Castro Pablo Granero, de León Alberto Miyar, Trancón Jose Granero, Martínez Paloma Alvarez, Alvarez Pérez Jose A, Fernández Fernández Jose C, García Bernardo Carmen M, Serra Luis Barneo, González González Juan J
Department of General Surgery and Gastroenterology, Hospital Universitario Central de Asturias, Oviedo, Spain.
J Med Case Rep. 2011 Aug 22;5:402. doi: 10.1186/1752-1947-5-402.
Glucagonoma syndrome is a rare paraneoplastic phenomenon, with an estimated incidence of one in 20 million, characterized by necrolytic migratory erythema, hyperglucagonemia, diabetes mellitus, anemia, weight loss, glossitis, cheilitis, steatorrhea, diarrhea, venous thrombosis and neuropsychiatric disturbances in the setting of a glucagon-producing alpha-cell tumor of the pancreas. Necrolytic migratory erythema is the presenting manifestation in the majority of cases, so its early suspicion and correct diagnosis is a key factor in the management of the patient.
We present the case of a 70-year-old Caucasian woman with glucagonoma syndrome due to an alpha-cell tumor located in the tail of the pancreas, successfully treated with surgical resection.
Clinicians should be aware of the unusual initial manifestations of glucagonoma. Early diagnosis allows complete surgical resection of the neoplasm and provides the only chance of a cure.
胰高血糖素瘤综合征是一种罕见的副肿瘤现象，估计发病率为2000万分之一，其特征为坏死性游走性红斑、高胰高血糖素血症、糖尿病、贫血、体重减轻、舌炎、唇炎、脂肪泻、腹泻、静脉血栓形成以及在胰腺产生胰高血糖素的α细胞肿瘤背景下出现的神经精神障碍。坏死性游走性红斑在大多数病例中为首发表现，因此早期怀疑并正确诊断是患者管理的关键因素。
我们报告一例70岁白种女性因位于胰尾的α细胞肿瘤导致胰高血糖素瘤综合征，经手术切除成功治疗。
临床医生应了解胰高血糖素瘤不寻常的初始表现。早期诊断可实现肿瘤的完整手术切除，并提供唯一的治愈机会。
