Sacral Primitive Neuroectodermal Tumor: Case Report and Review of the Literature

薦椎原始神經外胚層細胞瘤：病例報告及文獻回顧

洪吉志(Jyi-Jyh Hung)；何文祥(Wen-Shiang Ho)

中華放射線醫學雜誌

33卷4期（2008 / 12 / 01）

281 - 285

繁體中文

一位15歲的男孩表現出漸進式的腰痛、小腿麻木和神經性膀胱功能異常。手術前的磁振造影檢查發現了一個薦椎腫瘤，並侵犯到硬腦膜外、薦骨和薦骨前區域，因此緊急開刀將腫瘤切除。病理檢查結果發現是薦椎原始神經外胚層細胞瘤。這類腫瘤對化療和放射線治療只有部份病例有反應。英文期刊文獻至今只有少數病例被報告，而且此病例是非常少數的原始神經外胚層細胞瘤原發於薦椎被報告的病例。大多數原始神經外胚層細胞瘤患者存活期間少於2年。

Primary intraspinal primitive neuroectodermal tumors (PNETs) are rare. We report a case of sacral PNET with review the literature. A 15-year-old boy presented with progressive lumbar area pain, lower leg numbness and neurogenic bladder. Preoperative magnetic resonance imaging revealed a sacral tumor with epidural, presacral, and sacral bone extensions. An urgent operation was performed with gross tumor removal. The pathological findings were consistent with a PNET. This tumor is somewhat responsive to chemotherapy and radiotherapy thus far. A review of the English literatures showed that only some cases of primary intraspinal PNETs have been reported to date, and the present case is one of very rare cases reported in English literatures in which the tumors were of purely sacral origin sites. Most of the reported PNET patients survived less than 2 years. Primary intraspinal sacral PNETs are rare tumors and carry a poor prognosis.