Latteri S, Russo G, Vecchio R, Meli G, Latteri F
Dipartimento di Chirurgia, Università degli Studi, Catania.
Minerva Chir. 1996 Sep;51(9):729-35.
Pancreatoblastoma is a very rare tumor of the pancreas, affecting children in the first decade of life. Ultrasound and CT scan may be useful but pre-operative diagnosis is often quite difficult. High level of seric alpha-fetoprotein could be suggestive of pancreatoblastoma. The definitive diagnosis is however made by histologists when an organoid structure, well encapsulated, consisting of acinar cells with zymogen-like granules and squamoid corpuscle is demonstrated, sometimes associated with mesenchymal tissue (mixed-type pancreatoblastoma). Immunohistochemical studies may help in differentiating pancreatoblastoma from papillary cystic tumors, acinar cell carcinomas and endocrine pancreatic tumors of the pancreas. We report a case of mixed-type pancreatoblastoma in a 12-year-old female patient, located in the head of the pancreas. The treatment has been surgical, consisting of a duodeno-cephalo-pancreasectomy. Neither radiotherapy nor chemotherapy has been performed. The patient is alive, without recurrence, four months after the operation. Diagnostic, therapeutic and prognostic aspects are discussed.
胰腺母细胞瘤是一种非常罕见的胰腺肿瘤，主要影响10岁前的儿童。超声和CT扫描可能有用，但术前诊断往往相当困难。血清甲胎蛋白水平升高可能提示胰腺母细胞瘤。然而，当显示出由具有酶原样颗粒的腺泡细胞和鳞状小体组成的、包膜良好的类器官结构，有时伴有间叶组织（混合型胰腺母细胞瘤）时，组织学家才能做出明确诊断。免疫组织化学研究可能有助于将胰腺母细胞瘤与胰腺乳头状囊性肿瘤、腺泡细胞癌和胰腺内分泌肿瘤区分开来。我们报告一例12岁女性患者的混合型胰腺母细胞瘤，位于胰头。治疗方式为手术，即十二指肠-胰头-胰腺切除术。未进行放疗和化疗。患者术后四个月存活，无复发。文中讨论了诊断、治疗和预后方面的问题。
