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Enlarging Lesion of the Upper Eyelid
CLINICOPATHOLOGIC CONFERENCE J Oral Maxillofac Surg 64:943-948, 2006
Enlarging Lesion of the Upper Eyelid Fabio Roccia, MD,* Amedeo Tavolaccini, MD,† Dino Daniele, MD,‡ and Sid Berrone, DDS§ Report of a Case
Vascular malformations of the orbit are rare. Medium-flow, capillary-venous, and venous malformations (also known as cavernous hemangiomas) are often retroconal, round, and well-defined. They can produce more marked exophthalmos in the prone or supine position, or when performing the Valsalva maneuver. CT may show intralesional calcifications with moderate enhancement, while magnetic resonance imaging (MRI) shows an isointense signal in T1-weighted images or a hyperintense signal in T2weighted images. The angiographic blush is moderate, but is intense in high-flow vascular malformations, which produce a strong signal on T2-weighted MRI, with significant enhancement in both CT and MRI. High-flow vascular malformations may also be associated with a volumetric increase of the ophthalmic vein and mimic the presence of a carotid-cavernous fistula. Lymphangiomas, which are more common in younger age groups, may be intraconal or extraconal and produce bone changes. On MRI they appear hypointense on T1 and markedly hyperintense on T2; on CT they are isodense to muscle and can have variable enhancement and fluid-fluid levels related to old hemorrhages. Hemangiopericytomas are a form of vascular tumors or, more accurately, sarcoma; 50% of cases are malignant, and there is a tendency for recurrence.1-3 Although approximately 25% of hemangiopericytomas are located in the head and neck, they represent only a small percentage of the tumors found in this area. They are rare in the orbit and are located more frequently in the retrobulbar area, causing exophthalmos. They often show marked enhancement on CT and with bone invasion, while the MRI signal characteristics are atypical, as they cannot be differentiated from meningiomas or cavernous hemangiomas. Angiography often shows an intense pathologic blush. Lymphomas may occur either as isolated lesions or as part of a systemic lymphoma. There are benign forms and pseudolymphomas, which are difficult to differentiate from pseudotumors and malignant forms. They are often retrobulbar or located in the lacrimal gland. The CT and MRI signs are often nonspecific. CT shows hyperdense pathologic tissue with distinct edges in the anterior part of the orbit, or in the lacrimal gland, which blends with the surround-
A 44-year-old man presented to our department for a swelling approximately 1.5 cm in diameter located in the medial aspect of the left upper eyelid. The swelling was painless, elastic in consistency, slightly pulsatile, and slow growing (Fig 1). Present for approximately 1 year, the lesion had produced ingravescent blepharoptosis. Clinical examination showed normal visual acuity and preserved ocular motility. Based on an ultrasound carried out elsewhere, a vascular lesion was suspected. A computer tomography (CT) scan with contrast medium was carried out, which confirmed the presence of a well-defined lesion below the orbicularis muscle of the left upper eyelid with distinct contrast and no bone structure involvement (Fig 2). Therefore, preoperative angiography was performed, which confirmed that the eyelid lesion was vascularized by branches of the ophthalmic artery, and a medium-flow vascular malformation was diagnosed (Fig 3). Given the risks associated with embolization of the ophthalmic artery branches and the surgically accessible location of the lesion, it was decided not to proceed with embolization.
Differential Diagnosis Dino Daniele, MD When a patient presents with a slow-growing, painless swelling in the upper eyelid and the slightest pulsatile of the mass is perceived, a vascular lesion is often suspected, particularly when supported by an angiography. However, in the radiological differential diagnosis of an adult orbital lesion using angiographic blush and CT enhancement, certain benign and malignant pathologies need to be considered, including vascular malformations, hemangiopericytomas, lymphomas, neural sheath tumors, fibrous tumors of the orbit, rhabdomyosarcomas, meningiomas, and orbital metastases. Received from San Giovanni Battista Hospital, Turin, Italy. *Assistant Professor, Division of Maxillofacial Surgery. †Professor, Division of Maxillofacial Surgery. ‡Resident, Division of Maxillofacial Surgery. §Assistant Professor, Neuroscience Department. Address correspondence and reprint requests to Dr Roccia: Division of Maxillofacial Surgery, San Giovanni Battista Hospital, Via Febo 19, 10133 Torino, Italy; e-mail: [email protected] © 2006 American Association of Oral and Maxillofacial Surgeons
0278-2391/06/6406-0012$32.00/0 doi:10.1016/j.joms.2006.02.016
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FIGURE 2. Axial computer tomography scan showing well-circumscribed mass of the upper eyelid without any calcification or bony structure involvement. Roccia et al. Enlarging Lesion of the Upper Eyelid. J Oral Maxillofac Surg 2006.
FIGURE 1. Preoperative case showing left blepharoptosis. Roccia et al. Enlarging Lesion of the Upper Eyelid. J Oral Maxillofac Surg 2006.
ing tissue and rarely erodes bone, with weak enhancement. On MRI, the signal is medium or low on T1weighted images and isointense to fat on T2 images. Of the neural sheath tumors, neurofibromas and schwannomas should be considered. Neurofibromas may be plexiform, diffuse, or localized and can involve the upper eyelid or be located in the retrobulbar area. On CT and MRI there is marked enhancement. The radiological appearance on MRI is atypical and features a low signal on T1 and a high signal on T2. Schwannomas typically originate in the retrobulbar area, with CT and MRI features that are similar to neurofibromas. Angiography rarely produces a pathologic blush. Fibrous histiocytomas, fibromas, solitary fibrous tumors, fibrosarcomas, and nodular fasciitis are all fibrous tumors of the orbit. Fibrous histiocytomas are the most frequent mesenchymal tumors of the orbit. They are benign in 63% of cases, locally aggressive in 26% of cases, and malignant in 11% of cases.4 They appear as a well-defined intraconal or extraconal mass in the medial or upper part of the orbit. The most common symptoms include proptosis, decreased vision, diplopia and pain. CT shows a mass with moderate to intense enhance-
ment; sometimes there is no contrast in the central area owing to tissue necrosis. Malignant forms may cause bone erosion. On MRI, fibrous histiocytomas show an intense signal on T2, but they are difficult to differentiate from neurofibromas or other fibrous lesions of the orbit. Fibromas are the rarest fibrous tumors of the orbit and typically occur in young adults. They can be solitary, multicentric, or generalized. They originate
FIGURE 3. Lateral projection arteriogram showing the feeder vessel to the orbital lesion. Roccia et al. Enlarging Lesion of the Upper Eyelid. J Oral Maxillofac Surg 2006.
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from Tenon’s capsule or the fascia of the extrinsic ocular muscles, but can also involve the eyelid. On CT, fibromas appear as a well-defined round or oval homogeneous high-density mass that clearly stands out from the surrounding structures and is similar to cavernous hemangiomas. MRI features are nonspecific and are associated with the benign or invasive nature of the tumor. Solitary fibrous tumors are generally benign tumors of mesenchymal origin. They can be located anywhere in the orbit and may cause proptosis. CT shows a mass with well-defined edges and possible bone changes typical of slow-developing tumors, with inhomogeneous enhancement. MRI shows a parenchymal isointense signal on T1 images and inhomogeneous iso/hypointensity on T2 images (a feature that differentiates them from other fibrous tumors); enhancement is homogeneous, as is the blush that is observed in the angiography arterial phase. The known forms of fibrosarcoma include congenital, infantile, juvenile, and adult form. Adult fibrosarcomas can be well delimited or infiltrative and cause nerve and functional deficits. CT and MRI images are nonspecific for orbital fibrosarcomas, as they usually show an orbital infiltrating mass. MRI shows a welldemarcated mass on T1 and T2 images. Nodular fasciitis is a fibrous tumor that is common in the limbs and torso in adults, but involvement of the head and neck is more commonly observed in pediatric patients. Orbital involvement affects mainly the eyelid, conjunctiva, and Tenon’s capsule. It may develop within weeks or months, mimicking a malignant lesion. The CT and MRI results are nonspecific and similar to those for other fibrous tumors of the orbit. Meningiomas, which are mainly endocranial and originate in the dura mater, may have extracranial extensions involving the face area, including the orbital region. Rarely (1%), extracranial meningiomas can occur mainly in the orbit, nasal sinuses, mouth, middle ear, cervical soft tissues, and mediastinum. On CT, they appear as a mass of parenchymatous density with moderate enhancement and produce bone changes. On MRI, the signal features are similar to those of the cerebral parenchyma. Angiography produces an intense and persistent blush. Of the malignant neoplasias, rhabdomyosarcomas commonly occur in children, but the pleomorphic form also occurs in adults. They occur anywhere in the orbit, primarily in the upper part. They cause proptosis and rapid-onset exophthalmos. On CT, they appear as a well-defined, isodense mass in muscle, with moderate to intense enhancement. Bone erosion may be observed with large rhabdomyosarcomas, whereas the CT results may appear nonhomogeneous after entoptic hemorrhages. On MRI, the signal is
FIGURE 4. Intraoperative appearance of the upper lid lesion. Roccia et al. Enlarging Lesion of the Upper Eyelid. J Oral Maxillofac Surg 2006.
isointense in the encephalic parenchyma on T1 images and hyperintense on T2 images, while the angiographic blush is intense. Lastly, metastases in the orbit are uncommon, but the incidence is increasing as a result of the improved survival of carcinoma patients. The primary tumor is most frequently in the breasts, followed by the lungs, prostate, and choroid. There are no specific symptoms with orbital metastases, as opposed to other orbital pathologies, but the most common are lid swelling, diplopia, red eye, decreased visual acuity, and proptosis. On CT and MRI, they appear as wellcircumscribed focal lesions in muscle, with possible bone involvement and sensitivity to contrast medium.
Subsequent Course Two days after angiography, the lesion on the patient’s eyelid was excised under general anesthesia. An incision was made along the upper palpebral sulcus to expose the lesion, which was located below the palpebral orbicularis muscle. The lesion was well encapsulated and violet in color, and was enucleated with minimal bleeding during the procedure (Fig 4). There were no postoperative complications, and the patient was discharged 2 days after the surgical procedure.
Pathologic Diagnosis The histological examination of the lesion confirmed the diagnosis of solitary fibrous tumor of the upper eyelid (Fig 5). Therefore, the patient had periodical follow-ups. Four years after the surgical procedure, no clinical or radiological recurrence of the lesion has been observed (Fig 6).
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FIGURE 5. Haematoxylin and eosin-stained tumor section (original magnification, ⫻100) showing storiform pattern of SFTs. Roccia et al. Enlarging Lesion of the Upper Eyelid. J Oral Maxillofac Surg 2006.
Discussion Solitary fibrous tumors (SFTs) are spindle-cell neoplasms (first described by Leutard and then classified by Klemperer and Rabin in 1931)5 that are generally located on serosal surfaces, particularly in the visceral pleura. Owing to their presumed mesothelial origin, they have also been called solitary fibrous mesotheliomas, although recent cell studies lean toward a mesenchymal origin.6 In addition to the well-documented pleural location, extrapleural locations have been reported during the last decade, less frequently in cervicocephalic locations, such as the nasal cavities and paranasal sinuses,6-8 orbit,6,8-41 oral cavity,42-47 parotid,48 temporal region,49 upper respiratory tract,50-52 and soft tissue of the scalp.53,54 Over the last 10 years, 59 cases of orbital SFTs have been described with a distinct prevalence in men (5:1), an age range of 9 to 76 years, and no predominance in either orbit. As with other extrapleural SFTs, orbital SFTs appear nonaggressive, although 9 recurrences attributable to incomplete surgical excision have been documented,9,20,26,36,37 as well as 1 case of malignant transformation.29 However, no local or remote metastases have been described. The slow development of the lesion often delays the initial clinical presentation, and consists mainly of a mass effect of the tumor with progressive asymptomatic unilateral proptosis. Visual disturbances, a palpable orbital mass, and reduced ocular motility are also described. In our case, the palpebral location with no orbital involvement only produced palpebral ptosis, with progressive limitation of the patient’s visual field. As in our case, a radiologist may not be able to provide an accurate diagnosis, which is usually obtained by microscopic examination and immunohistological testing of the excised tissue.
ENLARGING LESION OF THE UPPER EYELID
Histologically, SFTs consist of a mass of spindle cells in a haphazard arrangement. The cells often adopt a storiform pattern, similar to fibrous histiocytoma. Mitotic figures are infrequent, and the count is greater than 4 per 10 high-power fields in association with cellular pleomorphism and nuclear atypia, while areas of focal necrosis are considered indicators of malignity.55 SFTs are highly vascularized, with many dilated vessels that have a stag-horn appearance similar to those observed in hemangiopericytomas.27 However, the final diagnosis of SFTs is based on their strong, diffuse immunoreactivity to the antigen CD34 and to vimentin, as well as their irregular positivity to actin, whereas the markers desmin and S-100 protein are not reactive. Above all, intense CD34 immunohistochemical staining helps differentiate SFTs from hemangiopericytomas (weak CD34 staining), fibrous histiocytomas, and fibrosarcomas (both CD34-negative).10 Schwannomas are positive for CD34 but also express S-100 protein.55 The immunoreactivity of CD34 has only recently been described in SFTs. Therefore, it is assumed that, before the discovery of CD34, or in cases with no
FIGURE 6. Postoperative case showing resolution of the left blepharoptosis. Roccia et al. Enlarging Lesion of the Upper Eyelid. J Oral Maxillofac Surg 2006.
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immunohistochemistry support, SFTs of the orbit were classified under other diagnostic categories, mainly as hemangiopericytomas or fibrous histiocytomas, which are the most common primary mesenchymal tumors of the orbit. This also explains the recent increase in SFT cases described in the literature. Furthermore, the CT and MRI results, as well as the clinical presentation, are rarely helpful in the differential diagnosis of other vascularized orbital masses, as was seen in our case. While SFTs of the pleura and mediastinum can appear aggressive, with invasion of the surrounding tissue, local recurrences, and remote metastases, SFTs in the orbit are usually benign and asymptomatic, and can be cured by complete excision. However, incomplete excision results in a higher percentage of recurrence and the possibility of malignant transformation. Recurring orbital tumors tend to spread locally and invade surrounding tissue, including the orbital bone. Therefore, prolonged follow-up is necessary, as recurrence may occur many years after the tumor has been excised.
References 1. Farhood AI, Hajdu SI, Shiu MH, et al: Soft tissue sarcomas of the head and neck in adults. Am J Surg 160:365, 1990 2. Kraus DH, Dubner S, Harrison LB, et al: Prognostic factors for recurrence and survival in head and neck soft tissue sarcomas. Cancer 74:697, 1994 3. Carew JF, Singh B, Kraus DH: Hemangiopericytoma of the head and neck. Laryngoscope 109:1409, 1999 4. Font RL, Hidayat AA: Fibrous histiocytoma of the orbit: A clinicopathologic study of 150 cases. Hum Pathol 13:199, 1982 5. Klemperer P, Rabin CB: Primary neoplasms of the pleura: A report of five cases. Arch Pathol 11:385, 1931 6. Kim HY, Lee SY, Kang SJ, et al: Solitary fibrous tumor of the orbit: A poorly-recognized orbital lesion. Acta Ophthalmol Scand 77:704, 1999 7. Zukerberg LR, Rosenberg AE, Randolph G, et al: Solitary fibrous tumour of the nasal cavity and paranasal sinuses. Am J Surg Pathol 15:126, 1991 8. Fukunaga M, Ushigome S, Nomura K, et al: Solitary fibrous tumor of the nasal cavity and orbit. Pathol Int 45:952, 1995 9. Dorfman DM, To K, Dickersin GR, et al: Solitary fibrous tumor of the orbit. Am J Surg Pathol 18:281, 1994 10. Westra WH, Gerald WL, Rosai J: Solitary fibrous tumor. Consistent CD34 immunoreactivity and occurrence in the orbit. Am J Surg Pathol 18:992, 1994 11. Lucas DR, Campbell RJ, Fletcher CDM, et al: Solitary fibrous tumor of the orbit. Int J Surg Pathol 2:193, 1995 12. Suster S, Nascimento AG, Miettinen M, et al: Solitary fibrous tumor. A clinicopathologic and immunohistochemical study of 12 cases. Am J Surg Pathol 19:1257, 1995 13. DeBacker CM, Bodker F, Putterman AM, et al: Solitary fibrous tumor of the orbit. Am J Ophthalmol 121:447, 1996 14. McElvanney AM, Noble JL, O’Donovan DG, et al: Solitary fibrous tumor: An atypical presentation within the orbit. Eye 10:396, 1996 15. Ramdial PK, Nadvi S: An unusual cause of proptosis: Orbital solitary fibrous tumor: Case report. Neurosurgery 38:1040, 1996 16. Ruska KM, Westra WH: Pathologic quiz case 1. Solitary fibrous tumor (SFT) of the orbit. Arch Otolaryngol Head Neck Surg 122:1130, 1996
947 17. Sciot R, Goffin J, Fossion E, et al: Solitary fibrous tumor of the orbit. Histopathology 28:188, 1996 18. Scott IU, Tanenbaum M, Rubin D, et al: Solitary fibrous tumor of the lacrimal gland fossa. Ophthalmology 103:1613, 1996 19. Cho NH, Kie JH, Yang WI, et al: Solitary fibrous tumor with an unusual adenofibromatous feature in the lacrimal gland. Histopathology 33:289, 1998 20. Ing EB, Kennerdell JS, Olson PR, et al: Solitary fibrous tumor of the orbit. Ophthal Plast Reconstr Surg 14:57, 1998 21. Lanuza A, Lazaro R, Salvador M, et al: Solitary fibrous tumor of the orbit. Report of a new case. Int Ophthalmol 22:265, 1998 22. Zamecnik M, Michal M: [Solitary fibrous tumor (fibrous mesothelioma). Report of 2 cases in an extraserous location]. Cesk Patol 34:58, 1998 23. de Saint Aubain Somerhausen N, Rubin BP, Fletcher CD: Mixoid solitary fibrous tumor: A study of seven cases with emphasis on differential diagnosis. Mod Pathol 12:463, 1999 24. Festa S, Lee H-J, Langer P, et al: Solitary fibrous tumor of the orbit: CT and pathologic correlation. Neuroradiology 41:52, 1999 25. Woo KI, Suh YL, Kim YD: Solitary fibrous tumor of the lacrimal sac. Ophthal Plast Reconstr Surg 15:450, 1999 26. Alexandrakis G, Johnson TE: Recurrent orbital solitary fibrous tumor in a 14-year-old girl. Am J Ophthalmol 130:373, 2000 27. Havlik DM, Farnath DA, Bocklage T: Solitary fibrous tumor of orbit with a t(9;22) (q31; p13). Arch Pathol Lab Med 124:756, 2000 28. Fenton S, Moriarty P, Kennedy S: Solitary fibrous tumor of the orbit. Eye 15:124, 2001 29. Carrera M, Prat J, Quintana M: Malignant solitary fibrous tumor of the orbit: Report of a case with 8 years follow-up. Eye 15:102, 2001 30. Gigantelli JW, Kincaid MC, Soparkar CN, et al: Orbital solitary fibrous tumor: Radiographic and histopathologic correlations. Ophthal Plast Reconstr Surg 17:207, 2001 31. Giuffrè I, Faiola A, Bonanno E, et al: Solitary fibrous tumor of the orbit. Case report and review of the literature. Surg Neurol 56:242, 2001 32. Hayashi S, Kurihara H, Hirato J, et al: Solitary fibrous tumor of the orbit with extraorbital extension: Case report. Neurosurgery 49:1241, 2001 33. Lucci LM, Anderson RL, Harrie RP, et al: Solitary fibrous tumor of the orbit in a child. Ophthal Plast Reconstr Surg 17:369, 2001 34. Takamura H, Kano M, Yamashita H, et al: A case of orbital solitary fibrous tumor. Jpn J Ophthalmol 45:412, 2001 35. O’Donovan DA, Bilbao JM, Fazi M, et al: Solitary fibrous tumor of the orbit. J Craniofac Surg 13:641, 2002 36. Bernardini FP, de Conciliis C, Schneider S, et al: Solitary fibrous tumor of the orbit. Is it rare? Report of a case series and review of the literature. Ophthalmology 110:1442, 2003 37. Krishnakumar S, Subramanian N, Mohan Ravindra E, et al: Solitary fibrous tumor of the orbit: A clinicopathologic study of six cases with review of the literature. Surv Ophthalmol 48: 544, 2003 38. Schellini S, Hoyama E, Marques ME, et al: Orbital solitary fibrous tumor: Report of two cases and literature review. Jpn J Ophthalmol 47:415, 2003 39. Arturi L, Bollero D, Pucci A, et al: Solitary fibrous tumor of the orbit: Clinical and histological evidence. Eur J Plast Surg 26: 419, 2004 40. Hsu SS, Lai PH, Wang JS, et al: Solitary fibrous tumor of the orbit. J Chin Med Assoc 67:483, 2004 41. Romer M, Bode B, Schuknecht B, et al: Solitary fibrous tumor of the orbit—Two cases and a review of the literature. Eur Arch Otorhinolaryngol 26:81, 2005 42. Eversole LR, Christensen R, Ficarra G, et al: Nodular fasciitis and solitary fibrous tumor of the oral region. Tumors of fibroblast heterogeneity. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 87:471, 1999 43. Iwai S, Nakazawa M, Yoshikawa F, et al: Solitary fibrous tumor of buccal mucosa. Report of a case with immunohistochemical studies. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 88:461, 1999
948 44. Kurihara K, Mizuseki K, Sonobe J, et al: Solitary fibrous tumor of the oral cavity. Report of a case. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 87:223, 1999 45. Perez-Ordonez B, Koutlas IG, Strich E, et al: Solitary fibrous tumor of the oral cavity. An uncommon location for ubiquitous neoplasm. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 87:589, 1999 46. Yamashita Y, Satoh T, Goto M, et al: Solitary fibrous tumor of the tongue: A case report with immunohistochemical studies. Int J Oral Maxillofac Surg 31:681, 2002 47. Veltrini VC, Etges A, Magalhaes MHCG, et al: Solitary fibrous tumor of the oral mucosa—Morphological and immunohistochemical profile in the differential diagnosis with hemangiopericytoma. Oral Oncol 39:420, 2003 48. Ferreiro JA, Nascimento AG: Solitary fibrous tumor of the major salivary glands. Histopathology 28:261, 1996
ENLARGING LESION OF THE UPPER EYELID 49. Hong JP, Chung YK, Kim S-W, et al: Solitary fibrous tumor of the face: A rare case report. Br J Plast Surg 55:75, 2002 50. Witkin GB, Rosai J: Solitary fibrous tumor of the upper respiratory tract: A report of six cases. Am J Surg Pathol 15:842, 1991 51. Safneck JR, Alguacil-Garcia A, Dort JC, et al: Solitary fibrous tumour: Report of two new locations in the upper respiratory tract. J Laryngol Otol 107:252, 1993 52. Gangopadhyay K, Taibah K, Manohar MB, et al: Solitary fibrous tumor of the parapharyngeal space: A case report and review of the literature. Ear Nose Throat 75:681, 1996 53. Okamura JM, Barr RJ, Battifora H: Solitary fibrous tumor of the skin. Am J Dermatopathol 19:515, 1997 54. Cowper SE, Kilpatrick T, Proper S, et al: Solitary fibrous tumor of the skin. Am J Dermatopathol 21:213, 1999 55. Heathcote JG: Pathology update: Solitary fibrous tumor of the orbit. Can J Ophthalmol 32:432, 1997
Enlarging Lesion of the Upper Eyelid Fabio Roccia, MD,* Amedeo Tavolaccini, MD,† Dino Daniele, MD,‡ and Sid Berrone, DDS§ Report of a Case
Vascular malformations of the orbit are rare. Medium-flow, capillary-venous, and venous malformations (also known as cavernous hemangiomas) are often retroconal, round, and well-defined. They can produce more marked exophthalmos in the prone or supine position, or when performing the Valsalva maneuver. CT may show intralesional calcifications with moderate enhancement, while magnetic resonance imaging (MRI) shows an isointense signal in T1-weighted images or a hyperintense signal in T2weighted images. The angiographic blush is moderate, but is intense in high-flow vascular malformations, which produce a strong signal on T2-weighted MRI, with significant enhancement in both CT and MRI. High-flow vascular malformations may also be associated with a volumetric increase of the ophthalmic vein and mimic the presence of a carotid-cavernous fistula. Lymphangiomas, which are more common in younger age groups, may be intraconal or extraconal and produce bone changes. On MRI they appear hypointense on T1 and markedly hyperintense on T2; on CT they are isodense to muscle and can have variable enhancement and fluid-fluid levels related to old hemorrhages. Hemangiopericytomas are a form of vascular tumors or, more accurately, sarcoma; 50% of cases are malignant, and there is a tendency for recurrence.1-3 Although approximately 25% of hemangiopericytomas are located in the head and neck, they represent only a small percentage of the tumors found in this area. They are rare in the orbit and are located more frequently in the retrobulbar area, causing exophthalmos. They often show marked enhancement on CT and with bone invasion, while the MRI signal characteristics are atypical, as they cannot be differentiated from meningiomas or cavernous hemangiomas. Angiography often shows an intense pathologic blush. Lymphomas may occur either as isolated lesions or as part of a systemic lymphoma. There are benign forms and pseudolymphomas, which are difficult to differentiate from pseudotumors and malignant forms. They are often retrobulbar or located in the lacrimal gland. The CT and MRI signs are often nonspecific. CT shows hyperdense pathologic tissue with distinct edges in the anterior part of the orbit, or in the lacrimal gland, which blends with the surround-
A 44-year-old man presented to our department for a swelling approximately 1.5 cm in diameter located in the medial aspect of the left upper eyelid. The swelling was painless, elastic in consistency, slightly pulsatile, and slow growing (Fig 1). Present for approximately 1 year, the lesion had produced ingravescent blepharoptosis. Clinical examination showed normal visual acuity and preserved ocular motility. Based on an ultrasound carried out elsewhere, a vascular lesion was suspected. A computer tomography (CT) scan with contrast medium was carried out, which confirmed the presence of a well-defined lesion below the orbicularis muscle of the left upper eyelid with distinct contrast and no bone structure involvement (Fig 2). Therefore, preoperative angiography was performed, which confirmed that the eyelid lesion was vascularized by branches of the ophthalmic artery, and a medium-flow vascular malformation was diagnosed (Fig 3). Given the risks associated with embolization of the ophthalmic artery branches and the surgically accessible location of the lesion, it was decided not to proceed with embolization.
Differential Diagnosis Dino Daniele, MD When a patient presents with a slow-growing, painless swelling in the upper eyelid and the slightest pulsatile of the mass is perceived, a vascular lesion is often suspected, particularly when supported by an angiography. However, in the radiological differential diagnosis of an adult orbital lesion using angiographic blush and CT enhancement, certain benign and malignant pathologies need to be considered, including vascular malformations, hemangiopericytomas, lymphomas, neural sheath tumors, fibrous tumors of the orbit, rhabdomyosarcomas, meningiomas, and orbital metastases. Received from San Giovanni Battista Hospital, Turin, Italy. *Assistant Professor, Division of Maxillofacial Surgery. †Professor, Division of Maxillofacial Surgery. ‡Resident, Division of Maxillofacial Surgery. §Assistant Professor, Neuroscience Department. Address correspondence and reprint requests to Dr Roccia: Division of Maxillofacial Surgery, San Giovanni Battista Hospital, Via Febo 19, 10133 Torino, Italy; e-mail: [email protected] © 2006 American Association of Oral and Maxillofacial Surgeons
0278-2391/06/6406-0012$32.00/0 doi:10.1016/j.joms.2006.02.016
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FIGURE 2. Axial computer tomography scan showing well-circumscribed mass of the upper eyelid without any calcification or bony structure involvement. Roccia et al. Enlarging Lesion of the Upper Eyelid. J Oral Maxillofac Surg 2006.
FIGURE 1. Preoperative case showing left blepharoptosis. Roccia et al. Enlarging Lesion of the Upper Eyelid. J Oral Maxillofac Surg 2006.
ing tissue and rarely erodes bone, with weak enhancement. On MRI, the signal is medium or low on T1weighted images and isointense to fat on T2 images. Of the neural sheath tumors, neurofibromas and schwannomas should be considered. Neurofibromas may be plexiform, diffuse, or localized and can involve the upper eyelid or be located in the retrobulbar area. On CT and MRI there is marked enhancement. The radiological appearance on MRI is atypical and features a low signal on T1 and a high signal on T2. Schwannomas typically originate in the retrobulbar area, with CT and MRI features that are similar to neurofibromas. Angiography rarely produces a pathologic blush. Fibrous histiocytomas, fibromas, solitary fibrous tumors, fibrosarcomas, and nodular fasciitis are all fibrous tumors of the orbit. Fibrous histiocytomas are the most frequent mesenchymal tumors of the orbit. They are benign in 63% of cases, locally aggressive in 26% of cases, and malignant in 11% of cases.4 They appear as a well-defined intraconal or extraconal mass in the medial or upper part of the orbit. The most common symptoms include proptosis, decreased vision, diplopia and pain. CT shows a mass with moderate to intense enhance-
ment; sometimes there is no contrast in the central area owing to tissue necrosis. Malignant forms may cause bone erosion. On MRI, fibrous histiocytomas show an intense signal on T2, but they are difficult to differentiate from neurofibromas or other fibrous lesions of the orbit. Fibromas are the rarest fibrous tumors of the orbit and typically occur in young adults. They can be solitary, multicentric, or generalized. They originate
FIGURE 3. Lateral projection arteriogram showing the feeder vessel to the orbital lesion. Roccia et al. Enlarging Lesion of the Upper Eyelid. J Oral Maxillofac Surg 2006.
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from Tenon’s capsule or the fascia of the extrinsic ocular muscles, but can also involve the eyelid. On CT, fibromas appear as a well-defined round or oval homogeneous high-density mass that clearly stands out from the surrounding structures and is similar to cavernous hemangiomas. MRI features are nonspecific and are associated with the benign or invasive nature of the tumor. Solitary fibrous tumors are generally benign tumors of mesenchymal origin. They can be located anywhere in the orbit and may cause proptosis. CT shows a mass with well-defined edges and possible bone changes typical of slow-developing tumors, with inhomogeneous enhancement. MRI shows a parenchymal isointense signal on T1 images and inhomogeneous iso/hypointensity on T2 images (a feature that differentiates them from other fibrous tumors); enhancement is homogeneous, as is the blush that is observed in the angiography arterial phase. The known forms of fibrosarcoma include congenital, infantile, juvenile, and adult form. Adult fibrosarcomas can be well delimited or infiltrative and cause nerve and functional deficits. CT and MRI images are nonspecific for orbital fibrosarcomas, as they usually show an orbital infiltrating mass. MRI shows a welldemarcated mass on T1 and T2 images. Nodular fasciitis is a fibrous tumor that is common in the limbs and torso in adults, but involvement of the head and neck is more commonly observed in pediatric patients. Orbital involvement affects mainly the eyelid, conjunctiva, and Tenon’s capsule. It may develop within weeks or months, mimicking a malignant lesion. The CT and MRI results are nonspecific and similar to those for other fibrous tumors of the orbit. Meningiomas, which are mainly endocranial and originate in the dura mater, may have extracranial extensions involving the face area, including the orbital region. Rarely (1%), extracranial meningiomas can occur mainly in the orbit, nasal sinuses, mouth, middle ear, cervical soft tissues, and mediastinum. On CT, they appear as a mass of parenchymatous density with moderate enhancement and produce bone changes. On MRI, the signal features are similar to those of the cerebral parenchyma. Angiography produces an intense and persistent blush. Of the malignant neoplasias, rhabdomyosarcomas commonly occur in children, but the pleomorphic form also occurs in adults. They occur anywhere in the orbit, primarily in the upper part. They cause proptosis and rapid-onset exophthalmos. On CT, they appear as a well-defined, isodense mass in muscle, with moderate to intense enhancement. Bone erosion may be observed with large rhabdomyosarcomas, whereas the CT results may appear nonhomogeneous after entoptic hemorrhages. On MRI, the signal is
FIGURE 4. Intraoperative appearance of the upper lid lesion. Roccia et al. Enlarging Lesion of the Upper Eyelid. J Oral Maxillofac Surg 2006.
isointense in the encephalic parenchyma on T1 images and hyperintense on T2 images, while the angiographic blush is intense. Lastly, metastases in the orbit are uncommon, but the incidence is increasing as a result of the improved survival of carcinoma patients. The primary tumor is most frequently in the breasts, followed by the lungs, prostate, and choroid. There are no specific symptoms with orbital metastases, as opposed to other orbital pathologies, but the most common are lid swelling, diplopia, red eye, decreased visual acuity, and proptosis. On CT and MRI, they appear as wellcircumscribed focal lesions in muscle, with possible bone involvement and sensitivity to contrast medium.
Subsequent Course Two days after angiography, the lesion on the patient’s eyelid was excised under general anesthesia. An incision was made along the upper palpebral sulcus to expose the lesion, which was located below the palpebral orbicularis muscle. The lesion was well encapsulated and violet in color, and was enucleated with minimal bleeding during the procedure (Fig 4). There were no postoperative complications, and the patient was discharged 2 days after the surgical procedure.
Pathologic Diagnosis The histological examination of the lesion confirmed the diagnosis of solitary fibrous tumor of the upper eyelid (Fig 5). Therefore, the patient had periodical follow-ups. Four years after the surgical procedure, no clinical or radiological recurrence of the lesion has been observed (Fig 6).
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FIGURE 5. Haematoxylin and eosin-stained tumor section (original magnification, ⫻100) showing storiform pattern of SFTs. Roccia et al. Enlarging Lesion of the Upper Eyelid. J Oral Maxillofac Surg 2006.
Discussion Solitary fibrous tumors (SFTs) are spindle-cell neoplasms (first described by Leutard and then classified by Klemperer and Rabin in 1931)5 that are generally located on serosal surfaces, particularly in the visceral pleura. Owing to their presumed mesothelial origin, they have also been called solitary fibrous mesotheliomas, although recent cell studies lean toward a mesenchymal origin.6 In addition to the well-documented pleural location, extrapleural locations have been reported during the last decade, less frequently in cervicocephalic locations, such as the nasal cavities and paranasal sinuses,6-8 orbit,6,8-41 oral cavity,42-47 parotid,48 temporal region,49 upper respiratory tract,50-52 and soft tissue of the scalp.53,54 Over the last 10 years, 59 cases of orbital SFTs have been described with a distinct prevalence in men (5:1), an age range of 9 to 76 years, and no predominance in either orbit. As with other extrapleural SFTs, orbital SFTs appear nonaggressive, although 9 recurrences attributable to incomplete surgical excision have been documented,9,20,26,36,37 as well as 1 case of malignant transformation.29 However, no local or remote metastases have been described. The slow development of the lesion often delays the initial clinical presentation, and consists mainly of a mass effect of the tumor with progressive asymptomatic unilateral proptosis. Visual disturbances, a palpable orbital mass, and reduced ocular motility are also described. In our case, the palpebral location with no orbital involvement only produced palpebral ptosis, with progressive limitation of the patient’s visual field. As in our case, a radiologist may not be able to provide an accurate diagnosis, which is usually obtained by microscopic examination and immunohistological testing of the excised tissue.
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Histologically, SFTs consist of a mass of spindle cells in a haphazard arrangement. The cells often adopt a storiform pattern, similar to fibrous histiocytoma. Mitotic figures are infrequent, and the count is greater than 4 per 10 high-power fields in association with cellular pleomorphism and nuclear atypia, while areas of focal necrosis are considered indicators of malignity.55 SFTs are highly vascularized, with many dilated vessels that have a stag-horn appearance similar to those observed in hemangiopericytomas.27 However, the final diagnosis of SFTs is based on their strong, diffuse immunoreactivity to the antigen CD34 and to vimentin, as well as their irregular positivity to actin, whereas the markers desmin and S-100 protein are not reactive. Above all, intense CD34 immunohistochemical staining helps differentiate SFTs from hemangiopericytomas (weak CD34 staining), fibrous histiocytomas, and fibrosarcomas (both CD34-negative).10 Schwannomas are positive for CD34 but also express S-100 protein.55 The immunoreactivity of CD34 has only recently been described in SFTs. Therefore, it is assumed that, before the discovery of CD34, or in cases with no
FIGURE 6. Postoperative case showing resolution of the left blepharoptosis. Roccia et al. Enlarging Lesion of the Upper Eyelid. J Oral Maxillofac Surg 2006.
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immunohistochemistry support, SFTs of the orbit were classified under other diagnostic categories, mainly as hemangiopericytomas or fibrous histiocytomas, which are the most common primary mesenchymal tumors of the orbit. This also explains the recent increase in SFT cases described in the literature. Furthermore, the CT and MRI results, as well as the clinical presentation, are rarely helpful in the differential diagnosis of other vascularized orbital masses, as was seen in our case. While SFTs of the pleura and mediastinum can appear aggressive, with invasion of the surrounding tissue, local recurrences, and remote metastases, SFTs in the orbit are usually benign and asymptomatic, and can be cured by complete excision. However, incomplete excision results in a higher percentage of recurrence and the possibility of malignant transformation. Recurring orbital tumors tend to spread locally and invade surrounding tissue, including the orbital bone. Therefore, prolonged follow-up is necessary, as recurrence may occur many years after the tumor has been excised.
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