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[卡尼三联征。关于一例复发性胃平滑肌肉瘤和双侧肺软骨瘤病例]

[Carney's triad. Apropos of a case of recurrent gastric leiomyosarcoma and bilateral pulmonary chondroma].

作者信息

Pignatelli V, Campani D, Grassi L, Pingitore R

机构信息

U.O. di Radiodiagnostica dell'USSL, Università, Pisa.

出版信息

Radiol Med. 1988 Dec;76(6):577-9.

PMID:2850598
Abstract

Carney's triad is a rare syndrome characterized by gastric leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma. Twenty-seven cases are reported in literature: the most commonly observed neoplasm is gastric leiomyosarcoma (26/27) followed by lung tumor (22/27), and gastric paraganglioma (15/27). Three patients bearing a gastric leiomyosarcoma developed liver metastases, and 6 had a local recurrence after some years. The occurrence of paraganglioma makes the prognosis worse: 4 deaths from local infiltration, metastases or severe hypertension. The triad only occurred in 9/27 cases, mostly young female patients. A common embryologic origin is difficult to assess. The case of a young female patient with recurrent gastric leiomyosarcoma (12 years later) and bilateral pulmonary chondroma is described. It is important that patients with any of the three tumors be periodically examined.

摘要

卡尼三联征是一种罕见的综合征,其特征为胃平滑肌肉瘤、功能性肾上腺外副神经节瘤和肺软骨瘤。文献报道了27例病例:最常观察到的肿瘤是胃平滑肌肉瘤(26/27),其次是肺部肿瘤(22/27)和胃副神经节瘤(15/27)。3例患有胃平滑肌肉瘤的患者发生了肝转移,6例在数年后出现局部复发。副神经节瘤的出现使预后更差:4例死于局部浸润、转移或严重高血压。三联征仅在9/27的病例中出现,大多为年轻女性患者。难以评估其共同的胚胎学起源。本文描述了一名年轻女性患者的病例,该患者复发性胃平滑肌肉瘤(12年后)并伴有双侧肺软骨瘤。对于患有这三种肿瘤中任何一种的患者进行定期检查很重要。

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