A rare case report of orbital malignant solitary fibrous tumor


Article PDF :

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Article type :

Case Report

Author :

Priyam Gupta, Somanath N, Chinmayee Thrishulamurthy, Dayananda S Biligi

Volume :

5

Issue :

4

Abstract :

Purpose: Orbital Solitary Fibrous Tumor (SFT) is a rare spindle cell neoplasm of mesenchymal origin.1 We describe the clinical presentation, radiological features, pathological features of a 23yr old female patient with orbital malignant SFT. Observation: We report a case of Malignant Orbital Solitary Fibrous Tumor of the orbit in a 23yr old female who presented with painless unilateral proptosis of the left globe noticed by her in the last 1 month. Computed Tomography scan showed a well-defined homogeneous enhancing soft tissue density in superior and extraconal compartment, with no evidence of bony erosion or calcification. Enbloc tumor resection confirmed SFT based on histopathological and immunohistochemistry studies. Microscopic examination showed a highly cellular tumor, moderate amount of pleomorphism and increased mitosis (>4/10). Immunohistochemistry studies confirmed diagnosis as tumor was diffuse positivity with CD34, BcL2 positive, STAT6 positive with ki 67 high i.e. a 9-10% proliferative index in hot spot areas. Desmin, CD99, S100 and EMA was negative. Patient subsequently was also given External Beam Radiotherapy. Conclusion: Solitary Fibrous Tumors are rare tumor, which should be included in differential diagnosis of soft tissue tumor of the orbit. Combination of Computed Tomography scan, histological examination and immunohistochemical markers provide an accurate diagnosis. Complete surgical resection along with long term follow up is essential.

Keyword :

 Solitary fibrous tumor (SFT), Proptosis, Tumor.