A case of caecal “PECOMA”: An uncommon entity

INTRODUCTION AND IMPORTANCE: World Health Organization (WHO) defines PEComa as a mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. The symptoms and clinical signs of PEComa patients are nonspecific. Hence, diagnosis is usually difficult. Since it's a rare diagnosis, further research might help in understanding the disease better. CASE PRESENTATION: The patient in this study was an asymptomatic patient, who did colonoscopy as part of a regular check-up. A submucosal cecal tumor was detected in colonoscopy, and apart from that, all other investigative parameters were within normal limits. CLINICAL DISCUSSION: Laparoscopic Ileocecectomy was performed, and the histopathology report was suggestive of Pecomatosis (PEComa – Perivascular epithelioid cell tumor). The PEComas, neoplasms with perivascular epithelioid cell differentiation, are mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells (PEC). The characteristic features of PEC are the positivity of melanocytic markers and smooth muscle markers. CONCLUSION: Perivascular epithelioid tumors are mostly rare in the gastrointestinal tract, and even more unusual to be detected in Cecum. Surgery is the mainstay of the treatment, although, adjuvant therapy has been tried in recent times. The patients have to be kept in close follow-up, as there are reported cases of recurrences and distant metastasis.