Epithelioid Angiosarcoma of Perineum with Lung and Bone Metastasis; A Case Report and Review of Literature

Epithelioid angiosarcoma (EA) is an extremely rare subtype of angiosarcoma, which is characterized by large cells with an epithelioid morphology. Epithelioid angiosarcoma is a rare malignancy of lymphatic or vascular endothelium[1]. That can arises from any part of body like vulva, peritoneum, ovary, uterus, spine, or bones, cutaneous etc. A 47 yrs old man presenting with complain of right inguinal swelling since one year, swelling was progressively increases in size. CEMRI(6/10/2018) Whole abdomen showed Evidence of multiloculated solid focal lesion of size 3.5x3.2 cm at perineum with right predominance with 2.0x2.0 nodular discrete lesion involving upper most right thigh. Initially biopsy was done on 23/1/2018, biopsy was suggestive of round cell soft tissue tumour probably Alveolar rhabdomyosarcoma showed vimentin, CD34, pan-CK, fli-1 positive. IHC confirmed diagnosis of epithelioid angiosarcoma of perineal regions. Contrast enhance computed tomography thorax was suggestive of lung and bony metastasis. This case highlights the difficulty of diagnosing EA, which requires careful pathological examination and immunophenotype labelling. At present, CD31 and CD34 are the most sensitive marker for detecting EA Keywords- Epithelioid angiosarcoma,immunohistochemistry, perineum, endothelium.