Leal N, López J C, Díaz M, Ros Z, Pérez Alonso P, Tovar J
Departamento de Cirugía Pediátrica, Hospital Infantil, La Paz, Paseo de la Castellana 261, 28046 Madrid.
Cir Pediatr. 2000 Oct;13(4):156-8.
Most of the soft tissue sarcomas of childhood other than rabdomyosarcoma, have as final diagnosis fibrosarcoma, specially in the first months of life. Overlapping features between infantile myofibromatosis and congenital fibrosarcoma, hemangiopericytoma and fibrohistiocytoma have seen noted. Five patients with congenital fibrosarcoma were operated since 1991. Anatomical sites included lower limb, hand and forearm, and three of them had previous diagnoses of hemangiopericytoma, fibrohistiocytoma and myofibromatosis, with provocated non radical surgery and reoperation. The postoperative course was satisfactory and all are live one to eight years later. In our experience accurate histological diagnosis must be achieved to perform radical surgery on these cases.
除横纹肌肉瘤外，大多数儿童软组织肉瘤最终诊断为纤维肉瘤，尤其是在生命的最初几个月。已注意到婴儿肌纤维瘤病与先天性纤维肉瘤、血管外皮细胞瘤和纤维组织细胞瘤之间存在重叠特征。自1991年以来，对5例先天性纤维肉瘤患者进行了手术。解剖部位包括下肢、手部和前臂，其中3例先前被诊断为血管外皮细胞瘤、纤维组织细胞瘤和肌纤维瘤病，接受了非根治性手术并再次手术。术后过程令人满意，所有患者在1至8年后均存活。根据我们的经验，对这些病例进行根治性手术必须获得准确的组织学诊断。
