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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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

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	YU Jiaole1
	2 CHEN Zhifeng2

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YU Jiaole1,2 CHEN Zhifeng2. Clinical analysis of two kinds of cystic-like liver tumors in children with similar appearance and different prognosis[J]. 中国医药导报, 2017, 14(24): 114-117,129.

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http://www.yiyaodaobao.com.cn/EN/ OR http://www.yiyaodaobao.com.cn/EN/Y2017/V14/I24/114

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