European Journal of Radiology Extra 65 (2008) 25–28
Solitary fibrous tumors of the pelvis: Report of two cases with radio-pathological correlation Christine Jarlot a , Sophie Michalak c , V´eronique Verriele e , Catherine Ridereau-Zins a , Denis Chautard b , G´erard Lorimier d , Christophe Aub´e a,∗ a
Department of Diagnostic Radiology, University Hospital Angers, France b Department of Urology, University Hospital Angers, France c Pathology Unit, University Hospital Angers, France d Department of Surgery, Centre Paul Papin Angers, France e Pathology Unit, Centre Paul Papin Angers, France
Received 5 May 2007; received in revised form 2 November 2007; accepted 6 November 2007
Abstract Solitary fibrous tumors (SFT) are rare neoplasm usually arising from the pleura. The literature contains few reports on the radiologic findings of extrathoracic SFT. We present two cases of SFT of the pelvis and emphasize the diagnostic value of MRI and pathoanatomic features and necessity of long-term follow-up. © 2007 Elsevier Ireland Ltd. All rights reserved. Keywords: MRI; Extrathoracic solitary fibrous tumor; Peritoneal pelvic tumors; Immunohistochemistry
1. Introduction Solitary fibrous tumors (SFT) are rare neoplasms of mesenchymal origin, which occur in equal proportions in men and women [1,2], between the ages of 40 and 70 years. These tumors were initially described in the thorax as arising from the pleura [3,4,5] but some extrathoracic SFT localisations have been reported in the orbit, meninges, thyroid, kidney, liver, stomach, peritoneum and retro peritoneum [6]. We report two cases of SFT of the pelvic space, which, according to the literature is an unusual location. 2. Observations 2.1. Case no. 1 A 71-year-old man was admitted to our institution for acute urinary retention. He had a history of progressive right sciatica over a 1-year period. Biological data were unremarkable. ∗ Corresponding author at: University Hospital of Angers, Department of Radiology, 4 rue Larrey, 49933 Angers, France. Tel.: +33 2 41 35 42 81; fax: +33 2 41 35 42 79. E-mail address:
[email protected] (C. Aub´e).
1571-4675/$ – see front matter © 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ejrex.2007.11.002
A hypertrophic prostate gland was identified by digital rectal examination. During trans-urethral resection of the prostate, examination under anesthesia showed a tumoral mass adjacent to and posterior to the rectum. A pelvic MRI demonstrated a well-circumscribed mass, with heterogeneous high-signal intensity on T2-weighted images (Fig. 1a and b) and homogeneous intermediate signal intensity on T1-weighted images. Areas of flow void were seen in the peripheral part of the tumor on T2-weighted images (Fig. 1d). After gadolinium injection there was intensive enhancement of the lesion, but the center remained unenhanced with an intermediate intensity on T1-weighted images (Fig. 1c). The MRI in and out phase sequences did not show any fat composition. The lesion appeared well distinct from the bladder, prostate gland, seminal vesicles, rectum and ischial fossa with a development above the levator ani muscle (Fig. 1d). No metastatic lymphadenopathy or local extension was found in the pelvis. The diagnosis of a solitary fibrous tumor of the pelvis was suggested considering the well-circumscribed form and hypervascular aspect of the tumor which remained independent of the pelvic organs. A thoraco–abdomino–pelvic scan did not identify any pulmonary, hepatic, renal or bone lesions. Surgical resection was difficult because of hemorrhage. The tumor was free of adherences or loco-regional extension.
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Fig. 1. Case no. 1: 71-year-old man with pelvic mass causing acute urinary retention. MRI: (a) axial T2-weighted image, (b) sagittal T2-weighted image, (c) axial contrast enhanced T1-weighted image, (d) coronal T2-weighted image. Well-circumscribed mass with heterogeneous high intensity signal on T2-weighted images. The mass is independent of pelvis organs, separate from bladder and rectum. It is surrounded by a fibrous pseudo-capsule with low intensity signal (arrows). The center has a high intensity signal on T2-weighted images and intermediate signal intensity on T1-weighted images, without enhancement. This area corresponds to the hypocellular myxoid area. The peripheral area of the tumor, hypercellular, shows intense enhancement with blood vessels responsible for flow void phenomena (arrow).
On gross pathology, the mass was well-circumscribed, sometimes surrounded by a fibrous pseudocapsule (Fig. 2a) (well identified on MRI (Fig. 1)), and sometimes directly in contact with pelvic fat. The cut surface was white–yellow–tan, with fleshy territories, myxoid microcystic territories, and fibrous territories (Fig. 2b). Histopathologically, spindle tumor cells were observed with hypocellular areas containing abundant myxoid stroma and hypercellular areas associated with small-dilated vessels with hemangiopericytoma-like features (Fig. 2c). Mitoses were rare.
Immunohistochemistry showed that the mass was strongly positive for CD34 and Bcl2, confirming the diagnosis of an SFT (Fig. 2d). There was no local recurrence or metastasis during the 15month follow-up. 2.2. Case no. 2 A 51-year-old man had a medical history of widespread dorsolumbar pain that had been present for at least 6 months. A CT scan was performed, showing a hypervascularized pelvic mass,
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Fig. 2. Case no. 1: (a) gross pathology showing a well-circumscribed tumor smooth and hemorrhagic (b) in the cut section, tumor showing some fleshy territories (black arrow), myxoid cystic territories (white arrow) and fibrous territories (yellow arrow), (c) microscopic examination showing spindle cell cellularity (white arrow) and hypocellular territories with abundant myxoid stroma (black arrow) with vascular hemangiopericytoma like dilatation (hematoxylin–phloxin–safran, G100×), (d) immunohistochemistry: strong positivity of cytoplasm with anti-CD34 (G200×).
14 cm in diameter, behind the bladder and displacing the rectum laterally. As in the first case, pelvic MR examination showed a mass with heterogeneous signal intensity on T2-weighted images with flow void phenomena and intermediate signal intensity on T1weighted images. There was intensive heterogeneous enhancement except in the center with high intensity signal on T2weighted images. It seemed to be independent of other pelvic organs. Pre-operative arterial embolisation of the mass was performed to limit hemorrhagic risk. A surgical resection was successfully performed. Gross pathology showed that the mass was well surrounded by a fibrous capsule. At histology, there was a high cellular area of spindle cells without cytonuclear atypia and area of oedema or fibrosis and myxoid stroma with small, hemangiopericytoma-like vessels. Immunohistochemistry also showed strong positivity for CD34 and Bcl2. Ac AE1, AE3, EMA, CD117 were negative. There was no increase in mitotic activity. The surgical margin was not invaded. The 4-month follow-up did not show any local recurrence or metastasis.
3. Discussion SFT were first described by Klemper and Rabin in 1931 as arising from the pleura. It was previously thought to represent a benign fibrous mesothelioma [5]. However, recent studies have shown that they most likely develop from submesothelial cells [3,4]. SFT seem to be a result of pluripotential primitive mesenchymal cells and thus this tumor can potentially develop in any organ with mesenchymal tissue. At histopathology, SFT show a patternless architecture characterized by a combination of alternating hypocellular and hypercellular areas. It contains thin-walled branching vessels, overlapping morphologically with haemangiopericytoma [7]. Approximately, one third of SFT are extrathoracic [1]. Morphologically, extrapleural solitary fibrous tumors resemble pleural SFT [7]. Myxoid changes, areas of fibrosis and interstitial mast cells are commonly observed. [7]. The histological variability of SFT can make the differential diagnosis difficult from other benign or malignant soft tissue tumors. Immunohistochemistry is very helpful in identifying this entity, showing a strong positivity for CD34 [3]. Most differential diagnoses are CD34 negative [5].
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Most SFT are histologically benign, but approximately 20% may be malignant [1], characterized by high cellularity, cellular pleomorphisms, atypia, high mitotic count, areas of necrosis, invasion of surgical margins [5]. Malignant SFT can occur de novo or because of dedifferentiation [1]. The clinical evolution cannot be predicted by histological examination alone [6]. The most important risk for recurrence is secondary to invaded surgical margins which are statistically more frequent in extrathoracic locations. On the other hand, the risk of metastases is not different between thoracic and extrathoracic locations [8]. Long-term monitoring is necessary to identify recurrence and metastases. Although SFT are often asymptomatic, there may be signs and symptoms from tumor compression. The paraneoplastic syndrome with hypoglycemia by insulin secretion has also been described [6]. MRI is probably the technique of choice to study pelvic SFT, because of its high soft-tissue contrast resolution and its ability to study the anatomical relationship of the visceral pelvic organs [9]. As described in the thoracic and extrathoracic cases of SFT in the literature [10,11], MRI shows a well-circumscribed tumor with heterogeneous high signal intensity on T2-weighted images and intermediate signal intensity on T1-weighted images, with contrast enhancement. In our patients, the center of the tumor was unenhanced, corresponding to the hypocellular area, and myxoid in the first case. SFT are hypervascular, because of the prominent haemangiopericytoma-like branching vascular pattern [7], as seen by the significant and early contrast enhancement in contrast sequences. This hypervascularization is also shown by blood vessels with flow void on T2-weighted images [6]. The absence of a fat component excludes the differential diagnosis of lipoma, liposarcoma, myelolipoma and angiomyolipoma. The challenge is the differential diagnosis with a malignant tumor with a myxoid component such as myxoid fibrosarcoma, myxoid liposarcoma and fibrous myxoid histiocytoma. These tumors present a delayed contrast enhancement and there is no “flow void” phenomena due to high blood flow through the SFT. Considering the risk of metastases and local recurrence of these tumors, the main treatment is complete surgical resection. Hypervascular features of the tumor may justify preoperative
embolisation to limit hemorrhage as illustrated in case no. 2. Thoraco–abdomino–pelvic CT scan is the best technique for long-term follow-up of tumors of intermediate malignancy. 4. Conclusion Diagnosis of pelvic SFT is usually fortuitous. MRI show a well-circumscribed tumor, distinct from pelvic organs, with heterogeneous high signal intensity on T2-weighted images, a hypervascular area with flow void phenomena. MRI features allow to include SFT into the differential diagnosis of soft tissue tumor in the pelvis. Diagnosis is confirmed by strong positivity for CD34 on immunohistochemistry. Although morphological and pathoanatomic features may be benign, long-term follow-up is required for all SFT, because of their unpredictable evolution. References [1] Vossough A, Torigian DA, Zhang PJ, Siegelman ES, Banner MP. Extrathoracic solitary fibrous tumor of the pelvic peritoneum with central malignant degeneration on CT and MRI. J Magn Reson Imaging 2005;22:684–6. [2] Kinoshita T, Ishii K, Higashiiwai H, Naganuma H. Malignant solitary fibrous tumor of the peritoneum. Clin Radiol 2000;55(2):157–60. [3] Berzal-Cantalejo F, Montesinos-Carbonell M, Montesinos-Carbonell ML, Calabuig-Crespo C, Martorell-Cebollada MA. Solitary fibrous tumor arising in the fallopian tube. Gynecol Oncol 2005:880–2. [4] Trastour C, Bafghi A, Checchi-Guichard C, Bongain A. Solitary fibrous tumor of the pelvis. Eur J Obstet Gynecol 2005;124(2):254–5. [5] Gonz´alez-Garc´ıa R, Gil-D´ıez Usandizaga JL, Hyun Nam S, Rodr´ıguez Campo FJ, Naval-G´ıas L. Solitary fibrous tumour of the oral cavity with histogical features of aggressiveness. Br J oral Maxillofac Surg; 2005. [6] Hasegawa T, Matsuno Y, Shimoda T, Hasegawa F, Sano T, Hirohashi S. Extrathoracic solitary fibrous tumors: their histological varibility and potentially agressive behavior. Human pathol 1999;30(12):1464–73. [7] Fletcher CDM, Unni K, Mertens K. WHO classification of tumours. Pathology and genetics of tumours of soft tissue and bone. WHO; 2002. pp. 86–89. [8] Gold JS, Antonescu CR, Hajdu C, et al. Clinicopathologic correlates of solitary fibrous tumors. Cancer 2002;94:1057–68. [9] Paramasivam S, Proietto A, Puvaneswary M. Pelvic anatomy and MRI. Best Pract Res Clin Obstet Gynaecol 2006;20(1):3–22. [10] Lehmann C, Mourra N, Tubiana J-M, Arriv´e L. Tumeur fibreuse solitaire du foie. J Radiol 2006;87:139–42. [11] Lary A, Robinson. Solitary fibrous tumor of the pleura. Cancer Control 2006;13(4):264–9. October.