两种表现近似但预后迥异的儿童囊样肝脏肿瘤临床分析

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摘要目的分析肝脏未分化胚胎肉瘤（UES）及间叶性错构瘤（MH）两种罕见疾病的临床表现、影像学特点、治疗及预后情况。方法对1990年1月～2015年12月在香港大学玛丽医院儿童及青少年科学系治疗的4例UES和MH病例的临床表现、影像学特点、治疗及预后进行分析。结果诊断为UES及MH的病例各2例，均经病理检查确诊，发病年龄11个月～15岁，其中3例为女性。2例MH患儿仅表现为腹部膨隆，不伴全身症状，1例UES患儿表现为腹部膨隆，同时伴腹部疼痛、发热、厌食及体重减轻。4例患儿实验室检查均无显著异常，仅有1例患儿出现肝酶及甲胎蛋白轻度升高。超声及CT检查均提示存在囊性和/或实性病灶。2例MH患儿接受肿瘤切除手术，1例UES患儿行三段肝脏切除术，另1例接受半肝切除术。2例UES患儿均于术后接受化疗治疗（IRS-Ⅳ方案）。1例患儿在治疗9年后复发死亡，其余3例患儿均存活至今。结论 UES与MH在临床症状、影像学检查上存在相似之处，但预后迥异，极易引起误诊。尽早进行病理活检及外科手术对于诊断及后续治疗的制订至关重要。早期诊断并采用手术联合化疗的治疗策略可显著改善UES预后。

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	于皎乐1
	2 陈志峰2

关键词 ：未分化胚胎肉瘤, 间叶性错构瘤, 儿童, 临床特征, 预后

Abstract：Objective To analyze the clinical manifestations, imaging features, treatment and prognosis of two rare cystic-like hepatic tumors: undifferentiated embryonal sarcoma (UES) and mesenchymal hamartoma (MH). Methods The clinical manifestations, imaging features, treatment and prognosis of four patients diagnosed as UES and MH in Department of Paediatrics & Adolescent Medicine, Queen Mary Hospital, the University of Hong Kong from January 1990 to December 2015 were analyzed. Results The patients diagnosed as UES and MH had 2 cases respectively and all were confirmed by pathological examination, the onset of age were from 11 months to 15 years old, and 3 of them were female. Two cases of patients with MH were only presented with abdominal distension without constitutional symptoms. One patient with UES had abdominal distension accompanied by abdominal pain, fever, anorexia and weight loss. There were no significant laboratory abnormal results in the four patients except one MH patient, who had mild elevated liver parenchymal enzymes and alpha-fetoprotein. Both ultrasonography and CT-scan showed cystic and/or solid lesions in all patients. Two patients with MH received tumor excision surgery, one patient with UES received hepatic trisegmentectomy and the other one patient underwent hemi-hepatectomy. Two patients with UES received chemotherapy after surgery(IRS-Ⅳ protocol). One patient with UES died of relapse after nine years of treatment and others achieved long-term survival. Conclusion MH and UES has similar clinical manifestations and imaging appearance. Due to the difference in prognosis, it is easy to be misdiagnosed. Surgical biopsy and excision remains mandatory to confirm the diagnosis and guide the subsequent treatment. Early diagnosis plus timely surgery and chemotherapy regime can significantly improve the prognosis of UES.

Key words： Undifferentiated embryonal sarcoma Mesenchymal hamartoma Children Clinical characteristics Prognosis

基金资助:国家自然科学基金青年基金项目（81400074）；
北京市医院管理局青年人才培养“青苗”计划项目（QML20161202）。

通讯作者: 于皎乐（1978.7-），女，博士；研究方向：儿童血液及肿瘤性疾病。陈志峰（1958.4-），男，硕士，教授；研究方向：儿童肿瘤。

引用本文:

于皎乐1,2 陈志峰2. 两种表现近似但预后迥异的儿童囊样肝脏肿瘤临床分析[J]. 中国医药导报, 2017, 14(24): 114-117,129.
YU Jiaole1,2 CHEN Zhifeng2. Clinical analysis of two kinds of cystic-like liver tumors in children with similar appearance and different prognosis. 中国医药导报, 2017, 14(24): 114-117,129.

链接本文:

http://www.yiyaodaobao.com.cn/CN/ 或 http://www.yiyaodaobao.com.cn/CN/Y2017/V14/I24/114

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