Popa Simona Georgiana, Ungureanu Bogdan Silviu, Gheonea Ioana-Andreea, Mitrea Adina, Ardeleanu Carmen Maria, Ghiluşi Mirela-Corina, Şurlin Valeriu, Georgescu Eugen Florin, Georgescu Ion, MoŢa Maria, Marioara Oana-Maria, Săftoiu Adrian
Department of Diabetes, Nutrition and Metabolic Diseases, University of Medicine and Pharmacy of Craiova, Romania;
Rom J Morphol Embryol. 2015;56(4):1495-502.
Pancreatic neuroendocrine tumors are a rare subset of pancreatic neoplasms. We report the case of a 33-year-old female patient who was admitted to the Diabetes Clinic of Craiova, Romania, due to a two-year history of episodic neuroglycopenic hyperinsulinemic hypoglycemic symptoms, suggestive for insulinoma associated with facial and upper trunk flushing characteristic to carcinoid syndrome. During these episodes, the laboratory investigations showed hypoglycemia (38 mg/dL), hyperinsulinemia (54.72 μU/mL) and normal values of beta-hydroxybutyrate, chromogranin A, serotonin, anti-insulin antibodies and urinary levels of 5-hydroxyindoleacetic acid. Endoscopic ultrasound with SonoVue and 3T MRI revealed an 18.3/16.3 mm hypervascular tissular mass situated in the uncinate process of the pancreatic head in close contact with the superior mesenteric vein without invasion and no other detectable secondary lesions in the pancreas or any other abdominal viscera. Patient underwent enucleation of pancreatic tumor. The histological and immunohistochemical findings indicated a functional well-differentiated pancreatic neuroendocrine tumor, G1 category according to the World Health Organization (WHO) criteria, with uncertain behavior (Ki67 index was 3%), confined to the pancreas, but with tumoral invasion of the delimiting conjunctive capsule. No evidence of tumoral CK19 staining, mitoses and necrosis, angioinvasion or extra-pancreatic invasion was observed. A post-operative nine-month follow-up showed resolution of hypoglycemic symptoms, normalized blood glucose and insulin levels and no evidence of recurrence. Our case report highlights the pitfalls in diagnosing a functional pancreatic neuroendocrine tumor due to atypical symptoms, the difficulty of identification and precise location of the small-size tumor and uncertain histopathological and immunohistochemical behavior.
胰腺神经内分泌肿瘤是胰腺肿瘤中罕见的一个亚群。我们报告一例33岁女性患者，因有两年发作性神经低血糖性高胰岛素血症性低血糖症状病史而入住罗马尼亚克拉约瓦糖尿病诊所，这些症状提示为胰岛素瘤，并伴有类癌综合征特有的面部和上半身潮红。在这些发作期间，实验室检查显示低血糖（38mg/dL）、高胰岛素血症（54.72μU/mL），β-羟丁酸、嗜铬粒蛋白A、血清素、抗胰岛素抗体及尿5-羟吲哚乙酸水平正常。超声内镜联合声诺维及3T磁共振成像显示，胰腺头部钩突部有一个18.3/16.3mm的高血管性组织肿块，与肠系膜上静脉紧密相邻但未侵犯，胰腺或其他任何腹部脏器未发现其他可检测到的继发性病变。患者接受了胰腺肿瘤摘除术。组织学和免疫组化结果表明，根据世界卫生组织（WHO）标准，这是一个功能良好分化型胰腺神经内分泌肿瘤，G1类别，行为不确定（Ki67指数为3%），局限于胰腺，但肿瘤侵犯了界定性结缔组织包膜。未观察到肿瘤CK19染色、有丝分裂及坏死、血管侵犯或胰腺外侵犯的证据。术后9个月随访显示低血糖症状消失，血糖和胰岛素水平正常，无复发迹象。我们的病例报告强调了因非典型症状诊断功能性胰腺神经内分泌肿瘤的陷阱、小尺寸肿瘤识别和精确定位的困难以及组织病理学和免疫组化行为的不确定性。
