[与神经纤维瘤病1型相关的神经肉瘤。附病例报告及文献复习]
[Neurosarcoma associated with neurofibromatosis 1. Apropos of a case and review of the literature].
作者信息
Ligneau B, Freyer G, Giraud S, Isaac S, Bombaron P, Espinouse D, Plauchu H, Fischer G, Gérard J P, Lenoir G, Trillet-Lenoir V
机构信息
Service de Radiothérapie-Oncologie, Centre Hospitalier Lyon-Sud, Pierre-Bénite.
出版信息
Presse Med. 1997 Mar 29;26(10):458-63.
BACKGROUND
Type 1 neurofibromatosis considerably increases the risk of cancer development, particularly neurosarcoma. We report a case in a patient with chemosensitive metastatic neurosarcoma.
CASE REPORT
A young female patient with familial type 1 neurofibromatosis developed pleural metastasis of a neurosarcoma located on the arm. This tumor was initially highly sensitive to chemotherapy, but relapse occurred.
DISCUSSION
Follow-up in the order members of the family was particularly difficult to organize. One sister developed cerebral astrocytoma. Neurosarcomas develop earlier in patients with type 1 neurofibromatosis, worsening prognosis. We suggest a prospective and structured registration of such cases using a network of clinicians and pathologists in order to improve management schemes.
背景
1型神经纤维瘤病显著增加了癌症发生风险，尤其是神经肉瘤。我们报告了1例对化疗敏感的转移性神经肉瘤患者。
病例报告
1例患有家族性1型神经纤维瘤病的年轻女性患者发生了位于手臂的神经肉瘤胸膜转移。该肿瘤最初对化疗高度敏感，但出现了复发。
讨论
对该家族成员进行随访特别难以组织。1名姐妹患了脑星形细胞瘤。神经肉瘤在1型神经纤维瘤病患者中发病较早，预后较差。我们建议通过临床医生和病理学家网络对这类病例进行前瞻性和结构化登记，以改进管理方案。
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