Zimmermann T, Blütters-Sawatzki R, Berghäuser K H, Christiansen H, Padberg W
Klinik für Allgemein- und Thoraxchirurgie, Justus Liebig-Universität Giessen.
Chirurg. 1997 Jul;68(7):732-7. doi: 10.1007/s001040050262.
The primitive neuroectodermal tumor is a rare soft tissue neoplasm occurring in children and young adults. It derives from a carcinogeneic alteration of pluripotent neural crest cells, caused by a balanced reciprocal translocation t(11;22) (q24;q12). Treatment of this undifferentiated, extremely malignant small cell tumor is carried out in compliance with the soft tissue trail (CWS) from the German Society of Pediatric Oncology. Biopsy-proven diagnosis is followed by primary chemotherapy, which in 95% of cases leads to remission, allowing excision of the remainder of the tumor without mutilation and avoidance of intraoperative tumor cell dissemination. After excision, irradiation of the tumor site and two further sequences of chemotherapy are performed. If PNETs of the paravertebral region cause symptoms of paralysis and immediate surgery is required, postoperative chemotherapy, a second-look operation and irradiation are mandatory. Between 1986 and 1994, in cooperation with our pediatric and radiotherapy colleagues, we treated ten patients. In four patients (median age, 14 years) the PNET originated from the chest wall, in six patients from the paravertebral and retroperitoneal region. Five patients died after 20 months on average, while the remaining five patients are in full remission after 31, 46, 50, 51 and 91 months, respectively.
原始神经外胚层肿瘤是一种罕见的软组织肿瘤，好发于儿童和青年。它起源于多能神经嵴细胞的致癌性改变，由平衡易位t(11;22)(q24;q12)引起。这种未分化的、高度恶性的小细胞肿瘤的治疗遵循德国儿科肿瘤学会的软组织治疗方案（CWS）。经活检证实诊断后进行初始化疗，95%的病例化疗后可缓解，从而能够完整切除肿瘤残余部分并避免术中肿瘤细胞播散。切除后，对肿瘤部位进行放疗并再进行两个疗程的化疗。如果椎旁区域的原始神经外胚层肿瘤引起瘫痪症状且需要立即手术，则术后化疗、二次探查手术和放疗是必需的。1986年至1994年期间，我们与儿科和放疗科同事合作，共治疗了10例患者。4例患者（中位年龄14岁）的原始神经外胚层肿瘤起源于胸壁，6例起源于椎旁和腹膜后区域。5例患者平均20个月后死亡，其余5例患者分别在31、46、50、51和91个月后完全缓解。
