治疗选择

肝脏未分化胚胎性肉瘤极其罕见,关于该肿瘤的治疗仅有极少数小规模病例分析。但高强度化疗方案似乎可以提高总生存率(OS)。目前公认的治疗方法是在可行的情况下尽量切除原发肿瘤。对于无法切除的原发肿瘤,新辅助化疗可以帮助缩小肿瘤,使之可切除[1][2][3][4]。尽管文献中分析的病例数较少,又倾向于报道成功的治疗案例,但联合所有文献,这类患儿的OS远远超过50%[1][2][3][4][5][6][7][8]。大多数患者使用的化疗方案不含顺铂,和儿科横纹肌肉瘤或尤文氏肉瘤患儿的方案相同。

肝移植偶可成功治疗无法切除的原发肿瘤[7][9]。唯一一项前瞻性病例分析由意大利与德国软组织肉瘤合作组进行,患者先接受保守性手术或活检术,随后行新辅助化疗,方案包括长春新碱、环磷酸胺、放线菌素、阿霉素和异环磷酰胺的不同组合。通常在4程化疗后对疾病进行评估,若条件允许,则在化疗后对原发肿瘤残留灶尝试行二次手术加(或)辅助化疗。17例患儿中10例在首次完全缓解后存活,1例患儿在第三次完全缓解后存活[5]

参考文献

1. Chowdhary SK, Trehan A, Das A, et al.: Undifferentiated embryonal sarcoma in children: beware of the solitary liver cyst. J Pediatr Surg 39 (1): E9-12, 2004.[PUBMED Abstract]

2. Baron PW, Majlessipour F, Bedros AA, et al.: Undifferentiated embryonal sarcoma of the liver successfully treated with chemotherapy and liver resection. J Gastrointest Surg 11 (1): 73-5, 2007.[PUBMED Abstract]

3. Kim DY, Kim KH, Jung SE, et al.: Undifferentiated (embryonal) sarcoma of the liver: combination treatment by surgery and chemotherapy. J Pediatr Surg 37 (10): 1419-23, 2002.[PUBMED Abstract]

4. Webber EM, Morrison KB, Pritchard SL, et al.: Undifferentiated embryonal sarcoma of the liver: results of clinical management in one center. J Pediatr Surg 34 (11): 1641-4, 1999.[PUBMED Abstract]

5. Bisogno G, Pilz T, Perilongo G, et al.: Undifferentiated sarcoma of the liver in childhood: a curable disease. Cancer 94 (1): 252-7, 2002.[PUBMED Abstract]

6. Urban CE, Mache CJ, Schwinger W, et al.: Undifferentiated (embryonal) sarcoma of the liver in childhood. Successful combined-modality therapy in four patients. Cancer 72 (8): 2511-6, 1993.[PUBMED Abstract]

7. Okajima H, Ohya Y, Lee KJ, et al.: Management of undifferentiated sarcoma of the liver including living donor liver transplantation as a backup procedure. J Pediatr Surg 44 (2): e33-8, 2009.[PUBMED Abstract]

8. Weitz J, Klimstra DS, Cymes K, et al.: Management of primary liver sarcomas. Cancer 109 (7): 1391-6, 2007.[PUBMED Abstract]

9. Kelly MJ, Martin L, Alonso M, et al.: Liver transplant for relapsed undifferentiated embryonal sarcoma in a young child. J Pediatr Surg 44 (12): e1-3, 2009.[PUBMED Abstract]

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