Scougall P, Staheli L T, Chew D E, Taylor T K, Almquist E E
Royal North Shore Hospital, St. Leonards, Sydney, Australia.
Orthop Rev. 1987 Jul;16(7):481-8.
Eight cases of extraabdominal desmoid tumors in children are reviewed. Seven were located in the pelvis or forearm, and the most common presenting complaint was a slowly enlarging mass. In all cases, diagnosis was established by open biopsy, and initial treatment was by surgical excision. At follow-up (average, 5.8 years), six patients were tumor free. Desmoids are benign tumors that usually carry a good prognosis. There is no difference between the tumor behavior in children and adults. The treatment of choice is wide local excision. If vital structures are involved, it may be more appropriate to preserve function by performing partial tumor excision. For quiescent recurrent tumors, observation is appropriate management, but if further treatment is required, reexcision or radiotherapy may be tried.
本文回顾了8例儿童腹外硬纤维瘤病例。其中7例位于骨盆或前臂，最常见的主诉是肿块缓慢增大。所有病例均通过开放活检确诊，初始治疗为手术切除。随访（平均5.8年）时，6例患者无肿瘤。硬纤维瘤是良性肿瘤，通常预后良好。儿童和成人的肿瘤行为无差异。治疗的选择是广泛局部切除。如果重要结构受累，通过部分肿瘤切除来保留功能可能更合适。对于静止性复发性肿瘤，观察是合适的处理方法，但如果需要进一步治疗，可以尝试再次切除或放疗。
