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[心脏黏液瘤合并原发性结节性肾上腺皮质发育异常。这种罕见家族综合征的又一亲属关系病例报告]

[Combination of heart myxoma with primary nodular adrenal cortex dysplasia. Case report of a further kinship of this rare familial syndrome].

作者信息

Schuiki E, Hedinger C

出版信息

Schweiz Med Wochenschr. 1987 Apr 18;117(16):595-603.

PMID:3589617
Abstract

Another case of the rare syndrome of combined papillary cardiac myxoma and primary adrenocortical nodular dysplasia is reported. The lesions were discovered incidentally at autopsy in a 64-year-old woman who had died of cerebrovascular stroke. Further investigation of the family by echocardiography revealed a small left atrial tumor in the 33-year-old son of the patient. Pathological examination after surgical resection of the tumor confirmed the diagnosis of atrial myxoma. The patient also exhibited cutaneous nodules with histologic features of myxofibroma. Some morphological aspects of this peculiar familial syndrome are discussed, with special emphasis on the finding of follicular tumors of the thyroid possibly associated with this syndrome.

摘要

本文报告了另一例罕见的合并乳头状心脏黏液瘤和原发性肾上腺皮质结节性发育异常综合征的病例。病变是在一名死于脑血管中风的64岁女性尸检时偶然发现的。通过超声心动图对该家族进行进一步调查发现,患者33岁的儿子左心房有一个小肿瘤。肿瘤手术切除后的病理检查证实为心房黏液瘤。该患者还表现出具有黏液纤维瘤组织学特征的皮肤结节。本文讨论了这种特殊家族综合征的一些形态学方面,特别强调了可能与该综合征相关的甲状腺滤泡性肿瘤的发现。

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