McCarthy P M, Piehler J M, Schaff H V, Pluth J R, Orszulak T A, Vidaillet H J, Carney J A
J Thorac Cardiovasc Surg. 1986 Mar;91(3):389-96.
We reviewed the Mayo Clinic records of 56 patients who underwent operation for cardiac myxoma and 29 cases in which cardiac myxoma was found at autopsy. Five patients had a "complex" of unusual findings including multiple pigmented skin lesions (lentiginosis), myxoid fibroadenomas of the breast, skin myxomas, and primary pigmented nodular adrenocortical disease (a cause of Cushing's syndrome). Four of these five patients had multiple cardiac myxomas. Three of the four patients who underwent surgical excision of the cardiac myxomas had recurrent myxomas (the only recurrences in our series), and one of these patients had a second recurrence. The occurrence of multiple and recurrent myxomas in patients with the complex was significantly (p less than 0.001) higher than in our 80 patients with sporadic myxomas. The world literature was searched for cases of cardiac myxomas with the unusual associations of the complex, and also for familial, multiple, and recurrent myxomas. A group of patients were identified who had unusual biologic behavior including early development of myxomas, atypical myxoma locations, and a high risk for the development of recurrent myxomas. For these patients, we recommend a thorough search for multiple tumors at operation, close postoperative follow-up, and careful screening of family members.
我们回顾了梅奥诊所56例接受心脏黏液瘤手术患者的记录以及29例尸检发现心脏黏液瘤的病例。5例患者有一组不寻常的表现，包括多发性色素沉着性皮肤病变（雀斑病）、乳腺黏液样纤维腺瘤、皮肤黏液瘤以及原发性色素沉着性结节性肾上腺皮质疾病（库欣综合征的病因之一）。这5例患者中有4例患有多发性心脏黏液瘤。4例接受心脏黏液瘤手术切除的患者中有3例出现复发性黏液瘤（是我们系列中仅有的复发病例），其中1例患者出现第二次复发。有该组表现的患者中多发性和复发性黏液瘤的发生率显著高于（p＜0.001）我们的80例散发性黏液瘤患者。检索世界文献以查找伴有该组不寻常关联的心脏黏液瘤病例，以及家族性、多发性和复发性黏液瘤病例。确定了一组具有不寻常生物学行为的患者，包括黏液瘤早期发生、非典型黏液瘤部位以及复发性黏液瘤发生的高风险。对于这些患者，我们建议在手术时彻底查找多发性肿瘤，术后密切随访，并仔细筛查家庭成员。
