Fresneau B, Oberlin O, Brugières L, Valteau-Couanet D, Patte C
Service d'oncologie pédiatrique, institut Gustave-Roussy, rue Camille-Desmoulins, 94800 Villejuif, France.
Arch Pediatr. 2010 May;17(5):495-501. doi: 10.1016/j.arcped.2010.02.009. Epub 2010 Mar 24.
Primary heart tumors are uncommon in children. The majority of them are benign, with only 10% malignant. Among malignant cardiac tumors, sarcoma (rhabdomyosarcoma, angiosarcoma, synovial sarcoma) and lymphoma (Burkitt's lymphoma, large B-cell lymphoma, lymphoblastic lymphoma) predominate. There are few published pediatric series on malignant primary cardiac tumors. We report here 3 observations of primary malignant cardiac tumors, 2 cases of sarcoma (angiosarcoma and synovial sarcoma) and 1 case of Burkitt's lymphoma. A precise pathological diagnosis is necessary for the proper management of these patients. For sarcoma, treatment associates surgery and chemotherapy. Surgery should be as complete as possible because of the lack of chemotherapy sensitivity of some sarcomas, mainly angiosarcoma and synovial sarcoma. Therefore, the prognosis of cardiac sarcoma remains poor. For primary cardiac lymphoma, management should not be different from lymphoma in other locations. Chemotherapy is the main treatment, and surgery has to be used only when complications occur. Prognosis depends on histology and not lymphoma location, and so is better than the prognosis for sarcoma.
原发性心脏肿瘤在儿童中并不常见。其中大多数是良性的，只有10%是恶性的。在恶性心脏肿瘤中，肉瘤（横纹肌肉瘤、血管肉瘤、滑膜肉瘤）和淋巴瘤（伯基特淋巴瘤、大B细胞淋巴瘤、淋巴母细胞淋巴瘤）占主导地位。关于原发性恶性心脏肿瘤的儿科系列报道很少。我们在此报告3例原发性恶性心脏肿瘤的观察病例，2例肉瘤（血管肉瘤和滑膜肉瘤）和1例伯基特淋巴瘤。对这些患者进行恰当管理需要精确的病理诊断。对于肉瘤，治疗包括手术和化疗。由于一些肉瘤（主要是血管肉瘤和滑膜肉瘤）对化疗不敏感，手术应尽可能彻底。因此，心脏肉瘤的预后仍然很差。对于原发性心脏淋巴瘤，其治疗与其他部位的淋巴瘤并无不同。化疗是主要治疗方法，仅在出现并发症时才使用手术。预后取决于组织学而非淋巴瘤的位置，因此比肉瘤的预后要好。
