Autosomal dominant familial angiolipomatosis clinically mimicking neurofibromatosis.

Neurofibromatosis Pub Date : 1989-01-01
J C Goodman, D S Baskin
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Abstract

The autosomal dominant form of familial angiolipomatosis may be mistaken for peripheral neurofibromatosis (NF-1) due to the similarity of the family history and the occurrence of multiple subcutaneous masses, but histopathological examination of the tumors readily distinguishes these two diseases. We report here a case of familial angiolipomatosis, which was initially though to be neurofibromatosis, and the occurrence in this patient of a granular cell tumor similar to such tumors occasionally seen in neurofibromatosis. A review of the literature discloses intriguing parallels between familial angiolipomatosis and neurofibromatosis, suggesting that similar pathogenetic mechanisms may operate in both diseases.

常染色体显性家族性血管脂肪瘤病,临床类似神经纤维瘤病。
家族性血管脂瘤病常染色体显性形式可能被误认为周围神经纤维瘤病(NF-1),由于家族史的相似性和多个皮下肿块的发生,但肿瘤的组织病理学检查很容易区分这两种疾病。我们在此报告一例家族性血管脂瘤病,最初被认为是神经纤维瘤病,在该患者中出现了类似于神经纤维瘤病的颗粒细胞肿瘤。一篇文献综述揭示了家族性血管脂肪瘤病和神经纤维瘤病之间有趣的相似之处,表明这两种疾病的发病机制可能相似。
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