Xu Chang,Chen Hou-ping,Zhong Lin,et al.Clinical Analysis of Pancreatoblastoma in child.[J].Journal of Clinical Pediatric Surgery,2010,9(02):0.
儿童胰母细胞瘤诊断与治疗分析
- Title:
- Clinical Analysis of Pancreatoblastoma in child.
- Keywords:
- pancreatoblastoma; alpha-foetoprotein; Child
- 摘要:
- 目的 儿童胰母细胞瘤是一种罕见的小儿恶性肿瘤，本文拟通过分析本院近年来收治的儿童胰母细胞瘤病例，探讨其诊断与治疗要点。 方法 回顾性分析本院2002 ～ 2006年收治的3例儿童胰母细胞瘤患儿病历资料。 结果 3例中，除1例因瘤体巨大出现腹痛、呕吐等非特异性症状，就诊发现包块外，其余两例均因偶然原因体检发现腹部包块。入院体查均可扪及左腹部包块，质硬，不活动。CT检查均提示为左腹膜后巨大实性占位性病变，血清AFP值均显著增高。术中发现，3例患儿肿瘤均位于胰尾部，边界清楚，包膜完整，手术完整切除肿瘤。术后病理检查证实为胰母细胞瘤。本组因手术完整切除肿瘤且未查见远处转移，3例患儿术后均未行化疗。所有患儿均生存，随访期间无复发，复查AFP均无异常升高。结论 儿童胰母细胞瘤多无特异性症状、体征及影像学表现，早期发现和诊断非常困难。血清AFP值可作为瘤标用于诊断与随访。儿童胰母细胞瘤往往呈膨胀性生长，有完整包膜，预后相对较好, 应积极治疗。在肿瘤发生远处转移之前，早期诊断并手术切除肿瘤是治愈该病的唯一途径。若能一期彻底切除肿瘤，术后可无需化疗。
- Abstract:
- Objective Pancreatoblastoma(PB) is a rare pancreatic tumor that generally affects infants and young children. In this study, three cases of childhood PB are reported to emphasize the clinical course of this rare disease. Methods Three cases of pathologically proven PB treated at West China Hospital were reviewed. Results Abdominal pain and vomiting were observed in one case for the huge size of the tumor. The other tow cases, who were previously well, complained of a mass in the abdomen after a casual physical examination. Elevated serum alpha-foetoprotein(AFP) levels were noted in all cases. Imaging findings indicated well-defined heterogeneous large mass in the left retroperitoneal space. Exploratory laparotomy revealed a large mass, arising from tail of pancreas, not infiltrating the adjacent structures in all cases. Surgery alone with complete excision of the masses was perfumed. The histopathological examination revealed PB. All of these three cases have a good outcome in the follow-up without adjuvant chemotherapy. Conclusions These data suggest that the diagnosis of PB is difficult and should be suspected at palpation of an abdominal mass. AFP may serve as a tumor marker for preoperative diagnosis and postoperative recurrence. PB is a curable tumor and adjuvant chemotherapy may not necessary if the tumor can be excised completely.
参考文献/References:
1 Shorter NA, Glick RD, Klimstra DS, et al. Malignant pancreatic tumors in childhood and adolescence: The Memorial Sloan-Kettering experience, 1967 to present [J]. J Pediatr Surg, 2002, 37(6):887-892.
2 Naik VR, Jaafar H, Leow VM, et al. Pancreatoblastoma: rare tumor accidentally found [J]. Singapore Med J, 2006, 47(3):232-234.
3 Gu WZ, Zou CC, Zhao ZY et al. Childhood pancreatoblastoma clinical features and immunohistochemistry analysis [J]. Cancer Lett, 2008, 264(1):119-126.
4 Levey JM, Banner BF. Adult pancreatoblastoma: a case report and review of the literature [J]. Am J Gastroenterol, 1996, 91(9):1841-1844.
5 Rajpal S, Warren RS, Alexander M, et al. Pancreatoblastoma in an adult case report and review of the literature [J]. J Gastrointerst Surg, 2006 , 10(6):829-836.
6 Kohda E, Iseki M, Ikawa H, et al. Pancreatoblastoma. Three original cases and review of the literature [J]. Acta Radiol, 2000, 41(4):334-337.
7 Cingolani N, Shaco-Levy R, Farruggio A, et al. Alpha-fetoproten production by pancreatic tumors exhibiting acinar cell differentiation. study of five cases, one arising in a mediastinal teratoma [J]. Hum Pathol, 2000, 31(8):938-944.
8 Lee YJ, Hah JO. Long-term survival of pancreatoblastoma in children [J]. J Pediatr Hematol Oncol, 2007, 29(12):845-847.
9 Cao L, Liu D. Diagnosis and treatment of pancreatoblastoma in China [J]. Pcreas, 2007, 34(1):92-95.
10 Salf MW. Pancreatoblastoma [J]. JOP, 2007, 8(1):55-63.
11 Dhebri AR, Connor S, Campbell F, et al. Diagnosis, treatment and outcome of pancreatoblastoma [J]. Pancreatology，2004, 4(5):441-451.
备注/Memo
1，四川大学华西医院小儿外科（成都市，610041），E-mail：xcxcxc52229081@163.com；2，四川大学华西医院病理科（成都市，610041），通讯作者：钟麟，E-mail：zhonglinabc@163.com