Peripheral Primitive Neuroectodermal Tumor of Uterus: A Case Report
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CASE REPORT
Peripheral Primitive Neuroectodermal Tumor of Uterus: A Case Report Rohini V. Kulkarni1 Sushil Kumar Giri1
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Bhagyalaxmi Nayak1 • Jagannath Mishra1 • Jita Parija1 • Surendranath Senapathy1
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Received: 31 May 2020 / Accepted: 29 June 2020 Ó Association of Gynecologic Oncologists of India 2020
Abstract Introduction 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. Objective 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. Case 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. Conclusion 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. Keywords PNET Indian study Uterine tumors Rare malignancy
Introduction 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 [1]. There are two types of PNETs depending on the location and cell of origin—central and peripheral. Central PNETs (cPNETs) are generally found in the CNS or spinal cord and arise from neural tube, while peripheral PNETs
& Rohini V. Kulkarni [email protected] Bhagyalaxmi Nayak [email protected] Jita Parija [email protected] 1
Department of Gynaecological Oncology, Acharya Harihar Regional Cancer Centre, Cuttack 753007, India
(pPNETs) arise from neural crest and are found involving the sympathetic nervous system, bone or soft tissues [2]. Peripheral PNETs show typical EWSR1 gene rearrangement, while central PNETs lack the EWSR1 rearrangement [3]. Most commonly, visceral PNETs as in uterine PNET lack this gene rearrangement and hence are categorized as central PNETs [3, 4]. PNETs of female genital tract origin are a rare occurrence by themselves, in that the comm
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