Ayoub Kusay, Niazi Ammar, Shebli Baraa, Batal Rand, Kozom Hamed, Ghabreau Lina, Mahli Nihad
Department of Surgery, Aleppo University Hospital, University of Aleppo, Syria.
Faculty of Medicine, University of Aleppo, Syria.
Ann Med Surg (Lond). 2020 May 16;55:93-96. doi: 10.1016/j.amsu.2020.05.010. eCollection 2020 Jul.
Peripheral Primitive NeuroEctodermal Tumors (pPNETs) are rare highly malignant tumors; originating from the neuroectoderm. Although PNETs may arise in various locations (most commonly in the extremities), very few cases have been reported in the pelvis. There is still poor evidence about the management of these tumors in the literature. We present a rare case of pelvic PNET in a 20-year-old male. The patient presented with symptoms mimicking a lumbar disk hernia, which delayed the diagnosis. He was managed with a combination of a debulking procedure, adjuvant chemotherapy, radiotherapy; and has been in remission for 2 years upon follow-up. This case highlights the importance of diagnosing such aggressive tumors as early as possible (as prognosis may vary significantly), and the challenge in the management of PNETs due to poor evidence.
外周原始神经外胚层肿瘤（pPNETs）是罕见的高度恶性肿瘤，起源于神经外胚层。尽管PNETs可能发生在各个部位（最常见于四肢），但盆腔发生的病例报道极少。文献中关于这些肿瘤的治疗仍缺乏充分证据。我们报告一例20岁男性盆腔PNET的罕见病例。患者表现出类似腰椎间盘突出症的症状，这延误了诊断。他接受了减瘤手术、辅助化疗、放疗联合治疗，随访2年病情缓解。该病例强调了尽早诊断此类侵袭性肿瘤的重要性（因为预后可能有显著差异），以及由于证据不足PNETs治疗面临的挑战。
