Ayala A G, Ro J Y, Goepfert H, Cangir A, Khorsand J, Flake G
Semin Diagn Pathol. 1986 May;3(2):138-50.
We studied 25 patients under 15 years of age with desmoid fibromatosis (DF). There were 15 boys and 10 girls; 13 were under seven years of age and 12 were above. Histologically, DF was identical to DF in adult patients, except for a higher mitotic rate in children's tumors. The tumors were located on the head and neck (8), upper (7) or lower (6) extremities, or the trunk (4). Patients with head and neck DF received preoperative adriamycin and 5-(dimethyltriazeno) imidazole-4-carboxamide or a combination of vincristine, actinomycin D, and cyclophosphamide. In seven patients, tumor reduction began to occur during chemotherapy, while in one patient, there was no response at all. Five patients underwent conservative resections of their residual tumors, and in two patients, all tumor disappeared necessitating no additional surgery. Follow-up in these patients indicates that 6 have no evidence of disease, 1 is alive with recurrent disease, and 1 is lost to follow-up. Surgery was the primary modality of treatment for the trunk and extremity lesions. Twelve patients had no evidence of disease from 2 months to 25 years and 5 months, two died from complications of chemotherapy, and three were lost to follow-up. Our experience agrees with that of other investigators that DF is best managed surgically. However, preoperative chemotherapy should be strongly considered for patients with tumors occurring in the head and neck areas.
我们研究了25例15岁以下的韧带样纤维瘤病（DF）患者。其中有15名男孩和10名女孩；13名年龄在7岁以下，12名年龄在7岁以上。组织学上，DF与成年患者的DF相同，只是儿童肿瘤的有丝分裂率较高。肿瘤位于头颈部（8例）、上肢（7例）或下肢（6例）或躯干（4例）。头颈部DF患者接受术前阿霉素和5-（二甲基三氮烯）咪唑-4-甲酰胺治疗，或长春新碱、放线菌素D和环磷酰胺联合治疗。7例患者在化疗期间开始出现肿瘤缩小，而1例患者完全没有反应。5例患者对残留肿瘤进行了保守切除，2例患者所有肿瘤消失，无需进一步手术。对这些患者的随访表明，6例无疾病证据，1例患有复发性疾病仍存活，1例失访。手术是躯干和四肢病变的主要治疗方式。12例患者在2个月至25年零5个月期间无疾病证据，2例死于化疗并发症，3例失访。我们的经验与其他研究者一致，即DF最好通过手术治疗。然而，对于头颈部出现肿瘤的患者，应强烈考虑术前化疗。
