软组织周围神经上皮瘤。回顾性分析15例儿科患者。

C C Silliman, G W Mierau, J D Strain, Y White, L McNeely, H Wilson, L McGavran, J W Cullen
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引用次数: 0

摘要

目的:本研究的目的是确定接受联合治疗的小儿周围神经上皮瘤患者的临床结果,并随访足够长的时间以考虑晚期复发。患者和方法:1980-1989年期间,在科罗拉多州丹佛市儿童医院确诊的15例周围神经上皮瘤患者,年龄3 3/12至19 10/12岁(中位随访91个月)。所有这些恶性肿瘤都起源于软组织。对这些病例进行了严格的审查,特别考虑了肿瘤的部位和分期以及出现时的放射学表现。13例患者有肿块(最大尺寸> 5cm)或转移性疾病。4例患者原发肿瘤累及胸壁。所有患者均接受化疗,其中至少包括阿霉素、长春新碱和环磷酰胺。15例患者中有13例进行了最终手术切除。结果:5例患者复发。3例为诊断后24 ~ 44个月晚期复发。5例复发患者中有3例为胸壁原发。本系列病例中有3例死于周围神经上皮瘤,1例死于败血症。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手术和强化化疗治疗,复发后51个月无复发。结论:综合治疗方式似乎对最佳结果很重要。本系列报道首次报道了周围神经上皮瘤的良好预后,随访时间足够长,足以解释晚期复发。
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Peripheral neuroepithelioma of the soft tissues. A retrospective analysis of fifteen pediatric patients.

Purpose: 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.

Patients and methods: 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.

Results: 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.

Conclusions: 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.

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