Tzou J S, Wu T C, Yang C M, Wang J S, Wei C F, Hwang B
Department of Pediatrics, Veterans General Hospital-Taipei, Taiwan, R.O.C.
Zhonghua Yi Xue Za Zhi (Taipei). 1994 Sep;54(3):198-203.
Primary neoplasms of the pancreas are rare in children. One variant of these tumors is pancreatoblastoma, comprising 0.5% of epithelial tumors of the pancreas. It usually affects children at 1-8 years of age, with quite equal sex ratio. The tumor can be found at any site of pancreas, but it most commonly arises in the head of pancreas. Histopathologically, pancreatoblastoma is an encapsulated tumor with distinct organoid structures and sometimes squamoid corpuscles. Acinar cells with zymogen granules are occasionally found. The tumor has favorable prognosis. We present a 14-year-old female who was admitted due to prolonged jaundice for about 2 months. Physical examination revealed pale conjunctiva, yellowish skin color and hepatosplenomegaly. Abdominal sonography and CT scan showed dilated common bile duct and a tumor mass about 2 x 2 cm in dimension located a pancreatic head. She received surgical operation to relieve obstructive jaundice and later Whipple's operation for radical resection. Six months after operation, the patient received the examination of abdominal sonography, CT scan and gallium tumor scan but there was no evidence of local recurrence or distant metastasis. Now the patient is living well for more than one year. Because of its rare occurrence, we demonstrate this case and review the literature.
胰腺原发性肿瘤在儿童中较为罕见。这些肿瘤的一种变体是胰腺母细胞瘤，占胰腺上皮性肿瘤的0.5%。它通常影响1至8岁的儿童，男女比例相当。肿瘤可发生于胰腺的任何部位，但最常见于胰头。组织病理学上，胰腺母细胞瘤是一种有包膜的肿瘤，具有明显的类器官结构，有时还有鳞状小体。偶尔可见有酶原颗粒的腺泡细胞。该肿瘤预后良好。我们报告一名14岁女性，因持续黄疸约2个月入院。体格检查发现结膜苍白、皮肤发黄和肝脾肿大。腹部超声和CT扫描显示胆总管扩张，胰头有一个约2×2 cm大小的肿瘤肿块。她接受了手术以缓解梗阻性黄疸，随后进行了惠普尔手术以根治性切除。术后6个月，患者接受了腹部超声、CT扫描和镓肿瘤扫描检查，但没有局部复发或远处转移的迹象。现在患者已健康生活一年多。由于其罕见性，我们展示此病例并复习相关文献。
