Carcinoid Syndrome in a Patient with Duodenal Carcinoid Tumor

Khan Md. Nazmus Saqeb, Fariah Sharmeen, Farzana Hafiz
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Abstract

Duodenal carcinoid tumors accounts for 5% of all Gastrointestinal Neuroendocrine Tumors (GI-NETs). Only 4% of all duodenal carcinoid tumors present with a full-blown carcinoid syndrome. We report a case of duodenal carcinoid tumor presenting as carcinoid syndrome in a 58 year old man, who presented with upper abdominal discomfort, diarrhoea, hot flushes and occasional wheezing. Histopathology following endoscopic resection of the tumor and 24 hour urinary 5-Hydroxyindolacetic acid (5-HIAA) confirmed the diagnosis. Duodenal carcinoid tumors are one of the rarest tumors of gastrointestinal tract and their association with a typical carcinoid syndrome is not that common. A high level of suspicion is required for an early diagnosis. With proper resection (endoscopic or surgical) of a localized tumor, recurrence and progression of the disease can be halted. Symptoms of carcinoid syndrome should be controlled by antidiarrheal agents, inhaled β-adrenergic agonist bronchodilators and somatostatin analogs (octreotide, lanreotide). Patients should be advised to avoid stress and conditions or substances that precipitate these symptoms. Dietary supplementation with nicotinamide can help in this regard. Patients with hepatic metastases are treated with synthetic analogues of somatostatin (octreotide, lanreotide). Systemic chemotherapy is not recommended in metastatic disease by the current guidelines. Patients should be followed up by monitoring serum chromogranin and urinary 5-HIAA. EUS, CT, MRI & somatostatin receptor scintigraphy can also be used for following up the patients.
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十二指肠类癌患者的类癌综合征
十二指肠类癌占胃肠道神经内分泌肿瘤(GI-NETs)的5%。只有4%的十二指肠类癌肿瘤表现为全面的类癌综合征。我们报告一例以类癌综合征表现的十二指肠类癌肿瘤,患者为58岁男性,表现为上腹部不适、腹泻、潮热和偶尔喘息。经内镜切除肿瘤和24小时尿5-羟基吲哚乙酸(5-HIAA)检查后的组织病理学证实了诊断。十二指肠类癌是消化道最罕见的肿瘤之一,其合并典型的类癌综合征并不常见。早期诊断需要高度的怀疑。适当切除局部肿瘤(内窥镜或手术),可以阻止疾病的复发和进展。类癌综合征的症状应通过止泻剂、吸入β-肾上腺素能激动剂、支气管扩张剂和生长抑素类似物(奥曲肽、兰雷肽)来控制。应建议患者避免压力和导致这些症状的条件或物质。在这方面,膳食补充烟酰胺可以有所帮助。肝转移患者用合成的生长抑素类似物(奥曲肽、兰雷肽)治疗。根据目前的指南,转移性疾病不推荐全身化疗。患者应监测血清嗜铬粒蛋白和尿5-HIAA。EUS、CT、MRI及生长抑素受体显像也可用于患者的随访。
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