Merrot T, Moutardier V, Viens P, Bladou F, Delpero J R
Département de Chirurgie Oncologique, Institut Paoli Calmette, Marseille.
J Urol (Paris). 1997;103(1-2):64-8.
We report an unusually uncommon case of genitourinary rhabdomyosarcoma in a child which was chemoresistant and complicated by a primary psoas abscess which presented as a pelvic mass associated with an abscess of the right iliac fossa. Ultrasound and CT investigations suggested the diagnosis of a centropelvic tumor which was confirmed at puncture-aspiration. MRI was most contributive giving a precise description of the local extension. Intensive multi-drug chemotherapy would appear to have improved outcome in patients with poor-prognosis Maurer group III tumors. In exceptional cases when no tumor response is obtained, carcinological surgery with large dissection, possibly with adjuvant radiotherapy, is indicated. Percutaneous drainage of the deep psoas abscess is as effective as classical surgery and spares the abdominal wall, particularly important if a second operation should be needed. Multidisciplinary management is required for the treatment of this highly malignant tumor.
我们报告了一例儿童泌尿生殖系统横纹肌肉瘤,极为罕见,该肿瘤对化疗耐药,并并发原发性腰大肌脓肿,表现为盆腔肿块伴右髂窝脓肿。超声和CT检查提示盆腔中央肿瘤的诊断,穿刺抽吸确诊。MRI对准确描述局部扩展情况贡献最大。强化多药化疗似乎改善了预后不良的Maurer III组肿瘤患者的结局。在极少数未获得肿瘤反应的情况下,需行广泛切除的肿瘤外科手术,可能还需辅助放疗。经皮引流深部腰大肌脓肿与传统手术效果相同,且可避免腹壁损伤,若需二次手术则尤为重要。治疗这种高度恶性肿瘤需要多学科管理。