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十二指肠上皮样间质肉瘤、肺软骨瘤性错构瘤和无功能胰腺胰岛细胞瘤并存。是卡尼三联征的一种可能类似情况?

The concurrence of duodenal epithelioid stromal sarcoma, pulmonary chondromatous hamartoma, and nonfunctioning pancreatic islet cell tumor. A possible analogue of Carney's triad?

作者信息

Ngadiman S, Horenstein M G, Campbell W G

机构信息

Department of Pathology, New York Hospital-Cornell Medical Center, New York 10021.

出版信息

Arch Pathol Lab Med. 1994 Aug;118(8):840-3.

PMID:8060238
Abstract

A 63-year-old man with non-insulin-dependent diabetes mellitus and peripheral vascular disease presented with acute pulmonary edema and ascites. He died after a brief illness. Coincidence of duodenal epithelioid stromal sarcoma, pulmonary chondromatous hamartoma, and pancreatic islet cell tumor was found at autopsy. This rare concurrence of three uncommon neoplasms may be a pathogenetic variant or an analogue of Carney's triad in an elderly individual. Since all three tumors expressed markers for neural differentiation, the coincidence of tumors with these features may represent a form of neurocristopathy.

摘要

一名患有非胰岛素依赖型糖尿病和周围血管疾病的63岁男性,出现急性肺水肿和腹水。他在患病不久后死亡。尸检发现十二指肠上皮样间质肉瘤、肺软骨瘤性错构瘤和胰岛细胞瘤同时存在。这三种罕见肿瘤的罕见并发情况可能是一种发病机制变异,或是老年个体中卡尼三联征的类似情况。由于所有三种肿瘤均表达神经分化标志物,具有这些特征的肿瘤并发情况可能代表了一种神经嵴病形式。

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