Department of Internal Medicine, University of Connecticut, 263 Farmington Avenue, Farmington, CT 06030, United States.
Pancreatology. 2012 May-Jun;12(3):211-4. doi: 10.1016/j.pan.2012.02.001. Epub 2012 Feb 8.
Pheochromocytomas rarely have 'composite' forms in which they demonstrate histologic features of a typical paraganglioma in combination with those of a neural component. Extra-adrenal 'composite' pheochromocytomas are distinctly uncommon. We describe herein a unique case of a 34-year-old female patient with type 1 neurofibromatosis who presented with abdominal pain and paroxysmal hypertension. Imaging revealed a pancreatic mass with biliary and pancreatic ductal dilatation and a hormonal assay led to the diagnosis of functional pheochromocytoma. She underwent surgical resection and histopathology revealed a composite paraganglioma-ganglioneuroma. Clinical, biochemical and radiological aspects of this rare tumor and its association with neurofibromatosis and other hereditary cancer syndromes are discussed.
嗜铬细胞瘤很少有“复合”形式，即它们同时具有典型副神经节瘤和神经成分的组织学特征。肾上腺外“复合”嗜铬细胞瘤非常罕见。本文描述了一例 34 岁女性患者，患有 1 型神经纤维瘤病，表现为腹痛和阵发性高血压。影像学显示胰腺肿块伴胆管和胰管扩张，激素检测提示功能性嗜铬细胞瘤。患者接受了手术切除，组织病理学显示为复合副神经节瘤-节细胞神经瘤。讨论了这种罕见肿瘤的临床、生化和影像学特征及其与神经纤维瘤病和其他遗传性癌症综合征的关系。
