Willnow U, Willberg B, Schwamborn D, Körholz D, Göbel U
Department of Pediatric Surgery, Heinrich-Heine University Medical Center, Düsseldorf, Germany.
Eur J Pediatr Surg. 1996 Dec;6(6):369-72. doi: 10.1055/s-2008-1071019.
Pancreatolblastomas are rare embryonal malignancies in childhood. We report a 3-year-old girl with a tumor of the head of pancreas. Staging by bone scintigraphy and CT scans of abdomen and chest did not show evidence of metastatic disease. Tumor markers showed elevated levels of alpha-1 fetoprotein (64 ng/ml; normal 0-10 ng/ml) and lactate dehydrogenase (423 U/l; normal range below 300 U/l). The tumor was macroscopically completely removed by local resection. Postoperative tumor grading was pT1, NO, MO. The child recovered very soon after surgery without severe complications. Tumor markers dropped to normal values, indicating complete remission (follow-up time 12 months). According to the biological growth characteristics of pancreatoblastomas and to the literature, localized and non-metastatic tumors should be completely resected without radical pancreatoduodenectomy and without adjuvant chemotherapy. This is the most conservative therapy with a good prognosis. However, metastatic disease, primarily inoperable conditions or local relapses are indications for chemotherapy combined with radiotherapy and followed by resection of the tumor. At present, the prognosis of such cases is rather poor.
胰腺母细胞瘤是儿童期罕见的胚胎性恶性肿瘤。我们报告了一名3岁患有胰头肿瘤的女孩。通过骨闪烁扫描以及腹部和胸部CT扫描进行分期，未发现转移病灶的证据。肿瘤标志物显示甲胎蛋白水平升高（64 ng/ml；正常范围0 - 10 ng/ml）以及乳酸脱氢酶升高（423 U/l；正常范围低于300 U/l）。肿瘤通过局部切除在肉眼下被完全切除。术后肿瘤分级为pT1、N0、M0。患儿术后恢复很快，无严重并发症。肿瘤标志物降至正常水平，表明完全缓解（随访时间12个月）。根据胰腺母细胞瘤的生物学生长特性及文献，局限性且无转移的肿瘤应行完整切除，无需进行根治性胰十二指肠切除术及辅助化疗。这是预后良好的最保守治疗方法。然而，转移性疾病、主要为不可手术的情况或局部复发是化疗联合放疗然后切除肿瘤的指征。目前，此类病例的预后相当差。
