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小儿肝间叶性错构瘤诊断和治疗

Diagnosis and treatment of hepatic mesenchymal hamartoma in children
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摘要 目的探讨小儿肝间叶性错构瘤的临床特征与诊治方法。方法结合文献,回顾性分析1998年2月至2007年10月南京军区普通外科研究所收治的8例肝间叶性错构瘤患儿的临床表现、诊断、治疗和预后。8例患儿术前均行超声与CT或(和)MRI检查,术中经快速冷冻切片病理检查,行肿瘤局部切除术5例,肝叶切除术3例。结果术前根据影像学检查结果诊断为肝间叶性错构瘤4例,肝脏肿瘤(性质待查)1例,误诊3例。术中冷冻切片确诊5例。术后8例均经常规病理确诊。术中出血5~800ml,平均出血量为119ml。术后除1例患儿出现肺部感染经对症治疗痊愈外,其余无并发症。住院时间为7~21d,平均12d。随访10~126个月,平均68.5个月,均健康无瘤存活,未见复发或恶变。结论术前影像学检查有助于诊断和治疗,但由于该病罕见,仍存在较高的误诊率,外科手术是首选治疗方法,可选择肿瘤局部切除术或规则性肝切除术。术中出血与术后感染是可能出现的并发症。肿瘤完整切除后,多预后良好,偶有复发或恶变,需长期随访,定期复查。 Objective To investigate the clinical features, diagnosis and treatment of hepatic mesenchymal hamartoma (HMH) in children. Methods Eight cases ( male 7, female 1 ) aging from 25d to 5yr (mean 1.4yr) of HMH that admitted between Feb. 1998 and Oct. 2007 were retrospective ly analyzed. Intraoperative frozen section examination was applied. The operative procedures included local tumor resection in 5 cases, the right or left hepatic lobectomy in 3. Results Based on the characteristics of preoperative imaging , four cases were confirmed the diagnosis of HMH . All cases were finally diagnosed by postoperative pathological examinations. The intraoperative blood loss ranged from 5 to 800 ml (average 119 ml). The postoperative course was uneventful except one case complicated with pulmonary infection. The hospitalization was from 7 to 21 days (average 12 days). All of the patients were alive and tumor-free during a follow-up period of 10-126 mon (mean 68. 5 months) . Conclusions HMH is a rare benign tumor in children. The imaging examination is helpful to confir mation of diagnosis. The first choice of treatment is surgical operation on local tumor resection or hepatectomy. The prognosis of HMH is rather favorable. Long-term and regular follow-up are necessary.
出处 《中华小儿外科杂志》 CSCD 北大核心 2009年第3期168-172,共5页 Chinese Journal of Pediatric Surgery
关键词 肝肿瘤 错构瘤 外科手术 Liver neoplasm Hamartoma Surgical procedures, operative
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参考文献20

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