Kawamoto E H, Weidner N, Agostini R M, Jaffe R
Cancer. 1987 May 15;59(10):1791-802. doi: 10.1002/1097-0142(19870515)59:10<1791::aid-cncr2820591018>3.0.co;2-#.
The clinical and pathologic findings of two patients with malignant ectomesenchymomas of soft tissue are reported. Malignant ectomesenchymomas are a composite of ganglion cells or neuroblasts and one or more malignant mesenchymal elements, usually rhabdomyosarcoma. Our first case was composed of ganglioneurosarcoma plus rhabdomyosarcoma, the second composed of neuroblastoma plus rhabdomyosarcoma. The name is derived from the suggestion that they arise from pluripotential ectomesenchyme. An English language literature search revealed 11 other cases that arose in soft tissue and had adequate clinicopathologic data. Of the 13 cases, 10 occurred in infants, three occurred in adults, and nine were males. Six patients were alive and free of disease at last follow-up (range, 0.6-12 years; mean, 3.4 years), four patients had died of tumor (within 0.5-3.3 years; mean, 1.3 years), one case had died of Adriamycin (doxorubicin) toxicity, and two cases had been lost to follow-up. Complete surgical resection is the mainstay of treatment and chemotherapy appears to be important.
报告了两例软组织恶性外胚层间叶瘤患者的临床和病理表现。恶性外胚层间叶瘤是神经节细胞或成神经细胞与一种或多种恶性间充质成分的复合体，通常为横纹肌肉瘤。我们的第一例由节细胞神经肉瘤加横纹肌肉瘤组成，第二例由神经母细胞瘤加横纹肌肉瘤组成。这个名称源于它们起源于多能外胚层间充质的推测。英文文献检索发现另外11例发生于软组织且有足够临床病理资料的病例。在这13例病例中，10例发生于婴儿，3例发生于成人，9例为男性。6例患者在最后一次随访时存活且无疾病（随访时间范围为0.6 - 12年；平均3.4年），4例患者死于肿瘤（在0.5 - 3.3年；平均1.3年），1例死于阿霉素（多柔比星）毒性，2例失访。完整的手术切除是主要治疗方法，化疗似乎也很重要。
