Department of Pathology (Cardiovascular and Thoracic Division), Seth G. S. Medical College and K. E. M. Hospital, Mumbai, India.
Cardiovasc Pathol. 2012 May-Jun;21(3):180-7. doi: 10.1016/j.carpath.2011.07.001. Epub 2011 Aug 11.
Myxomas are the most common among the primary cardiac neoplasms, seen mainly in adult population, and are typically attached to the interatrial septum, on the left side. Myxomas arising from other sites are designated as "atypical myxomas." In this article, we describe the clinicopathologic features of 28 such lesions, resected in 20 patients.
A 15-year study (1995-2009) of all cardiac myxomas, received as surgical excisions in our institution, was performed. Atypical myxomas were selected on the basis of their atypical sites of origin, and a systematic review and comparison of their clinicopathologic features with all typical myxomas excised during the same period were done.
Among a total of 84 patients who had undergone cardiac myxoma excisions in this 15-year duration, 64 patients had typical myxomas, while atypical myxomas (30 tumors) were diagnosed in 20 patients (23.8%). None of them had a family history of similar symptoms. There were six children. In the atypical subset, there were 12 males and eight females; the mean age of diagnosis was 33.7 years. This demography differed from the typical myxoma group where there were more females than males and the mean age of diagnosis being 40.8 years. The symptoms of dyspnea, episodic chest pain, and palpitation were common in both cohorts of patients, and all showed a mass lesion with varying degrees of valvular regurgitation and obstruction on echocardiography. Five of the 20 patients with atypical myxomas had multifocal or multicentric tumors. Grossly, like typical myxomas, the atypical ones also exhibited solid and papillary patterns with the usual histological features. Four patients had recurrence of the disease.
Atypical myxomas are rare lesions having clinical and pathological features, not entirely different from those of typical myxomas. With the advent of modern diagnostics, it is now imperative to do genetic studies and screen the relatives of patients having atypical myxomas to rule out additional occult familial cases as they are now known to occur more in this "atypical" group.
黏液瘤是原发性心脏肿瘤中最常见的一种，主要发生在成年人群中，通常附着在房间隔上，位于左侧。起源于其他部位的黏液瘤被称为“非典型黏液瘤”。本文描述了在我们机构接受手术切除的 20 例患者的 28 例此类病变的临床病理特征。
对我院 15 年来（1995-2009 年）所有心脏黏液瘤手术切除标本进行了研究。根据其非典型起源部位选择非典型黏液瘤，并对其临床病理特征与同期切除的所有典型黏液瘤进行系统回顾和比较。
在这 15 年期间，共有 84 例患者接受了心脏黏液瘤切除术，其中 64 例为典型黏液瘤，20 例（23.8%）诊断为非典型黏液瘤（30 个肿瘤）。他们都没有类似症状的家族史。其中有 6 例为儿童。在非典型组中，有 12 名男性和 8 名女性；诊断时的平均年龄为 33.7 岁。这一人群构成与典型黏液瘤组不同，后者女性多于男性，诊断时的平均年龄为 40.8 岁。呼吸困难、间歇性胸痛和心悸是两组患者共同的症状，所有患者的超声心动图均显示有不同程度的肿块和瓣膜反流及梗阻。20 例非典型黏液瘤患者中有 5 例为多灶性或多中心肿瘤。大体上，与典型黏液瘤一样，非典型黏液瘤也表现出实性和乳头状形态，具有通常的组织学特征。4 例患者疾病复发。
非典型黏液瘤是罕见病变，其临床和病理特征与典型黏液瘤不完全不同。随着现代诊断技术的出现，现在必须进行基因研究，并对患有非典型黏液瘤的患者的亲属进行筛查，以排除其他隐匿性家族病例，因为现在已知它们在这个“非典型”组中更为常见。
