Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0007095 (carcinoid)
 6,990 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 28-year-old man presented with epigastric pain and obstructive jaundice associated with a histologically and immunologically unusual variant of carcinoid tumor involving the ampulla of Vater. The tumor contained abundant psammoma bodies and exhibited immunoreactivity only for somatostatin. Immunoperoxidase studies for insulin, glucagon, vasoactive intestinal peptide, calcitonin, serotonin, and ACTH had negative results. In contrast to most somatostatinomas of pancreatic origin, clinically this ampullary somatostatinoma was not accompanied by features of the somatostatinoma syndrome. A literature review of the clinical and hormonal features in reported cases of gastrointestinal and pancreatic somatostatinomas is presented.
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PMID:Ampullary somatostatinoma: psammomatous variant of gastrointestinal carcinoid tumor--an immunohistochemical and ultrastructural study. Report of a case and review of the literature. 631 1

The occurrence of both gastrointestinal carcinoid and von Recklinghausen's disease in the same patient is uncommon. There seems to be a predilection for ampullary carcinoids in these patients. This article reports the sixth case, including a review of the literature and a brief discussion of the implications of this finding. A carcinoid tumor should be suspected in any patient with von Recklinghausen's disease presenting with gastrointestinal bleeding, obstruction, abdominal pain, and particularly obstructive jaundice.
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PMID:Von Recklinghausen's disease associated with gastrointestinal carcinoid tumors. 640 32

We report a case of von-Recklinghausen's disease presenting with obstructive jaundice and found to have a somatostatin-containing carcinoid tumor in the papilla of Vater and a small neurofibroma in the duodenum. A 42-year-old woman with von-Recklinghausen's disease presented with intermittent jaundice, pruritus, and mild steatorrhea of a two-year duration. Abdominal ultrasonography and computed tomography showed dilated intrahepatic ducts, common bile duct and pancreatic ducts. Duodenoscopy showed a tumor at the papilla of Vater, but a preoperative biopsy failed to provide a definite diagnosis. Laparotomy revealed a yellowish tumor at the papilla of Vater and another nodule on the mesenteric side of the second section of the duodenum. Microscopically, the tumor at the papilla of Vater was found to be a somatostatin-containing carcinoid tumor. The small nodule on the mesenteric side was a neurofibroma. The jaundice, pruritus and steatorrhea disappeared after surgery.
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PMID:Somatostatin-containing carcinoid tumor of the duodenum in neurofibromatosis: report of a case. 790 72

We report a patient with biliary obstruction of two years evolution. Blood chemistry evaluation was compatible with obstructive jaundice. Endoscopy and CT were suggestive of ampullary tumor. At laparotomy, the biopsy specimen was diagnosed as carcinoid tumor, and was treated by a Whipple's procedure.
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PMID:[Carcinoid tumor of the Vater's ampulla]. 830 62

Carcinoids of the stomach, duodenum, and pancreas are represented by a variety of tumors with variable histologic and clinical features. Multicentric gastric carcinoids and concomitant nonantral argyrophilic hyperplasia are common in chronic atrophic gastritis, more rarely due to a multiple endocrine neoplasia (MEN)-related Zollinger-Ellison syndrome (ZES). These tumors are infrequently associated with metastases and may generally be dealt with by repeated endoscopic fulguration. Sporadic carcinoids tend to be larger, invasive, and more often metastatic, especially in the presence of atypical histology. Small tumors may be removed by endoscopy, but larger lesions need to be surgically excised. In association with metastases a histamine-related atypical carcinoid syndrome may evolve and require treatment with a somatostatin analog. Poorly differentiated neuroendocrine carcinomas of the stomach constitute markedly aggressive tumors that rarely are suitable for radical surgery. Gastrinomas are the most prevalent duodenal carcinoids and a common cause of ZES especially in MEN-I. Despite a marked tendency for regional lymph node dissemination, liver metastases occur late and duodenal gastrinomas are often excisable, thereby offering favorable odds for cure in ZES. Unusual somatostatin-rich carcinoids in the ampulla of Vater relate to von Recklinghausen's disease and may be the cause of obstructive jaundice; depending on their size, these tumors may be removed by local excision or pancreaticoduodenectomy. Gangliocytic paragangliomas are unusual, generally benign lesions of the duodenum. Rare pancreatic tumors with serotonin immunoreactivity may be classified as carcinoids and constitute an unusual cause of the carcinoid syndrome.
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PMID:Management of carcinoid tumors of the stomach, duodenum, and pancreas. 866 14

Obstructive jaundice is most commonly due to luminal stones or lesions of the head of the pancreas and more rarely ampullary and primary common bile duct lesions. Obstruction due to lesions of the ampulla of Vater may be due to adenocarcinoma which has a significantly better long term prognosis than carcinomas located in the head of the pancreas. A case is presented where two tumours were identified at the ampulla of Vater of the resected specimen one an adenocarcinoma and the other a carcinoid tumour representing a collision tumour.
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PMID:Collision tumour of the ampulla of Vater: carcinoid and adenocarcinoma. 918 78

Carcinoids of the extrahepatic bile ducts and particularly the common bile duct are extremely rare. A 65-year-old woman presented with obstructive jaundice. Laboratory and imaging studies gave results that were consistent with an obstructing lesion in the common bile duct. In this case, a stent was inserted initially to decompress the bile ducts. Subsequently a laparotomy and pancreaticoduodenectomy were performed and a tissue diagnosis of carcinoid of the common bile duct was made. The patient was well with no evidence of recurrence 17 months postoperatively. The authors believe this is the 19th reported case of an extrahepatic bile duct carcinoid.
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PMID:Carcinoids of the common bile duct: a case report and literature review. 1007 90

The authors report seven patients with carcinoid tumors of the extrahepatic bile ducts (EHBDs). All patients were women, with an average age at diagnosis of 49.8 years (range, 37-67 yrs). The most common presenting symptom was painless jaundice with or without pruritus. Although one patient had peptic ulcer disease before the onset of obstructive jaundice, none had systemic endocrine manifestations. These neoplasms were most often located in the common bile duct. Grossly, the carcinoid tumors were usually nodular and poorly demarcated, and ranged from 1.1 to 2.7 cm in size. Only one of the neoplasms was polypoid. Microscopically, the tumors had a trabecular or nesting pattern with occasional tubule formation, and were composed of relatively small cells with granular chromatin. All of the neoplasms expressed chromogranin and two expressed synaptophysin. Three expressed serotonin and two of the three were also immunoreactive for pancreatic polypeptide or somatostatin. Two tumors were focally positive for gastrin and one of these two tumors was also positive for serotonin and pancreatic polypeptide. All seven carcinoid tumors showed no immunoreactivity for p53, and assays for p53 loss of heterozygosity analysis were negative in two, suggesting that p53 mutations do not play a role in the pathogenesis of EHBD carcinoids. A mutation in codon 12 of K-ras was found in one carcinoid tumor whereas two of two showed immunoreactivity for Dpc4 protein. In view of the small number of carcinoids studied, the importance of these findings in the pathogenesis of these tumors is unclear. Ultrastructural examination of three of the tumors revealed numerous membrane-bound, round neurosecretory granules. Clinically, these lesions had an indolent course. Even in the presence of lymph node metastases (noted in two patients), all of the patients remained disease free 2 to 11 years (average follow up, 6.6 yrs) after segmental resection or pancreaticoduodenectomy (Whipple's procedure). Because carcinoid tumors of the EHBD are of low malignant potential, they should be separated from the more common adenocarcinomas in this location.
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PMID:Carcinoid tumors of the extrahepatic bile ducts: a study of seven cases. 1107 51

A 39-year-old man presented with progressively increasing constipation and painful lumps in the abdomen. Exploration showed extensive nodal metastases but no primary lesion was seen. The masses were excised and sigmoid colostomy done. Histology revealed carcinoid tumor with small cell differentiation. A trial of chemotherapy gave no response. At follow up of 18 months he is leading a comfortable life with a colostomy and a billary stent in place (placed for obstructive jaundice due to porta node).
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PMID:Large nodal metastases from carcinoid tumor causing bowel obstruction. 1269 34

We report a 38-year-old lady with carcinoid tumor of the extrahepatic biliary tract who presented with recurrent obstructive jaundice and previous surgery for suspected choledocholithiasis. MRCP revealed a large bile duct tumor extending from the confluence up to the superior aspect of the pancreas; this was completely excised, with bilio-enteric anastomosis. These tumors are characteristically slow growing and, therefore, are amenable to aggressive surgical excision, which offers the best chance of cure.
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PMID:Carcinoid tumor of bile duct. 1642 27


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