患有神经纤维瘤病的一个家族三代人中的肉瘤。

Sarcomas in three generations of a family with neurofibromatosis.

作者信息

Hartley A L, Birch J M, Kelsey A M, Harris M, Jones P H

机构信息

Department of Epidemiology and Social Oncology, Christie Hospital and Holt Radium Institute, Manchester, England.

出版信息

Cancer Genet Cytogenet. 1990 Apr;45(2):245-8. doi: 10.1016/0165-4608(90)90089-s.

DOI:10.1016/0165-4608(90)90089-s

PMID:2107963

Abstract

The development of sarcomas in three generations of a family with neurofibromatosis (NF) is described. A 7-month-old boy developed a prostatic rhabdomyosarcoma. Family history revealed that his maternal grandmother had died of a malignant peripheral nerve sheath tumor at age 37 years and had been affected with neurofibromatosis. His mother (aged 36 years) has also developed a malignant peripheral nerve sheath tumor and NF has been confirmed in both mother and son. This family illustrates a cluster of sarcomas associated with NF and shows the importance of thorough investigation of reported family illnesses.

摘要

本文描述了一个患有神经纤维瘤病（NF）的家族三代人中肉瘤的发生情况。一名7个月大的男孩患了前列腺横纹肌肉瘤。家族史显示，他的外祖母在37岁时死于恶性外周神经鞘瘤，且患有神经纤维瘤病。他的母亲（36岁）也患了恶性外周神经鞘瘤，母亲和儿子均已确诊患有神经纤维瘤病。这个家族说明了与神经纤维瘤病相关的一组肉瘤，并显示了对所报告的家族疾病进行全面调查的重要性。