Perivascular epithelioid cell tumour-mimicking retroperitoneal leiomyosarcoma

A young man in his 40s was evaluated in the emergency department for abdominal and right flank pain. A CT scan of the abdomen and pelvis showed a solid, well-circumscribed lesion measuring 7.1×8.1×5.4 cm, which was arising from the retroperitoneum and extending from the third portion of the duodenum towards the right kidney. A percutaneous core biopsy was obtained, demonstrating an atypical smooth muscle neoplasm suggestive of a low-grade leiomyosarcoma. The patient underwent surgery for an en-block resection of the mass and the final pathology confirmed a perivascular epithelioid cell neoplasm without significant pleomorphism, mitosis or necrosis. Our case adds to the small number of perivascular epithelioid cell tumour cases reported in the literature and we present it in order to increase our understanding of this tumour and to assist in its appropriate diagnosis and management.