Neurofibromatosis type I and malignancy: review of 32 pediatric cases treated at a single institution.
Neurofibromatosis type I and malignancy: review of 32 pediatric cases treated at a single institution.
复制标题
I 型神经纤维瘤病和恶性肿瘤：对同一机构治疗的 32 例儿科病例进行回顾。
DOI：
10.1002/mpo.2950220203
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发表时间：
1994
期刊：
影响因子：
--
通讯作者：
Pratt,C
中科院分区：
文献类型：
--
作者：
Shearer,P;Parham,D;Kovnar,E;Kun,L;Rao,B;Lobe,T;Pratt,C
在圣裘德儿童研究医院接受治疗的6，678名儿童癌症患者中，发现了32例I型神经纤维瘤病（NF 1）。在这些患者中共诊断出35例恶性肿瘤。2/3例继发恶性肿瘤患者的原发或继发肿瘤为结肠癌。特别令人感兴趣的是两个病例，其中NF 1和恶性周围神经鞘瘤都存在于多个连续的世代中：一个患有结肠癌和非霍奇金淋巴瘤的患者，他有p53基因的体质异常，一个患有费城染色体和其他细胞遗传学异常的急性淋巴细胞白血病患者，包括t（8;14）。最大的亚组，恶性周围神经鞘肿瘤，仅对那些可切除的肢体病变的患者的预后有利。相比之下，所有中枢神经系统肿瘤患者均存活。这些病例反映了NF 1中可能存在的分子和细胞遗传学异常以及可能导致这些患者发生的各种肿瘤。© 1994 Wiley利斯公司
Thirty‐two cases of neurofibromatosis Type I (NF1) were identified among 6,678 pediatric cancer patients treated at St. Jude Children's Research Hospital over a 29‐year period. A total of 35 malignant neoplasms have been diagnosed in these patients. Two of three patients with second malignant neoplasms had colon cancer as the primary or second tumor. Of particular interest are two cases in which both NF1 and malignant peripheral nerve sheath tumors were present in multiple successive generations: a patient with colon cancer and non‐Hodgkin lymphoma who has a constitutional abnormality of the p53 gene, and a patient with acute lymphoblastic leukemia with the Philadelphia chromosome and other cytogenetic abnormalities, including the t(8;14). Outcome of patients in the largest subgroup, that of malignant peripheral nerve sheath tumors, was favorable only for those patients having resectable extremity lesions. In contrast, all patients with central nervous system tumors are surviving. These cases reflect the molecular and cytogenetic abnormalities that can be present in NF1 and the variety of tumors that may result in these patients. © 1994 Wiley‐Liss, Inc.