Department of Surgery, Howard University Hospital, Washington, DC.
JAMA Surg. 2013 Nov;148(11):1068-70. doi: 10.1001/jamasurg.2013.351.
Nonoperative management of focal nodular hyperplasia (FNH) is an accepted paradigm in adults, but current management strategies for children vary substantially between institutions. We reviewed medical records at Johns Hopkins Hospital between January 1, 1998, and December 31, 2008, to investigate the diagnosis, treatment, and outcome of pediatric patients with a pathologic diagnosis of FNH to provide additional data to help formulate management guidelines for this disease. Ten pediatric patients were identified as having a pathologic diagnosis of FNH, either by biopsy sample (n = 5) or hepatic resection (n = 5). The mean age of the patients was 12.1 years, and most were female (n = 7). Mean tumor size was 5.7 cm (range, 0.8-13 cm). Four of 5 patients whose FNH was diagnosed by biopsy alone developed no sequelae, and 1 patient eventually required surgery for mass effect. Patients with either large lesions (≥5 cm) or symptoms were referred for resection. Observational management of small lesions that can be confidently diagnosed as FNH appears to be safe and appropriate. Surgical resection should be reserved for large or symptomatic lesions amenable to resection.
成人中,局灶性结节性增生(FNH)的非手术治疗已被广泛接受,但目前儿童的治疗策略在不同机构之间存在很大差异。我们回顾了约翰霍普金斯医院 1998 年 1 月 1 日至 2008 年 12 月 31 日期间的病历,以调查病理诊断为 FNH 的儿科患者的诊断、治疗和转归,提供更多数据以帮助制定该疾病的管理指南。10 名儿科患者被诊断为 FNH,活检样本(n = 5)或肝切除术(n = 5)。患者的平均年龄为 12.1 岁,大多数为女性(n = 7)。平均肿瘤大小为 5.7cm(范围,0.8-13cm)。仅通过活检诊断为 FNH 的 5 名患者中的 4 名未出现任何后遗症,1 名患者最终因肿块效应而需要手术。对于大病灶(≥5cm)或有症状的患者建议进行切除术。对于能够明确诊断为 FNH 的小病灶进行观察性管理似乎是安全且合适的。对于大病灶或有症状的病灶,应保留手术切除。