McGrath P C, Neifeld J P, Kay S, Salzberg A M
Department of Surgery, Medical College of Virginia, Richmond 23298-0011.
J Pediatr Surg. 1988 Oct;23(10):939-41. doi: 10.1016/s0022-3468(88)80390-7.
Primary intestinal leiomyosarcomas in children are unusual tumors with little known about their natural history. Two patients (aged 9 and 10 years) with leiomyosarcoma of the jejunum treated at our institution are reported and added to the 20 other patients reported in the literature. The first patient had a grade 1 leiomyosarcoma completely resected, received no additional therapy, and remains alive with no evidence of disease 14 years later. The second patient presented with a perforated grade 2 leiomyosarcoma which was incompletely resected; he received combination chemotherapy with an initial good response, but eventually died from sarcomatosis 7 years after initial diagnosis. The 22 cases of pediatric intestinal leiomyosarcoma presented more commonly with obstruction or perforation, were able to be completely resected more often, and appear to have a better prognosis than in adults. Thus, intestinal leiomyosarcomas in children appear to have a natural history different from that of the same tumor arising in adults.
儿童原发性肠道平滑肌肉瘤是罕见肿瘤，对其自然病史知之甚少。本文报告了在我们机构接受治疗的2例空肠平滑肌肉瘤患儿（年龄分别为9岁和10岁），并将其纳入文献报道的另外20例患者。首例患者为1级平滑肌肉瘤，肿瘤完全切除，未接受额外治疗，14年后仍存活且无疾病证据。第二例患者表现为2级平滑肌肉瘤穿孔，肿瘤切除不完全；他接受了联合化疗，初始反应良好，但最终在初次诊断7年后死于肉瘤病。22例儿童肠道平滑肌肉瘤更常表现为梗阻或穿孔，更常能够完全切除，且预后似乎比成人更好。因此，儿童肠道平滑肌肉瘤的自然病史似乎与成人发生的同一肿瘤不同。
