic nerve with indentation of the globe and remodeling of the adjacent bone without erosion. An ultrasound study showed the lesion to have a homogenous internal reflectivity suggestive of compact tissues with indentation of the globe. No cystic areas were seen. He underwent medial orbitotomy. Postoperatively, there was an improvement of vision to 20/20 with resolution of the disk edema and choroidal folds. There was no recurrence at the 10-month follow-up visit.
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CASE 6
A 9-year-old boy presented with swelling of the left upper and lower eyelids that had been intermittent during the previous 6 months. There was no history of trauma, surgery, pain, redness, or systemic illnesses. He was otherwise healthy. His best corrected visual acuity was 20/20 in both eyes. External examination revealed increased periorbital pigmentation worse in the left eye. The orbital rims were intact. Ocular motility was within normal limits. Exophthalmometry readings were 13 and 15 in the right and left eye respectively with a base reading of 84. There was 2–3 mm of downward displacement of the left globe. There was no relative afferent papillary defect. Anterior segment and fundus examinations were unremarkable. MRI revealed a well-circumscribed brilliantly enhancing mass along the medial extra conal orbit not infiltrating the medial rectus or the bone. The rest of the orbit and the intracranial space were within normal limits. With a provisional diagnosis of soft tissue tumor he underwent left medial orbitotomy. The child is on 3-month follow-up and there is no recurrence of the tumor.
Imaging Studies ULTRASOUND
Four of the patients (cases 2,3,4,5) had undergone ultrasound evaluation of the orbit. All of them had well-circumscribed masses without infiltration of the optic nerve or rectus muscle. Two (cases 2 and 3) had intralesional cystic spaces. The other two had homogenous echolucency (cases 4 and 5), with one showing indentation of the globe (case 5). Case 6 did not undergo ultrasound study.
Fig. 6. Photomicrograph showing the gross tumor in case 3 showing a well-circumscribed pseudo encapsulated masses ranging from 2.1–5.7 cm in the greatest dimension (case 3).
PATHOLOGIC EXAMINATION
Gross examination of the tumor in all the cases showed well-circumscribed pseudo encapsulated masses, ranging from 2.1–5.7 cm in their greatest dimension (Fig. 6). Cut section of the tumor showed a tan and fleshy appearance with areas of hemorrhage in the tumor. Microscopically in all six cases the tumor was composed of fibrocollagenous tissue and spindle cells arranged in a fascicular pattern around thick collagen fibers, with occasional myxoid stroma (Fig. 7). In certain areas a keloid-like pattern surrounded with fibroblasts was seen (Fig 8). Numerous vascular channels with a pericytomatous arrangement were seen. Two of them (cases 2 and 3) had cystic component (Fig. 9). A possibility of solitary fibrous tumor
COMPUTERIZED TOMOGRAPHY
CT scan imaging of the orbits showed brilliant enhancement in all patients. Whereas in four patients (cases 1, 2, 4, and 5) there was diffuse intralesional enhancement, in one (case 3) there was peripheral ring of enhancement only. The feature of intense enhancement was so strong that in two patients (cases 3 and 4) a preoperative diagnosis of solitary fibrous tumor was suspected in two patients. The location of the tumor was in the inferomedial (case 1), superior and inferolateral (case 2), superotemporal (case 3), nasal (case 4), and medial (cases 5 and 6) portion of the orbit, respectively. MAGNETIC RESONANCE IMAGING
MRI in one (case 6) showed a brilliantly enhancing mass along the medial extraconal orbit.
Fig. 7. Photomicrograph showing spindle cells arranged in a fascicular pattern around thick collagen fibers, with occasional myxoid stroma (hematoxylin and eosin stain × 200).
SOLITARY FIBROUS TUMOR OF THE ORBIT
549
Fig. 8. Photomicrograph showing the thickened keloid like collagen fibers surrounded by the spindle cells (arrow) (hematoxylin and eosin stain × 200).
Fig. 10. Photomicrograph showing positive staining of the cytoplasm of the tumor cells with CD34 (immunoperoxidase CD 34 × 200).
of the orbit was considered based on these characteristics and subjected to immunohistochemical marker CD 34 study.
of orbital SFT occurring in the pediatric age group, including our case.1,38 Thus, SFT must also be included in the differential diagnosis of pediatric orbital masses. At presentation all the patients in our series had a well-circumscribed mass, the anterior surface of which had a smooth contour. The clinical features of the patients in our series are summarized in Table 3. All the patients presented with unilateral painless proptosis of insidious onset over a mean duration of 34.8 months (6–84 months). At presentation visual acuity in three patients (cases 1,2, and 6) was normal. In the others it was 6/24 (20/80), 6/60 (20/ 200) and perception of hand motions (cases 3,4, and 5). In the cases 1, 3, 4, and 5, visual acuity improved to 6/6 (20/20) postoperatively. Solitary fibrous tumor typically arises in the pleura and has been called “localized mesothelioma” because it was once thought to be of mesothelial cell origin. The neoplasm was originally described by Klemperer and Rabin in 1931.35 They characterized localized pleural tumors as large, slowly growing, pedunculated masses covered by visceral pleura and hypothesized that their origin proposed a mesothelial origin for the localized pleural tumor. However, their histogenesis was controversial and many reports claim that they are of submesothelial stromal cell or fibroblast-like mesenchymal cell origin, supported by evidence from histologic, immunohistochemical, ultrastructural, and tissue culture studies. Recently, SFT has been described in extrapleural sites, including lung, mediatinum,57 peritoneum,60 upper respiratory tract,33,58 thyroid, liver,3 and soft tissue.7,32 The first SFT presenting in the orbit was independently reported in 1994 by Dorfman et al15 and Westra et al,56 respectively. Stout and Murray first described the microscopic appearance of SFT.48 Solitary fibrous tumor shows a patternless, hypercellular and
IMMUNOHISTOCHEMICAL STUDY
The paraffin-embedded sections were subjected to standard immunohistochemical protocols using biotin-streptavidin-peroxidase detection (DAKO Corporation, Carpentaria, CA). Immunohistochemically the tumor cells were CD34 positive in the cytoplasm (Fig. 10) supporting a diagnosis of orbital SFT.
Discussion It is important to differentiate SFT occurring in the orbit from the other spindle cell tumors that mimic it (Table 2). Our series contained more men than women (M5:F1). The age ranged from 9 to 44 years, and the mean age was 34.4 years. Thus, SFT of the orbit occurs over a wide age range, including the pediatric age group. We have found three cases
Fig. 9. Photomicrograph showing the cystic component tumor. The cystic space contains pale eosinophilic fluid (arrow) (hematoxylin and eosin stain × 200) (case 2).
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TABLE 2
Important Differential Diagnosis of Spindle Cell Tumors of the Orbit Tumor
Histopathological Features
Monophasic synovial sarcoma
Densely packed fascicles, fibrosarcomatous, or hemangiopericytomatous pattern, oval to round nuclei
Fibrosarcoma
Herring bone pattern, slender spindle nuclei, interwoven collagen between cells Abundant eosinophilic cytoplasm, cigar-shaped nuclei
Smooth muscle tumors Malignant Peripheral nerve sheath tumor Solitary fibrous tumor Giant cell angiofibroma Giant cell fibroblastoma Spindle cell squamous cell carcinoma Cellular fibrous histiocytoma Desmoplastic amelanotic melanoma
Sweeping fascicles, wary nuclei, occasional heterologous elements Variety of patterns, deposition of collagen between cells Spindle cells arranged in a fascicular pattern, pseudovascular spaces, giant cells Spindle cells, pseudovascular spaces, giant cells, pleomorphism Spindle cells in fascicular pattern, mitosis Spindle cells, fascicular pattern, no mitosis Spindle cells, mitosis
Immunohistochemical Features Keratin⫹, focal EMA⫹, focal bcl 2⫹, CD34⫺, Desmin⫺, Vimentin ⫹ (spindle cells) Vimentin_⫺ ve (epithelioid cells) S 100⫹ rare focal Keratin ⫺, EMA⫺, bcl 2⫹, CD34⫹ Keratin ⫹ (rare focal), Vimentin ⫹ve, Desmin ⫹, SMA ⫹ bcl 2⫺, CD34 ⫺ve Keratin ⫹ rare, Vimentin ⫹ve, Focal EMA⫹ rare, Focal S100 ⫹ rare, bcl 2⫹ rare, CD34 ⫺ve Keratin⫺, EMA⫺, bcl 2⫹, Vimentin ⫹, CD34 ⫹ Vimentin ⫹, CD 34 ⫹, S100 ⫺ve, Keratin⫺, EMA ⫺ Vimentin ⫹, CD 34 ⫹, F VIII ⫺ve, S100 ⫺, Keratin ⫺, EMA ⫺ Cytokeratin ⫹, Vimentin ⫺ve, S 100 ⫺ve, Desmin ⫺ve, CD34 ⫺ve, EMA ⫺ve Keratin ⫺ve, Vimentin ⫹ve, S 100 ⫺ve, Desmin ⫺ve, CD34 ⫹, EMA ⫺ve Keratin ⫺ve, Vimentin ⫹ve, S100 ⫹ve, Desmin ⫺ve, CD34 ⫺ve, EMA ⫺ve
Cytogenetic/Molecular Abnormalities t(x:18)(p11.2:q11.2)
⫹8,⫹11,⫹17,⫹20 (for infantile fibrosarcoma) Del 1p(in ⬎75%) of leiomyosarcoma Complex chromosomal aberrations t(9:22) (q31:p13) Not known
Not known Not known Not known Not known
EMA ⫽ epithelial membrane antigen; SMA ⫽ smooth muscle actin; Del ⫽ deletion; p ⫽ chromosome long arm; q ⫽ chromosome short arm; t ⫽ translocation.
hypocellular region of spindle cells, against a background of thick collagen bands. Focal hemangiopericytoma like staghorn, fibrous histiocytoma like storiform pattern, synovial sarcomatous and neurallike palisading regional architecture have been described with SFT.2 This multiplicity of histologic patterns and an absence of single intralesional architecture characterize SFT. This variability could lead to mistaken diagnosis if limited tissue allows a single morphology to dominate the histopathologic specimen and immunohistochemical studies are not performed. Cases 2, 3, and 4 had previously undergone incisional biopsy elsewhere and were labeled as inflammatory tumor (case 2), fibrous histiocytoma or spindle cell sarcoma (case 3), and neurofibroma, neurilemomma (case 4). In each of these cases, intraoperatively there was no evidence of tumor invasion into adjacent soft tissue or bone enabling
relatively easy delivery except in case 4, in which the plane of dissection may have been complicated by the previous incisional biopsy. In this patient repeat orbitotomy was necessitated due to persistent growth or recurrence of the tumor. The light microscopic differential diagnosis of SFT includes giant cell angiofibroma, fibrous histiocytoma, hemangiopericytoma, peripheral nerve sheath tumor, fibromyxoid sarcoma, monophasic synovial sarcoma, and other rare mesenchymal tumors.13,23,25 Solitary fibrous tumor is immunoreactive for mesenchymal markers such as vimentin but negative for desmin, epithelial marker (cytokeratin), vascular marker (factor VIII related antigen), neural markers (S-100 protein), and muscle specific actin and smooth muscle actin.17,20,30,40 Immunoreactivity with the marker CD34 is present in 79–100% of cases.41,50,55 Westra and associates also found that 9/ 9 neurofibromas showed immunopositivity for CD34,
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SOLITARY FIBROUS TUMOR OF THE ORBIT TABLE 3
Summary of the Clinical Findings in Our Cases No./Age/Sex Duration of the Mass
Earlier Diagnosis
Site
Follow-up and Recurrence
Ultrasound
Case 1 30 years Male 12 months Case 2 26 years Male 36 months
Spindle cell tumor (FNAB)
Nasal
Lost to follow-up
Inflammatory pseudotumor
Superolateral
21 months
Case 3 42 years Female 84 months
Fibrous histiocytoma/ Spindle cell sarcoma (low grade) Neurofibroma/ Neurilemmoma
Superior and Inferolateral
18 months
Multilobulated mass, variable echolucency
Inferomedial
14 months with recurrence at 3 months
Not done
Intensely enhancing mass
None
Medial
10 months no recurrence
Homogeneous internal reflectivity, no cystic areas
Brilliantly enhancing well-circumscribed mass
None
Medial
3 months no recurrence
Not done
Not done
Case 4 30 years Male 36 months Case 5 44 years Male 6 months Case 6 9 years Male 6 months
suggesting that while sensitivity appears to be excellent for this marker, the potential diagnostic specificity is less than perfect.56 Uniform positivity for vimentin and CD34 supports a diagnosis of SFT. Although giant cell angiofibroma shares an identical immunostaining pattern, multinucleated giant cells are not usually seen in SFT. Recently a giant cell rich variant of SFT has been reported.28 Dei Tos et al described a series of seven cases of a morphologically distinct orbital tumor with rich vascularized and patternless proliferation of spindle cells and some pseudovascular spaces embedded in a variably collagenized stroma, some with myxoid deposit.14 The spindle cells and the multinucleated giant cells are intensely positive for vimentin and CD 34. GCA closely mimicks SFT and giant cell fibroblastoma. However, multinucleated giant cells and pseudovascular spaces are not features of SFT. Additionally, orbital SFT is thought to arise from more deeply situated and cause greater proptosis in contrast to GCA. Giant cell fibroblastoma mimicks GCA. Giant cell fibroblastoma is a childhood tumor and a rare mesenchymal tumor. It is composed of loosely arranged spinde cells with a biphasic pattern consisting of solid and angiectoid (pseudovascular) areas and moderate degree of nuclear pleomorphism.
Homogeneous mass, low to moderate internal reflectivity Well-defined lesion, multiple cystic spaces
Computerized Tomogram Well-circumscribed, extraconal mass Well-circumscribed, peripherally enhancing mass with central cystic areas Intensely enhancing well-circumscribed mass
This has a number of similarities to dermatofibrosarcoma protuberans. It is a juvenile analog of the latter. There is some degree of morphologic overlap between GCA and giant cell fibroblastoma. Giant cell fibroblastoma is also positive for CD34 (Table 4 lists the similarities and differences between GCA and giant cell fibroblastoma). Thus GCA and giant cell fibroblastoma must be also included in the differential diagnosis of orbital SFT. Solitary fibrous tumors, GCA, and giant cell fibroblastomas are positive for vimentin, CD34, and bcl-2.26,46,49 Thus these markers lack sufficient specificity to independently distinguish SFT from the other spindle cell tumors. Solitary fibrous tumor as an entity, however, is rarely diagnosed clinically. Radiologic characteristics being typical may lead to preoperative suspicion. In our series, four patients underwent ultrasound examination of the orbit and all of them had well-circumscribed masses without infiltration of optic nerve or rectus muscle. It was helpful in identifying the cystic component in case 2 and the other 3 had homogeneous echogenecity. CT scan may show moderate to intense enhancing well-circumscribed mass. Bony modeling or well-corticated erosion may be seen in long-standing lesions adjacent to the orbital wall. In all but one patient
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TABLE 4
Differences and Similarities Between Giant Cell Angiofibroma, Giant Cell Fibroblastoma, and Solitary Fibrous Tumor Features
Giant Cell Angiofibroma
Giant Cell Fibroblastoma
Age of presentation
Adults
Childhood
Light microscopy
Spindle cell tumor, vascular spaces
Spindle cell tumor, pseudovascular spaces
Vascular pattern
More prominent No
Much less conspicuos Yes
Yes Multinucleate giant cells CD 34⫹ve Vimentin ⫹ve, Factor VIII ⫺ve S 100 ⫺ve No
Yes Outpouchings from a single nucleus CD 34⫹ve Vimentin ⫹ve, Factor VIII ⫺ve S 100 ⫺ve Yes
Pleomorphism of the nucleus Giant cells Electron microscopy on giant cells Immunohistochemistry
Relationship to dermatofibrosarcoma
brilliant enhancement was seen, this finding was so characteristic that in two patients a diagnosis of SFT (cases 3 and 4) was suspected. Magnetic resonance imaging was done in only one of our cases. However, MRI of orbital SFT provides characteristic features. Solitary fibrous tumors demonstrate a T1 signal intensity that is isointense to gray matter with a homogeneous or heterogeneous enhancement with gadolinium.34 T2 intense signal intensity is predominantly hypo-intense to gray matter. SFT frequently demonstrate central foci that are profoundly hypo-intense on both T1 and T2 imaging.5 T2 weighted signal hypo-intensity and intralesional signal heterogeneity are distinguishing features of SFT.21 This low signal intensity on T2 weighted images may be more suggestive of a fibrous orbital lesion with high collagen content. These magnetic resonance imaging characteristics are similar to those described for non-orbital SFT. This may help guide the surgeon to proceed with complete excision of the mass rather than fine needle aspiration or excision biopsy. It is undisputed that this tumor is of mesenchymal origin, supported by the strong CD34 immunoreactivity.40,41 CD34 is a surface glycoprotein expressed by human hematopoietic progenitor cells on their surface.52 CD34 is positive in normal and neoplastic endothelial cells, which raises the possibility that the cells of the SFT may be differentiated towards the vascular endothelial cells.10 On the other hand, due to the lack of factor VIII expression, it is possible that the cells of the SFT are not endothelial in origin,
Solitary Fibrous Tumor Childhood and adults Spindle cell tumor, vascular spaces, cystic spaces Hemangiopericytoma like pattern Usually no No – CD 34⫹ve Vimentin ⫹ve, Factor VIII ⫺ve S 100 ⫺ve No
but rather undifferentiated mesenchymal cells that have the ability, possibly through stromal cell/matrix interaction, to induce vascular structures that may give rise to a pattern like hemangiopericytoma.24,50 Patchy actin positivity, focal intercellular junctions, and cytoplasmic dense bodies are suggestive of myofibroblastic/fibroblastic differentiation. Also, myofibroblastic tumors reported in the breast and inguinal lymph nodes also bear a striking morphologic similarity to SFT.51,54 Genetic analysis of SFT has also been reported. More recently a balanced translocation t (9; 22)(q31; p13), one distinct from Philadelphia chromosome, t (9; 22)(q34; q11), seen in certain hematologic malignancies, has been described.27 It is also different from the common translocation of myxoid chondrosarcoma, which is t (9;22) (q22;q12).47 Like other soft tissue tumors, SFT may have typical genetic aberrations that will be only known after more in depth studies are undertaken. Solitary fibrous tumor of the orbit does not have any systemic symptoms such as hypoglycemia, arthralgia, and pleural or peritoneal effusion, which are known to occur in cases of SFT occurring at pleural sites.2 Solitary fibrous tumor of the pleura have been responsible for patient death in 12% of cases, either because of late diagnosis or unresectable recurrence.4 However, there are no reports of orbital SFT leading to death. Although these tumors are always well-circumscribed, to facilitate total excision, occasional cases of recurrences have been reported. Recurrence was seen in four cases, including one of our cases in which recurrence was seen in 3 months.
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SOLITARY FIBROUS TUMOR OF THE ORBIT
Dorfman reported a 69-year-old patient who developed two recurrences 4 and 6 years postoperatively.15 Alexandrakis reported a 14-year-old girl who presented with proptosis of 5 months duration and developed recurrence 4 months postoperatively.1 Ing et al reported a patient who underwent an enucleation for neurofibroma and underwent exenteration 20 years later for an orbital recurrence when a diagnosis of SFT was made.31 Recurrences are due to incomplete excision. Solitary fibrous tumors generally behave in a benign manner and do not metastasize.6 Criteria for malignancy include the presence of more than four mitosis per high-power field (or the presence of abnormal mitotic figures), cellular pleomorphism, or tumor giant cells.17,25 There is only one reported case of malignant SFT of the orbit.6 Recurrent SFT can reveal more mitotic activity then the original tumor as reported by Dorfman and his colleagues,15 where the original tumor showed a mitotic activity of less than 2 per 10 high power field (HPF), while the recurrent tumor showed a mitotic activity of 5 to 6 per 10 HPF. Therefore, the most important prognostic factor is not the histologic appearance but the complete respectability of the tumor, whether it is located in the orbital or in the pleura.44
Conclusion In conclusion we present six cases of orbital SFT, which have pursued a benign course except in one (case 3), which had a recurrence within 3 months and the tumor did not show any increased mitotic activity. SFT of the orbit occurs in varied age groups, has a slightly male predominance, occurs in a younger age group, and it can also occur in the pediatric age group as in our series (case 6). Therefore, SFT should thus be considered in the differential diagnosis when a patient presents with a well-circumscribed tumor causing unilateral proptosis with intensely enhancing mass seen on the CT scan evaluation. Fine needle aspiration is also helpful in establishing a diagnosis, if the tissue sample is adequate and immunomarkers could be done on the fine needle aspiration material.9 Likewise when confronted with a spindle cell tumor of the orbit, the ophthalmic pathologist should also consider the possibility of SFT and judiciously use immunohistochemical markers to establish the diagnosis. Orbital SFT seems to be clinicopathologically very similar to pleural SFT. Both usually have an indolent behavior. In order to avoid recurrences a wide aggressive resection and prolonged follow up are favored. The value of adjuvant chemotherapy or irradiation is not known.
Method of Literature Search We searched the Medline database, using keywords such as solitary fibrous histiocytoma, orbit, histopathology, and various combinations of the above, for the years 1966–2001. We searched both English and foreign languages. There was one Slovak reference50 and its English abstract was used for the necessary information.
References 1. Alexandrakis G, Johnson TE: Recurrent orbital solitary fibrous tumor in a 14-year-old girl. Am J Ophthalmol 130:373– 6, 2000 2. Ali SZ, Hoon V, Hoda S, et al: Solitary fibrous tumor. A cytologic-histologic study with clinical, radiologic, and immunohistochemical correlations. Cancer 81:116–21, 1997 3. Barnoud R, Arvieux C, Pasquier D, et al: Solitary fibrous tumour of the liver with CD34 expression. Histopathology 28:551–4, 1996 4. Carrera M, Prat J, Quintana M: Malignant solitary fibrous tumour of the orbit: report of a case with 8 years follow-up. Eye 15:102–4, 2001 5. Briselli M, Mark EJ, Dickersin GR: Solitary fibrous tumors of the pleura: eight new cases and review of 360 cases in the literature. Cancer 47:2678–89, 1981 6. Cameron IL, Ord VA, Fullerton GD: Characterization of proton NMR relaxation times in normal and pathological tissues by correlation with other tissue parameters. Magn Reson Imaging 2:97–106, 1984 7. Chan JK: Solitary fibrous tumour—everywhere, and a diagnosis in vogue. Histopathology 31:568–76, 1997 8. Cho NH, Kie JH, Yang WI, Jung WH: Solitary fibrous tumour with an unusual adenofibromatous feature in the lacrimal gland. Histopathology 33:289–90, 1998 9. Clayton AC, Salomao DR, Keeney GL, Nascimento AG: Solitary fibrous tumor: a study of cytologic features of six cases diagnosed by fine-needle aspiration. Diagn Cytopathol 25:172–6, 2001 10. Cohen PR, Rapini RP, Farhood AI: Expression of the human hematopoietic progenitor cell antigen CD34 in vascular and spindle cell tumors. J Cutan Pathol 20:15–20, 1993 11. Dalton WT, Zolliker AS, McCaughey WT, et al: Localized primary tumors of the pleura: an analysis of 40 cases. Cancer 44:1465–75, 1979 12. DeBacker CM, Bodker F, Putterman AM, Beckmann E: Solitary fibrous tumor of the orbit. Am J Ophthalmol 121:447– 9, 1996 13. de Saint Aubain Somerhausen N, Rubin BP, Fletcher CD: Myxoid solitary fibrous tumor: a study of seven cases with emphasis on differential diagnosis. Mod Pathol 12:463–71, 1999 14. Dei Tos AP, Seregard S, Calonje E, et al: Giant cell angiofibroma. A distinctive orbital tumor in adults. Am J Surg Pathol 19:1286–93, 1995 15. Dorfman DM, To K, Dickersin GR, et al: Solitary fibrous tumor of the orbit. Am J Surg Pathol 18:281–7, 1994 16. el-Naggar AK, Ro JY, Ayala AG, et al: Localized fibrous tumor of the serosal cavities. Immunohistochemical, electronmicroscopic, and flow-cytometric DNA study. Am J Clin Pathol 92:561–5, 1989 17. England DM, Hochholzer L, McCarthy MJ: Localized benign and malignant fibrous tumors of the pleura. A clinicopathologic review of 223 cases. Am J Surg Pathol 13:640–58, 1989 18. Fenton S, Moriarty P, Kennedy S: Solitary fibrous tumour of the orbit. Eye 15:124–6, 2001 19. Festa S, Lee HJ, Langer P, Klein KM: Solitary fibrous tumor of the orbit. CT and pathologic correlation. Neuroradiology 41:52–4, 1994
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20. Fukunaga M, Ushigome S, Nomura K, Ishikawa E: Solitary fibrous tumor of the nasal cavity and orbit. Pathol Int 45:952– 7, 1995 21. Gigantelli JW, Kincaid MC, Soparkar CN, et al: Orbital solitary fibrous tumor: radiographic and histopathologic correlations. Ophthal Plast Reconstr Surg 17:207–14, 2001 22. Giuffre I, Faiola A, Bonanno E, Liccardo G: Solitary fibrous tumor of the orbit. Case report and review of the literature. Surg Neurol 56:242–6, 2001 23. Goodlad JR, Fletcher CDM: Solitary fibrous tumor arising at unusual sites: analysis of a series. Histopathology 19:5157– 57, 1989 24. Guillou L, Gebhard S, Coindre JM: Lipomatous hemangiopericytoma: a fat-containing variant of solitary fibrous tumor? Clinicopathologic, immunohistochemical, and ultrastructural analysis of a series in favor of a unifying concept. Hum Pathol 31:1108–15, 2000 25. Hanau CA, Miettinen M: Solitary fibrous tumor: histological and immunohistochemical spectrum of benign and malignant variants presenting at different sites. Hum Pathol 26:440–9, 1995 26. Hasegawa T, Matsuno Y, Shimoda T: Frequent expression of bcl-2 protein in solitary fibrous tumors. Jpn J Clin Oncol 28:86–91, 1998 27. Havlik DM, Farnath DA, Bocklage T: Solitary fibrous tumor of the orbit with a t(9;22)(q31;p13). Arch Pathol Lab Med 124:756–8, 2000 28. Hayashi N, Borodic G, Karesh JW, et al: Giant cell angiofibroma of the orbit and eyelid. Ophthalmology 106:1223– 9, 1999 29. Hayashi S, Kurihara H, Hirato J, Sasaki T: Solitary fibrous tumor of the orbit with extraorbital extension: case report. Neurosurgery 49:1241–5, 2001 30. Heathcote JG: Pathology update: solitary fibrous tumour of the orbit. Can J Ophthalmol 32:432–5, 1997 31. Ing EB, Kennerdell JS, Olson PR, et al: Solitary fibrous tumor of the orbit. Ophthal Plast Reconstr Surg 14:57–61, 1998 32. Khalifa MA, Montgomery EA, Azumi N, et al: Solitary fibrous tumors: a series of lesions, some in unusual sites. South Med J 90:793–9, 1997 33. Kim TA, Brunberg JA, Pearson JP, Ross DA: Solitary fibrous tumor of the paranasal sinuses: CT and MR appearance. AJNR Am J Neuroradiol 17:1767–72, 1996 34. Kim HY, Lee SY, Kang SJ, Kim HJ: Solitary fibrous tumor of the orbit: a poorly-recognized orbital lesion. Acta Ophthalmol Scand 77:704–8, 1999 35. Klemperer P, Rabin CB: Primary neoplasms of the pleura; a report of five cases. Arch Pathol 11:385–412, 1931 36. Lanuza A, Lazaro R, Salvador M, et al: Solitary fibrous tumour of the orbit. Report of a new case. Int Ophthalmol 22:265– 8, 1998 37. Lucas DR, Campbell RJ, Fletcher CDM, Garrity JA: Solitary fibrous tumor of the orbit. Int J Surg Pathol 2:193–8, 1995 38. Lucci LM, Anderson RL, Harrie RP, et al: Solitary fibrous tumor of the orbit in a child. Ophthal Plast Reconstr Surg 17:369–73, 2001 39. McElvanney AM, Noble JL, ODonovan DG, et al: Solitary fibrous tumour: an atypical presentation within the orbit. Eye 10:396–9, 1996 40. Nascimento AG: Solitary fibrous tumor; a ubiquitous neoplasm of mesenchymal differentiation. Adv Anat Pathol 3:388–95, 1996 41. Nickoloff BJ: The human progenitor cell antigen (CD34) is localized on endothelial cells, dermal dendritic cells, and perifollicular cells in formalin- fixed normal skin, and on
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42. 43. 44. 45. 46. 47.
48. 49.
50. 51.
52. 53. 54. 55. 56. 57. 58. 59. 60. 61.
proliferating endothelial cells and stromal spindle-shaped cells in Kaposis sarcoma. Arch Dermatol 127:523–9, 1991 Ramdial PK, Nadvi S: An unusual cause of proptosis: orbital solitary fibrous tumor: case report. Neurosurgery 38:1040– 3, 1996 Ruska KM, Westra WH: Pathologic quiz case 1. Solitary fibrous tumor (SFT) of the orbit. Arch Otolaryngol Head Neck Surg 122:1130, 1132, 1996 Sciot R, Goffin J, Fossion E, et al: Solitary fibrous tumour of the orbit. Histopathology 28:188–91, 1996 Scott IU, Tanenbaum M, Rubin D, Lores E: Solitary fibrous tumor of the lacrimal gland fossa. Ophthalmology 103:1613– 8, 1996 Sonobe H, Iwata J, Komatsu T, et al: A giant cell angiofibroma involving 6q. Cancer Genet Cytogenet 116:47–9, 2000 Stenman G, Andersson H, Mandahl N, et al: Translocation t(9;22)(q22;q12) is a primary cytogenetic abnormality in extraskeletal myxoid chondrosarcoma. Int J Cancer 62:398– 402, 1995 Stout AP, Murray MR: Localized pleural mesothelioma. Arch Pathol 34:951–64, 1942 Suster S, Fisher C, Moran CA: Expression of bcl-2 oncoprotein in benign and malignant spindle cell tumors of soft tissue, skin, serosal surfaces, and gastrointestinal tract. Am J Surg Pathol 22:863–72, 1998 Suster S, Nascimento AG, Miettinen M, et al: Solitary fibrous tumors of soft tissue. A clinicopathologic and immunohistochemical study of 12 cases. Am J Surg Pathol 19:1257–66, 1995 Suster S, Rosai J: Intranodal hemorrhagic spindle-cell tumor with amianthoid fibers. Report of six cases of a distinctive mesenchymal neoplasm of the inguinal region that simulates Kaposis sarcoma. Am J Surg Pathol 13:347–57, 1989 Sutherland DR, Watt SM, Dowden G, et al: Structural and partial amino acid sequence analysis of the human hemopoietic progenitor cell antigen CD34. Leukemia 2:793–803, 1988 Takamura H, Kanno M, Yamashita H, Maeda K: A case of orbital solitary fibrous tumor. Jpn J Ophthalmol 45:412– 9, 2001 Wargotz ES, Weiss SW, Norris HJ: Myofibroblastoma of the breast. Sixteen cases of a distinctive benign mesenchymal tumor. Am J Surg Pathol 11:493–502, 1987 Weiss SW: Histological typing of soft tissue tumors. in Sobin LH (ed) International histological classification of tumors. Berlin: Springer-Verlag; 1994 Westra WH, Gerald WL, Rosai J: Solitary fibrous tumor. Consistent CD34 immunoreactivity and occurrence in the orbit. Am J Surg Pathol 18:992–8, 1994 Witkin GB, Rosai J: Solitary fibrous tumor of the mediastinum. A report of 14 cases. Am J Surg Pathol 13:547–57, 1989 Witkin GB, Rosai J: Solitary fibrous tumor of the upper respiratory tract. A report of six cases. Am J Surg Pathol 15:842– 8, 1991 Woo KI, Suh YL, Kim YD: Solitary fibrous tumor of the lacrimal sac. Ophthal Plast Reconstr Surg 15:450–3, 1999 Young RH, Clement PB, McCaughey WTE: Solitary fibrous tumors (fibrous mesotheliomas) of the peritoneum. Arch Pathol Lab Med 114:493–5, 1999 Zamecnik M, Michal M: [Solitary fibrous tumor (fibrous mesothelioma). Report of 2 cases in an extraserous location]. Cesk Patol 34:58–62, 1998
The authors reported no proprietary or commercial interest in any product mentioned or concept discussed in this article. The authors were supported by a grant from the Vision Research Foundation, Chennai, India. The manuscript was presented in part at the 10th International Conference of Ocular Oncology Meeting, Netherlands, June 17–22, 2001.
VOLUME 48 • NUMBER 5 • SEPTEMBER–OCTOBER 2003
CLINICAL PATHOLOGIC REVIEW MILTON BONIUK AND STEFAN SEREGARD, EDITORS
Solitary Fibrous Tumor of the Orbit: A Clinicopathologic Study of Six Cases With Review of the Literature Subramanian Krishnakumar, MD,1 Nirmala Subramanian, MS, Mch,1 E. Ravindra Mohan, MD, FRCS Ed,1 Lakshmi Mahesh, DNB,1 Jyotirmay Biswas, MS,1 and Narsing A. Rao, MD2 1
Medical and Vision Research Foundations, Sankara Nethralaya, Chennai, India; and 2Doheny Eye Institute, Keck School Of Medicine, University Of Southern California, Los Angeles, California, USA
Abstract. Solitary fibrous tumor of the orbit is a rare spindle cell neoplasm. There are 42 cases of solitary fibrous tumor of the orbit available in the literature. We present six more cases of orbital solitary fibrous tumors, which presented to our institute between 1999 and 2001. We highlight the need for clinical recognition of these tumors as a distinct entity and inclusion of this tumor in the etiological differential diagnosis of well-circumscribed orbital lesions presenting as unilateral proptosis in both children and in adults. The diagnosis may be suspected based on radiological features supported by histopathologic and immunohistochemical study. The strong CD34 immunoreactivity of this tumor supports its diagnosis. Complete surgical resection is the most important prognostic factor of this tumor. (Surv Ophthalmol 48:544–554, 2003. 쑖 2003 Elsevier Inc. All rights reserved.) Key words. CD34
•
orbit
•
solitary fibrous tumor
Solitary fibrous tumor (SFT) is the name given to a distinct and rare spindle cell neoplasm, which most frequently arises from the pleura.4,11,16,17,35 Although initially described as a pleural mesothelial tumor,48,61 this tumor has also been reported at extra pleural sites.23 Solitary fibrous tumors arising in the orbit are relatively rare. However, in the last few years there have been several case reports and short series of orbital SFT (Table 1). A review of literature shows 42 reported cases of SFT occurring in the soft tissues of orbit.1,6,8,12,15,18–22,27,29–31,34,36–39,42–45,53,56,59 We report herein six cases of orbital SFT, and discuss the clinical presentation, previous diagnoses,
•
spindle cell tumor
radiological characteristics, histopathologic and immunohistochemical features, treatment, and prognosis of this not so uncommon tumor with a review of the literature.
Case Studies CASE 1
A 30-year-old man was seen in January 1999 with a gradual proptosis of the right eye of over 1 year duration. Ophthalmic examination revealed a visual acuity of 20/20 in both eyes. On the right eye there was an upward and outward proptosis of more than 15 mm 544
쑖 2003 by Elsevier Inc. All rights reserved.
0039-6257/03/$–see front matter doi:10.1016/S0039-6257(03)00087-0
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SOLITARY FIBROUS TUMOR OF THE ORBIT TABLE 1
List of Solitary Fibrous Tumors of the Orbit in the World Literature Author (year) Westra
56
(1994)
Dorfman15 (1994)
Fukunaga20 (1995) Lucas37 (1995) Sciot44 (1996) Scott45 (1996) Ruska43 (1996) De Backer12 (1996) Ramdial42 (1996) Mc Elvanney39 (1996) Suster50 (1995) Cho8 (1998) Ing31 (1998) Lanuza36 (1998) Zamenik61 (1998) De Sant Aubain13 (1999) Festa19 (1999) Kim34 (1999) Woo59 (1999) Alexandrakis1 (2000) Havlik27 (2000) Fenton18 (2001) Carrera6 (2001) Gigantelli21 (2001)
Takamura H et al53 (2001) Lucci LM et al38 (2001) Giuffre I et al22 (2001) Hayashi S et al29 (2001) Krishnakumar et al (2001)
Age/sex
No
60/Male 63/Female 44/Male 65/Female 69/Female
2
NG
Orbit
NG (not given)
3
NG
Orbit
21/Male 40/Female 20/Female 19/Female 23/Male 76/Male 48/Female 75/Female 43/Female 23/Female NG NG 62/Male
1 3
Orbit Orbit
1 1 1 1 1 1 1 1 1
4 months 1 month NG 9 months NG NG 2 year 8 months 5 years 2 years NG NG NG
35/Female 46/Female NG 61/Male 24/Male 20/Female 40/Female 23/Male 34/Female 14/Female
1 1 1 1 3
NG NG NG 3 year NG
2
NG
Orbit Orbit Orbit Orbit Lac gland Lac gland Orbit Lacrimal sac
18 months 12 years 10 years (2 recurrences one at 4th yr and other at 6th yr) 22 months 18 months 23 months 6 years 9 months 1 year NG NG NG 2 years NG NG Post enucleation followed by exenteration 20 years later 2 years NG NG NG 21 months 5 months 22 months NG
1
5 months
Orbit
38/Male NG NG 24/Female 69/Male 72/Male 45/Female 31/Male 18/Female 62/Male 38/Female 10/Male 40/Female 54/Male 30/Male
1 1 1
12 months NG NG NG
Orbit Orbit Orbit
42/Female 26/Male 30/Male 44/Male 9/Male
Duration
Location
Orbit Lac gland Orbit Orbit Orbit Lacrimal sac Orbit Lacrimal sac Orbit
7 Orbit
1 1 1 1
3 60 months 48 months 7 months 36 months
Orbit Orbit Orbit Orbit & extraorbit
6
84 months 36 months 12 months 6 months 6 months
Orbit
Follow-up
Recurrence after 4 months NG NG 8-year follow-up 30 months 12 months 12 months 6 months 6 months 18 months 12 months 15 months NS Not given Recurrence twice 14 months (recurrence at 3 months) 18 months 21 months Lost to follow-up 10 months 3 months
NG ⫽ not given.
with global limitation of movements. An ill-defined non-tender mass was felt along the inferomedial orbit with poorly defined margins. The anterior segment and fundus examination was normal in both eyes.
Computed tomography (CT) scan of the orbit study revealed a well-circumscribed extraconal mass lesion measuring 36 mm × 23 mm × 32 mm in the nasal orbit with well corticated erosion of the medial
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Fig. 1. Computerized tomography of the orbit showing a well-circumscribed peripherally enhancing lesion with cystic areas (case 2).
orbital wall. An ultrasound study showed low to moderate internal reflectivity with fairly homogenous structure without evidence of compressibility or vascularity. No cystic areas were seen. An initial fine needle biopsy showed clusters of small to mediumsized spindle cells arranged in a storiform pattern suggestive of a spindle cell tumor. A medial orbitotomy was performed and the tissue was submitted for histopathological study. The patient was lost to follow-up.
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Fig. 2. Ultrasound of the orbit showing a well-defined lesion in the supero-temporal quadrant with multiple cystic spaces without infiltration of the lateral or superior rectus (case 2).
CASE 3
A 42-year-old woman was seen in October 1999 with a proptosis of the right eye of 7 years duration. She had undergone an incisional biopsy earlier, and labeled to have fibrous histiocytoma or a low-grade spindle cell sarcoma of the orbit. Ophthalmic examination revealed a visual acuity of 20/80 in the right eye was. There was a 12-mm proptosis with a superior displacement of the globe, fullness of the superior orbit, and a firm multinodular mobile tumor along the inferolateral orbit (Fig. 3). There was global
CASE 2
A 26-year-old man was seen in June 1999 with complaints of swelling of the outer part of left upper lid of 3 years duration. He had undergone an open biopsy elsewhere 3 years earlier and labeled to have inflammatory pseudotumor of the orbit. Ophthalmic examination showed his visual acuity was 20/20 in both eyes. There was mild left upper eyelid ptosis with a prominent palpebral lobe of the lacrimal gland. There was a down and in proptosis of 4 mm with global restriction of ocular movements. The anterior segment and fundus examination was normal. The right eye and systemic examinations were normal. CT of the orbit revealed a well-circumscribed peripherally enhancing lesion with central cystic areas significantly increased in size from the previous studies (Fig. 1). An ultrasonogram of the orbit revealed a well-defined lesion in the supero-temporal quadrant with multiple cystic spaces without infiltration of the lateral or superior rectus (Fig. 2). The patient underwent a repeat lateral orbitotomy. At 2-year follow-up, the patient remained asymptomatic without recurrence of the tumor.
Fig. 3. External photograph showing proptosis with a superior displacement of the globe (case 3).
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Fig. 4. Computerized tomography of the orbit revealed an intensely enhancing well-circumscribed large orbital mass lesion measuring 5 × 4.8 × 4.5 cm (case 3).
restriction of all ocular movements with exposure keratopathy. Fundus revealed venous dilatation, tortuosity, and pallor of the optic nerve head. Examination of the left eye was normal. Regional and systemic examinations were normal. CT of the orbit revealed an intensely enhancing well-circumscribed large orbital mass lesion measuring 5 cm × 4.8 cm × 4.5 cm causing proptosis with stretching of the optic nerve (Fig. 4). An ultrasound study revealed a multilobulated mass with variable echolucency surrounding the optic nerve without indentation of the globe. The lateral orbital rim appeared thin without erosion. The patient underwent an inferolateral orbitotomy. At 18-month follow-up, visual acuity was 20/20 with full extraocular movements and no afferent defect. Repeat imaging ruled out residual tumor or recurrence of the tumor. CASE 4
A 30-year-old man was seen in February 2000 with a 3-year history of insidious prominence and decreased vision. A CT scan of the brain and orbit revealed an intensely enhancing mass lesion of the inferomedial orbit for which he had an incisional biopsy elsewhere and diagnosed as either a neurofibroma or schwannoma. Ophthalmic examination revealed perception of hand movements on the left eye. There was a relative afferent pupillary defect with gross supero-temporal displacement of the globe with a firm lobulated mass felt along the inferonasal orbit and conjunctiva, with scarring of the cornea (Fig. 5). Fundus examination revealed disk edema with venous dilatation and tortuosity on the left side. Right eye was normal. With a diagnosis of a benign soft tissue tumor, he underwent an anterior orbitotomy. Postoperatively, the patient continued to have severe globe
Fig. 5. External photograph showing a supero-temporal displacement of the globe with a firm lobulated mass felt along the infero-nasal orbit and conjunctiva, with scarring of the cornea (case 4).
malposition and a recurrence of firm lesions along the inferior orbit 3 months later. Vision was stable. An exploration with biopsy revealed findings consistent with the previous pathology. CASE 5
A 44-year-old man was seen in March 2000 with a 6-month history of gradual progressive visual loss with displacement of the right eye. Ophthalmic examination revealed a best corrected visual acuity of 20/200 in the right and 20/80 in the left. Examination of the left eye was unremarkable while on the right there was a gross superior and lateral displacement of the eye. There was a dense relative afferent pupillary defect. The inferior cornea was scarred with vascularization. A firm to hard mass was felt along the infero-medial orbit extending superiorly and laterally the posterior aspect of which could not be made out. Fundus examination revealed a hyperemic edematous disk with choroidal folds. CT scan of the orbits revealed a brilliantly enhancing well-circumscribed lesion along the medial orbit displacing the medial rectus and optic nerve with indentation of the globe and remodeling of the adjacent bone without erosion. An ultrasound study showed the lesion to have a homogenous internal reflectivity suggestive of compact tissues with indentation of the globe. No cystic areas were seen. He underwent medial orbitotomy. Postoperatively, there was an improvement of vision to 20/20 with resolution of the disk edema and choroidal folds. There was no recurrence at the 10-month follow-up visit.
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CASE 6
A 9-year-old boy presented with swelling of the left upper and lower eyelids that had been intermittent during the previous 6 months. There was no history of trauma, surgery, pain, redness, or systemic illnesses. He was otherwise healthy. His best corrected visual acuity was 20/20 in both eyes. External examination revealed increased periorbital pigmentation worse in the left eye. The orbital rims were intact. Ocular motility was within normal limits. Exophthalmometry readings were 13 and 15 in the right and left eye respectively with a base reading of 84. There was 2–3 mm of downward displacement of the left globe. There was no relative afferent papillary defect. Anterior segment and fundus examinations were unremarkable. MRI revealed a well-circumscribed brilliantly enhancing mass along the medial extra conal orbit not infiltrating the medial rectus or the bone. The rest of the orbit and the intracranial space were within normal limits. With a provisional diagnosis of soft tissue tumor he underwent left medial orbitotomy. The child is on 3-month follow-up and there is no recurrence of the tumor.
Imaging Studies ULTRASOUND
Four of the patients (cases 2,3,4,5) had undergone ultrasound evaluation of the orbit. All of them had well-circumscribed masses without infiltration of the optic nerve or rectus muscle. Two (cases 2 and 3) had intralesional cystic spaces. The other two had homogenous echolucency (cases 4 and 5), with one showing indentation of the globe (case 5). Case 6 did not undergo ultrasound study.
Fig. 6. Photomicrograph showing the gross tumor in case 3 showing a well-circumscribed pseudo encapsulated masses ranging from 2.1–5.7 cm in the greatest dimension (case 3).
PATHOLOGIC EXAMINATION
Gross examination of the tumor in all the cases showed well-circumscribed pseudo encapsulated masses, ranging from 2.1–5.7 cm in their greatest dimension (Fig. 6). Cut section of the tumor showed a tan and fleshy appearance with areas of hemorrhage in the tumor. Microscopically in all six cases the tumor was composed of fibrocollagenous tissue and spindle cells arranged in a fascicular pattern around thick collagen fibers, with occasional myxoid stroma (Fig. 7). In certain areas a keloid-like pattern surrounded with fibroblasts was seen (Fig 8). Numerous vascular channels with a pericytomatous arrangement were seen. Two of them (cases 2 and 3) had cystic component (Fig. 9). A possibility of solitary fibrous tumor
COMPUTERIZED TOMOGRAPHY
CT scan imaging of the orbits showed brilliant enhancement in all patients. Whereas in four patients (cases 1, 2, 4, and 5) there was diffuse intralesional enhancement, in one (case 3) there was peripheral ring of enhancement only. The feature of intense enhancement was so strong that in two patients (cases 3 and 4) a preoperative diagnosis of solitary fibrous tumor was suspected in two patients. The location of the tumor was in the inferomedial (case 1), superior and inferolateral (case 2), superotemporal (case 3), nasal (case 4), and medial (cases 5 and 6) portion of the orbit, respectively. MAGNETIC RESONANCE IMAGING
MRI in one (case 6) showed a brilliantly enhancing mass along the medial extraconal orbit.
Fig. 7. Photomicrograph showing spindle cells arranged in a fascicular pattern around thick collagen fibers, with occasional myxoid stroma (hematoxylin and eosin stain × 200).
SOLITARY FIBROUS TUMOR OF THE ORBIT
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Fig. 8. Photomicrograph showing the thickened keloid like collagen fibers surrounded by the spindle cells (arrow) (hematoxylin and eosin stain × 200).
Fig. 10. Photomicrograph showing positive staining of the cytoplasm of the tumor cells with CD34 (immunoperoxidase CD 34 × 200).
of the orbit was considered based on these characteristics and subjected to immunohistochemical marker CD 34 study.
of orbital SFT occurring in the pediatric age group, including our case.1,38 Thus, SFT must also be included in the differential diagnosis of pediatric orbital masses. At presentation all the patients in our series had a well-circumscribed mass, the anterior surface of which had a smooth contour. The clinical features of the patients in our series are summarized in Table 3. All the patients presented with unilateral painless proptosis of insidious onset over a mean duration of 34.8 months (6–84 months). At presentation visual acuity in three patients (cases 1,2, and 6) was normal. In the others it was 6/24 (20/80), 6/60 (20/ 200) and perception of hand motions (cases 3,4, and 5). In the cases 1, 3, 4, and 5, visual acuity improved to 6/6 (20/20) postoperatively. Solitary fibrous tumor typically arises in the pleura and has been called “localized mesothelioma” because it was once thought to be of mesothelial cell origin. The neoplasm was originally described by Klemperer and Rabin in 1931.35 They characterized localized pleural tumors as large, slowly growing, pedunculated masses covered by visceral pleura and hypothesized that their origin proposed a mesothelial origin for the localized pleural tumor. However, their histogenesis was controversial and many reports claim that they are of submesothelial stromal cell or fibroblast-like mesenchymal cell origin, supported by evidence from histologic, immunohistochemical, ultrastructural, and tissue culture studies. Recently, SFT has been described in extrapleural sites, including lung, mediatinum,57 peritoneum,60 upper respiratory tract,33,58 thyroid, liver,3 and soft tissue.7,32 The first SFT presenting in the orbit was independently reported in 1994 by Dorfman et al15 and Westra et al,56 respectively. Stout and Murray first described the microscopic appearance of SFT.48 Solitary fibrous tumor shows a patternless, hypercellular and
IMMUNOHISTOCHEMICAL STUDY
The paraffin-embedded sections were subjected to standard immunohistochemical protocols using biotin-streptavidin-peroxidase detection (DAKO Corporation, Carpentaria, CA). Immunohistochemically the tumor cells were CD34 positive in the cytoplasm (Fig. 10) supporting a diagnosis of orbital SFT.
Discussion It is important to differentiate SFT occurring in the orbit from the other spindle cell tumors that mimic it (Table 2). Our series contained more men than women (M5:F1). The age ranged from 9 to 44 years, and the mean age was 34.4 years. Thus, SFT of the orbit occurs over a wide age range, including the pediatric age group. We have found three cases
Fig. 9. Photomicrograph showing the cystic component tumor. The cystic space contains pale eosinophilic fluid (arrow) (hematoxylin and eosin stain × 200) (case 2).
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TABLE 2
Important Differential Diagnosis of Spindle Cell Tumors of the Orbit Tumor
Histopathological Features
Monophasic synovial sarcoma
Densely packed fascicles, fibrosarcomatous, or hemangiopericytomatous pattern, oval to round nuclei
Fibrosarcoma
Herring bone pattern, slender spindle nuclei, interwoven collagen between cells Abundant eosinophilic cytoplasm, cigar-shaped nuclei
Smooth muscle tumors Malignant Peripheral nerve sheath tumor Solitary fibrous tumor Giant cell angiofibroma Giant cell fibroblastoma Spindle cell squamous cell carcinoma Cellular fibrous histiocytoma Desmoplastic amelanotic melanoma
Sweeping fascicles, wary nuclei, occasional heterologous elements Variety of patterns, deposition of collagen between cells Spindle cells arranged in a fascicular pattern, pseudovascular spaces, giant cells Spindle cells, pseudovascular spaces, giant cells, pleomorphism Spindle cells in fascicular pattern, mitosis Spindle cells, fascicular pattern, no mitosis Spindle cells, mitosis
Immunohistochemical Features Keratin⫹, focal EMA⫹, focal bcl 2⫹, CD34⫺, Desmin⫺, Vimentin ⫹ (spindle cells) Vimentin_⫺ ve (epithelioid cells) S 100⫹ rare focal Keratin ⫺, EMA⫺, bcl 2⫹, CD34⫹ Keratin ⫹ (rare focal), Vimentin ⫹ve, Desmin ⫹, SMA ⫹ bcl 2⫺, CD34 ⫺ve Keratin ⫹ rare, Vimentin ⫹ve, Focal EMA⫹ rare, Focal S100 ⫹ rare, bcl 2⫹ rare, CD34 ⫺ve Keratin⫺, EMA⫺, bcl 2⫹, Vimentin ⫹, CD34 ⫹ Vimentin ⫹, CD 34 ⫹, S100 ⫺ve, Keratin⫺, EMA ⫺ Vimentin ⫹, CD 34 ⫹, F VIII ⫺ve, S100 ⫺, Keratin ⫺, EMA ⫺ Cytokeratin ⫹, Vimentin ⫺ve, S 100 ⫺ve, Desmin ⫺ve, CD34 ⫺ve, EMA ⫺ve Keratin ⫺ve, Vimentin ⫹ve, S 100 ⫺ve, Desmin ⫺ve, CD34 ⫹, EMA ⫺ve Keratin ⫺ve, Vimentin ⫹ve, S100 ⫹ve, Desmin ⫺ve, CD34 ⫺ve, EMA ⫺ve
Cytogenetic/Molecular Abnormalities t(x:18)(p11.2:q11.2)
⫹8,⫹11,⫹17,⫹20 (for infantile fibrosarcoma) Del 1p(in ⬎75%) of leiomyosarcoma Complex chromosomal aberrations t(9:22) (q31:p13) Not known
Not known Not known Not known Not known
EMA ⫽ epithelial membrane antigen; SMA ⫽ smooth muscle actin; Del ⫽ deletion; p ⫽ chromosome long arm; q ⫽ chromosome short arm; t ⫽ translocation.
hypocellular region of spindle cells, against a background of thick collagen bands. Focal hemangiopericytoma like staghorn, fibrous histiocytoma like storiform pattern, synovial sarcomatous and neurallike palisading regional architecture have been described with SFT.2 This multiplicity of histologic patterns and an absence of single intralesional architecture characterize SFT. This variability could lead to mistaken diagnosis if limited tissue allows a single morphology to dominate the histopathologic specimen and immunohistochemical studies are not performed. Cases 2, 3, and 4 had previously undergone incisional biopsy elsewhere and were labeled as inflammatory tumor (case 2), fibrous histiocytoma or spindle cell sarcoma (case 3), and neurofibroma, neurilemomma (case 4). In each of these cases, intraoperatively there was no evidence of tumor invasion into adjacent soft tissue or bone enabling
relatively easy delivery except in case 4, in which the plane of dissection may have been complicated by the previous incisional biopsy. In this patient repeat orbitotomy was necessitated due to persistent growth or recurrence of the tumor. The light microscopic differential diagnosis of SFT includes giant cell angiofibroma, fibrous histiocytoma, hemangiopericytoma, peripheral nerve sheath tumor, fibromyxoid sarcoma, monophasic synovial sarcoma, and other rare mesenchymal tumors.13,23,25 Solitary fibrous tumor is immunoreactive for mesenchymal markers such as vimentin but negative for desmin, epithelial marker (cytokeratin), vascular marker (factor VIII related antigen), neural markers (S-100 protein), and muscle specific actin and smooth muscle actin.17,20,30,40 Immunoreactivity with the marker CD34 is present in 79–100% of cases.41,50,55 Westra and associates also found that 9/ 9 neurofibromas showed immunopositivity for CD34,
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SOLITARY FIBROUS TUMOR OF THE ORBIT TABLE 3
Summary of the Clinical Findings in Our Cases No./Age/Sex Duration of the Mass
Earlier Diagnosis
Site
Follow-up and Recurrence
Ultrasound
Case 1 30 years Male 12 months Case 2 26 years Male 36 months
Spindle cell tumor (FNAB)
Nasal
Lost to follow-up
Inflammatory pseudotumor
Superolateral
21 months
Case 3 42 years Female 84 months
Fibrous histiocytoma/ Spindle cell sarcoma (low grade) Neurofibroma/ Neurilemmoma
Superior and Inferolateral
18 months
Multilobulated mass, variable echolucency
Inferomedial
14 months with recurrence at 3 months
Not done
Intensely enhancing mass
None
Medial
10 months no recurrence
Homogeneous internal reflectivity, no cystic areas
Brilliantly enhancing well-circumscribed mass
None
Medial
3 months no recurrence
Not done
Not done
Case 4 30 years Male 36 months Case 5 44 years Male 6 months Case 6 9 years Male 6 months
suggesting that while sensitivity appears to be excellent for this marker, the potential diagnostic specificity is less than perfect.56 Uniform positivity for vimentin and CD34 supports a diagnosis of SFT. Although giant cell angiofibroma shares an identical immunostaining pattern, multinucleated giant cells are not usually seen in SFT. Recently a giant cell rich variant of SFT has been reported.28 Dei Tos et al described a series of seven cases of a morphologically distinct orbital tumor with rich vascularized and patternless proliferation of spindle cells and some pseudovascular spaces embedded in a variably collagenized stroma, some with myxoid deposit.14 The spindle cells and the multinucleated giant cells are intensely positive for vimentin and CD 34. GCA closely mimicks SFT and giant cell fibroblastoma. However, multinucleated giant cells and pseudovascular spaces are not features of SFT. Additionally, orbital SFT is thought to arise from more deeply situated and cause greater proptosis in contrast to GCA. Giant cell fibroblastoma mimicks GCA. Giant cell fibroblastoma is a childhood tumor and a rare mesenchymal tumor. It is composed of loosely arranged spinde cells with a biphasic pattern consisting of solid and angiectoid (pseudovascular) areas and moderate degree of nuclear pleomorphism.
Homogeneous mass, low to moderate internal reflectivity Well-defined lesion, multiple cystic spaces
Computerized Tomogram Well-circumscribed, extraconal mass Well-circumscribed, peripherally enhancing mass with central cystic areas Intensely enhancing well-circumscribed mass
This has a number of similarities to dermatofibrosarcoma protuberans. It is a juvenile analog of the latter. There is some degree of morphologic overlap between GCA and giant cell fibroblastoma. Giant cell fibroblastoma is also positive for CD34 (Table 4 lists the similarities and differences between GCA and giant cell fibroblastoma). Thus GCA and giant cell fibroblastoma must be also included in the differential diagnosis of orbital SFT. Solitary fibrous tumors, GCA, and giant cell fibroblastomas are positive for vimentin, CD34, and bcl-2.26,46,49 Thus these markers lack sufficient specificity to independently distinguish SFT from the other spindle cell tumors. Solitary fibrous tumor as an entity, however, is rarely diagnosed clinically. Radiologic characteristics being typical may lead to preoperative suspicion. In our series, four patients underwent ultrasound examination of the orbit and all of them had well-circumscribed masses without infiltration of optic nerve or rectus muscle. It was helpful in identifying the cystic component in case 2 and the other 3 had homogeneous echogenecity. CT scan may show moderate to intense enhancing well-circumscribed mass. Bony modeling or well-corticated erosion may be seen in long-standing lesions adjacent to the orbital wall. In all but one patient
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TABLE 4
Differences and Similarities Between Giant Cell Angiofibroma, Giant Cell Fibroblastoma, and Solitary Fibrous Tumor Features
Giant Cell Angiofibroma
Giant Cell Fibroblastoma
Age of presentation
Adults
Childhood
Light microscopy
Spindle cell tumor, vascular spaces
Spindle cell tumor, pseudovascular spaces
Vascular pattern
More prominent No
Much less conspicuos Yes
Yes Multinucleate giant cells CD 34⫹ve Vimentin ⫹ve, Factor VIII ⫺ve S 100 ⫺ve No
Yes Outpouchings from a single nucleus CD 34⫹ve Vimentin ⫹ve, Factor VIII ⫺ve S 100 ⫺ve Yes
Pleomorphism of the nucleus Giant cells Electron microscopy on giant cells Immunohistochemistry
Relationship to dermatofibrosarcoma
brilliant enhancement was seen, this finding was so characteristic that in two patients a diagnosis of SFT (cases 3 and 4) was suspected. Magnetic resonance imaging was done in only one of our cases. However, MRI of orbital SFT provides characteristic features. Solitary fibrous tumors demonstrate a T1 signal intensity that is isointense to gray matter with a homogeneous or heterogeneous enhancement with gadolinium.34 T2 intense signal intensity is predominantly hypo-intense to gray matter. SFT frequently demonstrate central foci that are profoundly hypo-intense on both T1 and T2 imaging.5 T2 weighted signal hypo-intensity and intralesional signal heterogeneity are distinguishing features of SFT.21 This low signal intensity on T2 weighted images may be more suggestive of a fibrous orbital lesion with high collagen content. These magnetic resonance imaging characteristics are similar to those described for non-orbital SFT. This may help guide the surgeon to proceed with complete excision of the mass rather than fine needle aspiration or excision biopsy. It is undisputed that this tumor is of mesenchymal origin, supported by the strong CD34 immunoreactivity.40,41 CD34 is a surface glycoprotein expressed by human hematopoietic progenitor cells on their surface.52 CD34 is positive in normal and neoplastic endothelial cells, which raises the possibility that the cells of the SFT may be differentiated towards the vascular endothelial cells.10 On the other hand, due to the lack of factor VIII expression, it is possible that the cells of the SFT are not endothelial in origin,
Solitary Fibrous Tumor Childhood and adults Spindle cell tumor, vascular spaces, cystic spaces Hemangiopericytoma like pattern Usually no No – CD 34⫹ve Vimentin ⫹ve, Factor VIII ⫺ve S 100 ⫺ve No
but rather undifferentiated mesenchymal cells that have the ability, possibly through stromal cell/matrix interaction, to induce vascular structures that may give rise to a pattern like hemangiopericytoma.24,50 Patchy actin positivity, focal intercellular junctions, and cytoplasmic dense bodies are suggestive of myofibroblastic/fibroblastic differentiation. Also, myofibroblastic tumors reported in the breast and inguinal lymph nodes also bear a striking morphologic similarity to SFT.51,54 Genetic analysis of SFT has also been reported. More recently a balanced translocation t (9; 22)(q31; p13), one distinct from Philadelphia chromosome, t (9; 22)(q34; q11), seen in certain hematologic malignancies, has been described.27 It is also different from the common translocation of myxoid chondrosarcoma, which is t (9;22) (q22;q12).47 Like other soft tissue tumors, SFT may have typical genetic aberrations that will be only known after more in depth studies are undertaken. Solitary fibrous tumor of the orbit does not have any systemic symptoms such as hypoglycemia, arthralgia, and pleural or peritoneal effusion, which are known to occur in cases of SFT occurring at pleural sites.2 Solitary fibrous tumor of the pleura have been responsible for patient death in 12% of cases, either because of late diagnosis or unresectable recurrence.4 However, there are no reports of orbital SFT leading to death. Although these tumors are always well-circumscribed, to facilitate total excision, occasional cases of recurrences have been reported. Recurrence was seen in four cases, including one of our cases in which recurrence was seen in 3 months.
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Dorfman reported a 69-year-old patient who developed two recurrences 4 and 6 years postoperatively.15 Alexandrakis reported a 14-year-old girl who presented with proptosis of 5 months duration and developed recurrence 4 months postoperatively.1 Ing et al reported a patient who underwent an enucleation for neurofibroma and underwent exenteration 20 years later for an orbital recurrence when a diagnosis of SFT was made.31 Recurrences are due to incomplete excision. Solitary fibrous tumors generally behave in a benign manner and do not metastasize.6 Criteria for malignancy include the presence of more than four mitosis per high-power field (or the presence of abnormal mitotic figures), cellular pleomorphism, or tumor giant cells.17,25 There is only one reported case of malignant SFT of the orbit.6 Recurrent SFT can reveal more mitotic activity then the original tumor as reported by Dorfman and his colleagues,15 where the original tumor showed a mitotic activity of less than 2 per 10 high power field (HPF), while the recurrent tumor showed a mitotic activity of 5 to 6 per 10 HPF. Therefore, the most important prognostic factor is not the histologic appearance but the complete respectability of the tumor, whether it is located in the orbital or in the pleura.44
Conclusion In conclusion we present six cases of orbital SFT, which have pursued a benign course except in one (case 3), which had a recurrence within 3 months and the tumor did not show any increased mitotic activity. SFT of the orbit occurs in varied age groups, has a slightly male predominance, occurs in a younger age group, and it can also occur in the pediatric age group as in our series (case 6). Therefore, SFT should thus be considered in the differential diagnosis when a patient presents with a well-circumscribed tumor causing unilateral proptosis with intensely enhancing mass seen on the CT scan evaluation. Fine needle aspiration is also helpful in establishing a diagnosis, if the tissue sample is adequate and immunomarkers could be done on the fine needle aspiration material.9 Likewise when confronted with a spindle cell tumor of the orbit, the ophthalmic pathologist should also consider the possibility of SFT and judiciously use immunohistochemical markers to establish the diagnosis. Orbital SFT seems to be clinicopathologically very similar to pleural SFT. Both usually have an indolent behavior. In order to avoid recurrences a wide aggressive resection and prolonged follow up are favored. The value of adjuvant chemotherapy or irradiation is not known.
Method of Literature Search We searched the Medline database, using keywords such as solitary fibrous histiocytoma, orbit, histopathology, and various combinations of the above, for the years 1966–2001. We searched both English and foreign languages. There was one Slovak reference50 and its English abstract was used for the necessary information.
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The authors reported no proprietary or commercial interest in any product mentioned or concept discussed in this article. The authors were supported by a grant from the Vision Research Foundation, Chennai, India. The manuscript was presented in part at the 10th International Conference of Ocular Oncology Meeting, Netherlands, June 17–22, 2001.