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[I型多发性内分泌肿瘤中的胸腺类癌]

[Thymus carcinoid in multiple endocrine neoplasms type I].

作者信息

Zahner J, Borchard F, Schmitz U, Schneider W

机构信息

Klinik für Hämatologie, Onkologie und klinische Immunologie, Universität Düsseldorf.

出版信息

Dtsch Med Wochenschr. 1994 Feb 4;119(5):135-40. doi: 10.1055/s-2008-1058672.

Abstract

Thrombosis of the left subclavian vein occurred in a 44-year-old man. It was found to be caused by an atypical thymus carcinoid of the anterior mediastinum without carcinoid syndrome. Primary resection was not possible, but it was removed after three cycles of neoadjuvant chemotherapy with doxorubicin, cisplatin, vincristine and cyclophosphamide. Increased concentrations of alkaline phosphatase and parathormone were then noted. Subtotal parathyroidectomy revealed hyperplastic parathyroids. A gastrinoma was suspected from a history of peptic ulcer for many years which had persisted despite a Billroth II gastric resection 10 years ago. Serum gastrin, analysis of gastric secretion and a secretin-stimulating test confirmed the diagnosis. Recurrent episodes of weakness and syncope, in the presence of low blood sugar levels and a positive C-peptide suppression test, were interpreted as due to an insulinoma. There was no evidence of increased hypophyseal or adrenal function. Finally, in the absence of a family history, multiple endocrine neoplasia type 1 (MEN 1) was diagnosed with co-existing primary hyperparathyroidism, gastrinoma, insulinoma and thymus carcinoid. Somatostatin-receptor scintigraphy provided localization of the MEN 1 with enrichment in the thorax and abdomen.

摘要

一名44岁男性发生左锁骨下静脉血栓形成。发现其病因是前纵隔的非典型胸腺类癌,无类癌综合征。无法进行一期切除,但在接受阿霉素、顺铂、长春新碱和环磷酰胺三个周期的新辅助化疗后将其切除。随后发现碱性磷酸酶和甲状旁腺激素浓度升高。次全甲状旁腺切除术显示甲状旁腺增生。因有多年消化性溃疡病史,尽管10年前已行毕罗Ⅱ式胃切除术但仍持续存在,怀疑患有胃泌素瘤。血清胃泌素、胃液分泌分析及促胰液素刺激试验确诊了该诊断。在低血糖水平和C肽抑制试验阳性的情况下,反复出现的虚弱和晕厥发作被解释为由于胰岛素瘤所致。没有垂体或肾上腺功能亢进的证据。最后,在无家族史的情况下,诊断为1型多发性内分泌肿瘤(MEN 1),并存原发性甲状旁腺功能亢进、胃泌素瘤、胰岛素瘤和胸腺类癌。生长抑素受体闪烁扫描显示MEN 1在胸部和腹部有富集定位。

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