原发性心脏嗜铬细胞瘤伴多发性内分泌肿瘤。
Primary cardiac pheochromocytoma with multiple endocrine neoplasia.
机构信息
Department of Cardiac surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, 100730, China.
出版信息
J Cancer Res Clin Oncol. 2011 Sep;137(9):1289-91. doi: 10.1007/s00432-011-0985-1. Epub 2011 Jun 26.
PURPOSE
Cardiac pheochromocytoma with multiple endocrine neoplasia syndrome (MENS) is rare. We present a rare case of concurrent cardiac pheochromocytoma and pituitary adenoma, a rare variant of the MEN syndromes and a review of the literature with special emphasis on diagnosis and treatment.
METHODS
Different from the single MENS type I or type II, Variant MENS' symptoms and signs are so nontypical that it is easy to make a misdiagnosis or missed diagnosis. One patient with variant MENS was treated surgically and relevant case data were collected.
RESULTS
The perioperative course was uneventful. At 24-month follow-up, catecholamine levels returned to normal and no symptoms of pheochromocytoma was found.
CONCLUSIONS
Cardiac pheochromocytoma with Multiple Endocrine Neoplasia can be treated by operation with good prognosis. Surgical removal to provide relief or effective control of symptoms is the treatment of choice.
目的
心脏嗜铬细胞瘤伴多发性内分泌肿瘤综合征（MENS）较为罕见。我们报告了一例同时患有心脏嗜铬细胞瘤和垂体腺瘤的罕见病例，这是 MEN 综合征的罕见变异，并对其进行了文献复习，特别强调了其诊断和治疗。
方法
与单一的 MENS Ⅰ型或Ⅱ型不同，变异型 MENS 的症状和体征不典型，容易误诊或漏诊。对 1 例变异型 MENS 患者进行了手术治疗，并收集了相关的病例资料。
结果
围手术期无并发症。24 个月随访时，儿茶酚胺水平恢复正常，未发现嗜铬细胞瘤的症状。
结论
心脏嗜铬细胞瘤伴多发性内分泌肿瘤可以通过手术治疗，预后良好。手术切除是缓解或有效控制症状的首选治疗方法。
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