A rare case of duodenal carcinoid presenting as ectopic Cushing’s syndrome

ACTH-dependent Cushing syndrome (CS) due to an ectopic source is responsible for approximately 10-15% cases of Cushing’s syndrome. It is associated with various tumors such as small cell lung cancer and well-differentiated bronchial or gastrointestinal neuroendocrine tumors. Many a times ectopic ACTH production is difficult to manage, and identification of the source may take many years. Hormonal diagnostics include assessments in basic conditions as well as dynamic tests, such as the high-dose dexamethasone suppression test and corticotrophin releasing hormone (CRH) stimulation test. Treatment selection depends on the type of tumor and its extent. In the case of neuroendocrine tumors, the main treatments are surgery and administration of somatostatin analogues or bilateral adrenalectomy in refractory cases and if the source remains unidentified. Here, we report a case who presented with features of Cushing’s syndrome which eventually through workup led us to a diagnosis of duodenal carcinoid producing ectopic ACTH which is extremely rare and was successfully treated.

Duodenal Carcinoid Tumor Treated by Endoscopic Mucosal Resection / 대한소화기내시경학회지

Duodenal carcinoid tumors are rare, and they represent only 2.0~8.9% of all gastrointestinal carcinoid tumors in the western countries. Duodenal carcinoid tumors have previously been treated by surgical resection. The recently recommended treatment for a carcinoid tumor smaller than 1 cm in diameter is endoscopic resection. We experienced a case of a duodenal carcinoid tumor in a 56 year-old man who presented with upper abdominal pain. On the endoscopic examination, a 6 mm sized polypoid lesion with a central depression was noticed on the duodenal bulb. The biopsy confirmed the diagnosis of carcinoid tumor. The endoscopic ultrasound probe showed a hypoechoic tumor that was confined to the submocosal layer. The tumor was completely resected by an endoscopic mucosal resection technique and using a transparent cap.

A Case of Small Duodenal Carcinoid Tumor Treated by Strip Biopsy Technique / 대한소화기내시경학회지

Carcinoid tumors of the duodenum are infrequent, represent 2.0-3.0% of all gastrointestinal carcinoid tumors in western countries and 14.8% of those in Japan. Small carcinoid tumors of the duodenum are often asymptomatic, discovered endoscopically, Duodenal carcinoid tumors have been treated by surgical resection or endoscopic resection with strip biopsy or polypectomy. We experience a case of carcinoid tumor of duodenum in 49 years woman. Endoscopic examination, 0.7 cm sized polypoid mass with central depression was noted on duodenal bulb area. We present a case of a small duodenal carcinoid treated with strip biopsy technique.

A Case of Multicentric Duodenal Carcinoid / 대한소화기내시경학회지

Carcinoid tumors are the most common endocrine tumors of gastrointestinal tract. Argentaffin cells are the origin of carcinoids. These cells belong to the amino precursor uptake and decarboxylation (APUD) system. These cells give the tumor its most distinguishing feature: the ability to produce biogenic amines and polypeptide hormones that, in turn, give rise to the dramatic carcinoid syndrome. We treated a case of multi-centric carcinoid tumor of the duodenum in a 63-year-old male patient. He was admitted to the hospital because of epigastric pain. On the gastrofiberscopic examination, 1.0 x1.5 cm and 0.7 x0.8 cm sized two polyps (Yamada type II, I) were noticed on the duodenal bulb. The biopsy specimen showed carcinoid tumors of different histologic types. The level of 24-hour urine 5-HIAA of this case was normal. He was treated with subtotal gastrectomy with Billroth-II anastomosis. We report this case with literature review.

A Case of Duodenal Bulb Carcinoid Tumor Causing a Carcinoid Syndrome without Liver Metastasis / 대한소화기내시경학회지

A primary duodenal carcinoid tumor causing carcinoid syndrome is rare. In case of accompanying carcinoid syndrome in a primary duodenal carcinoid tumor, it mostly suggests massive liver metastasis. In rare case, venous drainage of carcinoid tumor and systemic venous drainage are directly connected without passing through the portal system. Therefore, it is rare and interesting case which a primary duodenal carcinoid tumor accompanies carcinoid syndrome without liver metastasis. We experienced an occasion of a primary carcinoid tumor located in duodenal bulb in a 56 year-old woman. This patient came to our hospital because of intermittent diarrhea, epigastric pain and facial flushing. And it was surely diagnosed as carcinoid tumor by gastrointestinal endoscopic biopsy. She was hospitalized for surgery and we were able to confirm that carcinoid syndrome was accompanied through biochemical test. However, metastatic lesion was not found at liver, small and large intestine. We report this case with reference to documents due to rarity.