Muhammad Zain Mehdi, Sajid Mushtaq, Noreen Akhtar, Usman Hassan, Asif Loya, Mudassar Hussain, Muhammad Azam.
Soft Tissue Tumours with Epithelioid Morphoogy.
J Rawal Med Coll Jan ;21(1):37-41.

Background: To study the morphological findings of soft tissue sarcomas with epithelioid morphology and their distribution with respect to the age, gender and location. Methods: In this descriptive study, soft tissue sarcomas (n=100) with epithelioid morphology were evaluated by two histopathologists Cinical and morphological features like age, gender, site and type of tumor were quantified and documented. Epithelioid Morphology was defined as cells with polygonal to polyhedral shape, abundant cytoplasm and round nuclei .All cases were diagnosed with help of a panel of immunohistochemical (IHC) stains, i.e., CK ,EMA, Desmin, S100, CD31, CD34, INI-1, CD99 and TFE3 in conjunction with clinical history and morphology. lCA and HMB45 IHC stains were also utilized to rule out the possibility of a lymphoproliferative disorder and melanoma. Results: Mean age of presentation was 36 years, with patients ranging in age from 1 to 80 years. There were n=37 females as compared to n=44 males. Most common site was thigh (n=21) followed by arm (n=13), head and neck region (n=5) and inguinal region (n=4).Epithelioid sarcoma (n=29) was the most common soft tissue sarcoma in our study followed by sclerosing epithelioid fibrosarcoma (n=10), alveolar soft part sarcoma (n=9), biphasic synovial sarcoma and epithelioid angiosarcoma (n=8), epithelioid malignant peripheral nerve sheath tumours (MPNST) (n=7), epithelioid hemangioendothelioma, undifferentiated epithelioid sarcoma, epithelioid angiomyolipoma and epithelioid leiomyosarcoma(n=6). rare tumors included in the study were 2 cases of epithelioid rhabdomyosarcoma and one case each of pseudomyogenic hemangioendothelioma, malignant rhabdoid tumor and dedifferentiated liposarcoma with rhabdoid features. Conclusion: Soft tissue sarcomas with epithelioid morphology require careful morphological and immunohistochemical evaluation to differentiate them from carcinomas, lymphomas and melanoma because of the differences in their clinical management and prognosis.

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