Du Emma, Katz Matthew, Weidner Noel, Yoder Suzanne, Moossa A R, Shabaik Ahmed
Department of Pathology, University of California, San Diego 92103, USA.
Arch Pathol Lab Med. 2003 Nov;127(11):1501-5. doi: 10.5858/2003-127-1501-APIAEP.
Pancreatoblastoma, generally regarded as a pediatric malignant tumor, is rarely found in the adult population. Only 13 adults with pancreatoblastoma, ranging in age from 19 to 68 years, have previously been reported in the world literature. A diagnosis of pancreatoblastoma relies on characteristic histologic features, including epithelial differentiation and, more importantly, squamoid differentiation. Despite aggressive therapy, adults with pancreatoblastoma have a poor outcome. We describe a 78-year-old woman who presented with painless jaundice and was found on abdominal computed tomographic scan to have a 2.7-cm ampullary mass. The patient underwent successful pancreaticoduodenectomy. Pathologic examination of the resected tumor revealed findings characteristic of pancreatoblastoma. The tumor formed acinar and glandular structures, solid areas, and contained many "squamoid corpuscles," a defining feature of pancreatoblastoma. The tumor cells also showed acinar and ductal phenotype by immunohistochemistry. To the best of our knowledge, this case represents the oldest patient with pancreatoblastoma to be described in the literature to date and the first to occur in the ampulla of Vater. We review previously published cases and discuss the clinical and histopathologic features of adult pancreatoblastoma.
胰腺母细胞瘤一般被认为是一种儿童恶性肿瘤，在成人中很少见。世界文献中此前仅报道过13例年龄在19至68岁之间的成人胰腺母细胞瘤患者。胰腺母细胞瘤的诊断依赖于特征性的组织学特征，包括上皮分化，更重要的是鳞状分化。尽管采取了积极的治疗措施，但成人胰腺母细胞瘤患者的预后较差。我们描述了一名78岁女性，她因无痛性黄疸就诊，腹部计算机断层扫描发现有一个2.7厘米的壶腹肿块。该患者成功接受了胰十二指肠切除术。切除肿瘤的病理检查显示出胰腺母细胞瘤的特征性表现。肿瘤形成腺泡和腺管结构、实性区域，并含有许多“鳞状小体”，这是胰腺母细胞瘤的一个决定性特征。免疫组化显示肿瘤细胞还表现出腺泡和导管表型。据我们所知，该病例是迄今为止文献中描述的年龄最大的胰腺母细胞瘤患者，也是首例发生在 Vater壶腹的病例。我们回顾了此前发表的病例，并讨论了成人胰腺母细胞瘤的临床和组织病理学特征。
