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1型多发性内分泌肿瘤伴脊髓室管膜瘤

Multiple endocrine neoplasia type 1 associated with spinal ependymoma.

作者信息

Kato H, Uchimura I, Morohoshi M, Fujisawa K, Kobayashi Y, Numano F, Goseki N, Endo M, Tamura A, Nagashima C

机构信息

Third Department of Internal Medicine, School of Medicine, Tokyo Medical.

出版信息

Intern Med. 1996 Apr;35(4):285-9. doi: 10.2169/internalmedicine.35.285.

Abstract

A 51-year-old man was hospitalized with a gait disturbance and hypoesthesia below the level of his chest. These symptoms were due to a spinal tumor which was surgically resected and identified as an ependymoma. Additionally, the patient had hypercalcemia and a family history of insulinoma. An endocrine evaluation revealed parathyroid hyperplasia and a pancreatic islet cell tumor. Magnetic resonance imaging disclosed a pituitary microadenoma. He was diagnosed with spinal ependymoma and multiple endocrine neoplasia type 1 (MEN 1). A review of the literature revealed that chromosome 11q13 abnormalities have been reported in both ependymoma and MEN 1. We discuss the pathogenesis of these diseases.

摘要

一名51岁男性因步态障碍和胸部以下感觉减退入院。这些症状是由一个脊髓肿瘤引起的,该肿瘤经手术切除后被鉴定为室管膜瘤。此外,该患者有高钙血症和胰岛素瘤家族史。内分泌评估显示甲状旁腺增生和胰腺胰岛细胞瘤。磁共振成像显示垂体微腺瘤。他被诊断为脊髓室管膜瘤和1型多发性内分泌肿瘤(MEN 1)。文献回顾显示,室管膜瘤和MEN 1均有11q13染色体异常的报道。我们讨论了这些疾病的发病机制。

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