Simpson B B, Reynolds E M, Kim S H, Ferguson W S, Graeme-Cook F, Doody D P
Department of Pediatric Surgery, Massachusetts General Hospital, Boston 02114, USA.
J Pediatr Surg. 1996 Nov;31(11):1577-80. doi: 10.1016/s0022-3468(96)90184-0.
A 7-week-old boy presented with a 6-week history of failure to thrive, acute intestinal obstruction, and an apparently irreducible intussusception (noted on contrast enema). He underwent abdominal exploration, during which a cecal mass was identified and resected. The mass proved to be a leiomyosarcoma. Histologically, it was an intermediate-grade malignancy with a predicted 5-year survival rate of 16% to 23% based on data from the adult experience. Three years after resection and without having received adjuvant therapy, he is healthy and free of disease. A review of the literature showed that in infants these tumors are predominantly colonic, compared with the predilection for small intestinal lesions found in the older pediatric and adult populations. Infantile intestinal leiomyosarcomata are rare malignancies that do well if complete surgical excision of the disease can be accomplished. The histological prognostic indicators proposed for intestinal leiomyosarcomas in the adult population cannot be extrapolated to infants because when they occur in infants, they appear to be less aggressive, and these patients do well without adjuvant therapy.
一名7周大的男婴，有6周发育不良、急性肠梗阻病史，且经造影灌肠发现存在明显不可复位的肠套叠。他接受了腹部探查，术中发现并切除了一个盲肠肿物。该肿物为平滑肌肉瘤。组织学检查显示，这是一种中度恶性肿瘤，根据成人相关数据预测其5年生存率为16%至23%。切除术后三年，未接受辅助治疗，他身体健康，无疾病迹象。文献回顾表明，与大龄儿童和成人更易发生小肠病变不同，婴儿的这些肿瘤主要发生在结肠。婴儿肠道平滑肌肉瘤是罕见的恶性肿瘤，如果能完整手术切除疾病，预后良好。针对成人肠道平滑肌肉瘤提出的组织学预后指标不能外推至婴儿，因为当平滑肌肉瘤发生在婴儿时，其侵袭性似乎较小，这些患者未经辅助治疗也预后良好。
