Peripheral Primitive Neuroectodermal Tumor of Uterus: A Case Report

Abstract

Primitive neuroectodermal tumors (PNETs) were first described in 1973 as a group of small round cell tumors that appeared to have developed from neuroectodermal cells. There are two types of PNETs—central and peripheral. Rarely they can present with female genital tract primary, most common being ovary followed by uterus. PNETs of the uterus are rare and have been reported in about 65 cases in the English literature. Even rarer are peripheral PNETs. Hence, we report this case as our contribution to the existing pool of data. A 29-year-old parous lady had a history of undergoing TAH with RSO for heavy bleeding per vagina. Histopathology was reported to be low-grade endometrial stromal sarcoma. A month later, she presented to our institute with complaints of cough. On evaluation, she was found to have a solid cystic lesion in the pelvis, multiple lung lesions and omental deposits. In view of the unlikely correlation of the clinical picture with low-grade ESS, we further investigated and arrived at a diagnosis of uterine PNET. Following this, she received 6 cycles of chemotherapy with ifosfamide, cisplatin and etoposide. There was persistent lesion in peritoneal cavity. Second-line chemotherapy was advised, but unfortunately, she died of the disease about a year from her first presentation. In view of extreme rarity of this aggressive disease, very little is known about its management. Hence, this case is being presented for information and pertinent discussion in order to increase awareness and urge for the much needed consensus regarding its management.