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目的探讨儿童及青少年中罕见的原发性心脏肿瘤的组织学类型和病理特征,为临床诊断治疗提供形态学依据。方法1976—2006年从中国医学科学院阜外心血管病医院近30年手术切除的562例原发性心脏肿瘤患者中,选择年龄不超过18周岁的病例。分析其临床病理所见,部分病例做了免疫组织化学和透射电镜检查。结果52例中年龄平均12岁,最小6个月;男女各26例。良性肿瘤48例,占93%,其中黏液瘤最常见(31例),其它依次为纤维瘤(4例)、横纹肌瘤(3例)、海绵状淋巴管瘤(3例)、海绵状血管瘤(2例)、脂肪瘤(2例),还有颗粒细胞瘤、神经纤维瘤、平滑肌瘤各1例。恶性肿瘤4例,占7%,分别为血管肉瘤、纤维肉瘤、黏液纤维肉瘤及低分化肉瘤。3岁以内婴幼儿原发心脏肿瘤以横纹肌瘤(3例)和纤维瘤(3例)较多,还可见恶性的低分化肉瘤(1例)。52例临床诊断与病理诊断符合或基本符合的占93%,误诊7%。结论儿童心脏肿瘤类型多样,年龄较大者也以黏液瘤最多,而婴幼儿期以横纹肌瘤和纤维瘤多见。恶性肿瘤为分化较差的肉瘤,好发于右心,可见于婴幼儿。肿瘤临床诊断时应密切结合患儿年龄、瘤体生长部位及影像形态特点,才能减少误诊。
Objective To investigate the histological types and pathological features of rare primary cardiac tumors in children and adolescents and provide morphological evidence for clinical diagnosis and treatment. Methods From 1976 to 2006, 562 patients with primary cardiac tumors surgically resected from the Fuwai Hospital of Cardiovascular Diseases of Chinese Academy of Medical Sciences in the recent 30 years were enrolled in this study. Analysis of its clinical and pathological findings, some cases done immunohistochemistry and transmission electron microscopy. Results The average age of 52 patients was 12 years old, 6 months minimum; 26 men and women each. 48 cases of benign tumors, accounting for 93%, of which the most common myxoma (31 cases), the other followed by fibroids (4 cases), rhabdomyoma (3 cases), cavernous lymphangioma (3 cases), cavernous hemangiomas (2 cases), lipoma (2 cases), as well as granulosa cell tumor, neurofibromatosis and leiomyoma each in 1 case. Malignant tumors in 4 cases, accounting for 7%, respectively, angiosarcoma, fibrosarcoma, mucinous fibrosarcoma and poorly differentiated sarcoma. Primary cardiac tumors in infants and young children aged 3 years to rhabdomyoma (3 cases) and fibroids (3 cases) more, but also to see the poorly differentiated sarcoma (1 case). Among the 52 cases, 93% of the 52 cases were found to be coincident or substantially consistent with the pathological diagnosis and 7% were misdiagnosed. Conclusion There are many types of cardiac tumors in children, and the oldest is myxoma, while the most common in childhood and infant is rhabdomyoma and fibroids. Malignant tumor is poorly differentiated sarcoma, occurs in the right heart, seen in infants and young children. Tumor clinical diagnosis should be closely combined with children’s age, tumor growth site and image features to reduce misdiagnosis.