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砂粒样黑色素性神经鞘瘤:卡尼综合征的一种附加成分。病例报告。

Psammomatous melanotic schwannoma: an additional component of Carney's complex. Report of a case.

作者信息

Martin-Reay D G, Shattuck M C, Guthrie F W

机构信息

Department of Pathology, Evanston Hospital, Illinois 60201.

出版信息

Am J Clin Pathol. 1991 Apr;95(4):484-9. doi: 10.1093/ajcp/95.4.484.

DOI:10.1093/ajcp/95.4.484
PMID:2014773
Abstract

The authors report a case of Carney's complex (the complex of myxomas, spotty pigmentation, and endocrine overactivity) in a 32-year-old white man. Psammomatous melanotic schwannoma has recently been included as a component of this disorder. The complex was recognized after a psammomatous melanotic schwannoma was resected from the sacral canal. Subsequent echocardiography revealed ventricular cardiac myxomata. Resection of these lesions was successfully performed. Review of previous history and pathologic material further supported the diagnosis. The authors conclude that recognition of each component as part of the syndrome is of crucial importance, because it may prevent a fatal outcome. A family screening program should be performed in each case.

摘要

作者报告了一例32岁白人男性的卡尼综合征(黏液瘤、斑点状色素沉着和内分泌功能亢进综合征)。砂粒体性黑素性神经鞘瘤最近被纳入该疾病的组成部分。在从骶管切除砂粒体性黑素性神经鞘瘤后,该综合征得以确诊。随后的超声心动图显示心室心脏黏液瘤。成功切除了这些病变。回顾既往病史和病理资料进一步支持了诊断。作者得出结论,将每个组成部分识别为综合征的一部分至关重要,因为这可能预防致命后果。应对每例患者进行家族筛查。

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Psammomatous melanotic schwannoma: an additional component of Carney's complex. Report of a case.砂粒样黑色素性神经鞘瘤:卡尼综合征的一种附加成分。病例报告。
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Cleve Clin J Med. 1991 May-Jun;58(3):248-50, 255-6. doi: 10.3949/ccjm.58.3.248.

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