Silliman C C, Mierau G W, Strain J D, White Y, McNeely L, Wilson H, McGavran L, Cullen J W
Department of Pediatrics, University of Colorado School of Medicine, Denver.
Am J Pediatr Hematol Oncol. 1993 Aug;15(3):299-305.
The purpose of this study was to determine the clinical outcome for pediatric patients with peripheral neuroepithelioma treated with combined modality therapy and followed long enough to account for late relapses.
Fifteen patients, ages 3 3/12 to 19 10/12 years, with peripheral neuroepithelioma (median follow-up 91 months) were diagnosed at The Children's Hospital, Denver, Colorado over the period 1980-1989. All of these malignancies originated in the soft tissues. A critical review of these cases was performed with particular consideration given to the site and stage of the tumor and to the radiographic findings at presentation. Thirteen patients had bulk (> 5 cm in the greatest dimension) or metastatic disease. Four patients had primary tumors involving the chest wall. All patients received chemotherapy, which included at least doxorubicin, vincristine, and cyclophosphamide. Definitive surgical resections were performed on 13 of 15 patients.
Five patients relapsed. Three were late relapses 24-44 months after diagnosis. Three of the five patients who relapsed had chest wall primaries. There were three deaths in this series due to peripheral neuroepithelioma and one due to sepsis. The overall survival was 68.5%, and the recurrence-free, survival 55.2%. Two patients with pulmonary relapses were treated with surgery and intensive chemotherapy and remain free of disease > 51 months following recurrence.
Combined treatment modalities appear to be important for optimal outcome. This series represents the first report of favorable outcome of peripheral neuroepithelioma using a series with follow-up that is long enough to account for late relapses.
本研究的目的是确定接受综合治疗且随访时间足够长以观察到晚期复发的小儿外周神经上皮瘤患者的临床结局。
1980年至1989年期间，科罗拉多州丹佛市儿童医院诊断出15例年龄在3岁3个月至19岁10个月之间的外周神经上皮瘤患者（中位随访时间91个月）。所有这些恶性肿瘤均起源于软组织。对这些病例进行了严格审查，特别考虑了肿瘤的部位和分期以及初诊时的影像学表现。13例患者有大块肿瘤（最大直径>5 cm）或转移性疾病。4例患者的原发肿瘤累及胸壁。所有患者均接受了化疗，化疗方案至少包括阿霉素、长春新碱和环磷酰胺。15例患者中有13例接受了根治性手术切除。
5例患者复发。3例为晚期复发，发生在诊断后24至44个月。复发的5例患者中有3例原发肿瘤位于胸壁。本系列中有3例因外周神经上皮瘤死亡，1例因败血症死亡。总生存率为68.5%，无复发生存率为55.2%。2例肺部复发患者接受了手术和强化化疗，复发后>51个月仍无疾病复发。
综合治疗方式似乎对获得最佳结局很重要。本系列是首次报道外周神经上皮瘤采用足够长时间随访以观察到晚期复发的系列研究后获得良好结局的情况。
