摘要
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 This study aimed to investigate the histopathological features and classification of primary cardiac neoplasms in Chinese children and adolescents in order to improve diagnosis and therapy of these rare diseases. Methods We collected and analyzed the pediatric cases （less than 18 years old,including all age groups of child ） from 562 patients, who suffered from primary neoplasm of heart and pericardium and underwent surgical resection in FW Hospital, the national Heart Center of China from 1976 to 2006. Their clinical records and tissue sections were reviewed. The histological types of tumor were established by routine light microscopy and by immunohistochemistry and electron microscopy in some cases. Results Fifty-two pediatric cases were found to have primary cardiac neoplasm. Their age ranged from O. 5 to 18 years （ mean age = 12 years） and 26 of them were female. There were 48 （93%） cases suffering with benign tumors. Myxoma was the most common histological type （31 cases ）. The other types were as follows： fibroma （4 cases ） , rhabdomyoma （3 cases ）, lymphoangioma （ 3 cases ）, hemangioma （ 2 cases ）, lipoma （ 2 cases ）, and neurofibroma, leiomyoma and granular cell tumor （ 1 case, respectively ）. Malignancy was diagnosed in 4 cases （7%） and consisted of hemoangiosarcoma, fibrosarcoma, myxofibrosarcoma and undifferentiated sarcoma. Among 9 infant cases less than 3 years old, the most common neoplasm was rhabdomyoma （3 cases ） and fibroma （3 cases ） , and we also found undifferentiated sarcoma （ 1 case）. About 93% of clinical diagnosis was completely or almost consistent with their pathological diagnosis while 7% cases were misdiagnosed clinically. Conclusion There are various histological types of primary cardiac tumors in children. The most common neoplasm is myxoma for eider kids and rhabdomyoma for infants. Malignant cardiac tumor is usually found in right side of heart and can involve some infant cases. Pathological examination is still critical in the differential diagnosis of tumors in spite of advanced medical imaging.
出处
《中国实用儿科杂志》
CSCD
北大核心
2008年第2期125-129,共5页
Chinese Journal of Practical Pediatrics
