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

Carcinoid tumors derived from neuroendocrine cells can release serotonin and other vasoactive substances into the systemic circulation, resulting in carcinoid syndrome. Testicular carcinoid, a rare disease accounting for less than 1% of all testicular neoplasms, rarely manifests symptoms of carcinoid syndrome. We describe a case of carcinoid syndrome arising from a primary testicular carcinoid tumor. A 21-year-old male patient presented with facial flushing and diarrhea for 5 years. He had an enlarged left testis and a 1-cm, ill-defined, hard, non-tender mass in his right testis. His 24 h urinary excretion of 5-hydroxyindoleacetic acid was elevated (16.1 mg/day). Somatostatin receptor scintigraphy correlated with carcinoid tumor in both testes. Following bilateral orchiectomy, the patient's facial flushing and diarrhea disappeared.
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PMID:Primary carcinoid tumor of the bilateral testis associated with carcinoid syndrome. 1550 16

This 6-month, open, non-controlled, multicenter, dose-titration study evaluated the efficacy and safety of 28-day prolonged-release (PR) lanreotide in the treatment of carcinoid syndrome. Eligible patients had a carcinoid tumor with > or =3 stools/day and/or > or =1 moderate/severe flushing episodes/day. Six treatments of 28-day PR lanreotide were administered by deep subcutaneous injection. The dose for the first two injections was 90 mg. Subsequent doses could be titrated (60, 90, 120 mg) according to symptom response. Seventy-one patients were treated. Flushing decreased from a mean of 3.0 at baseline to 2.3 on day 1, and 2.0 on day 2, with a daily mean of 2.1 for the first week post-treatment (p < 0.05). Diarrhea decreased from a mean of 5.0 at baseline to 4.3 on day 1 (p < 0.05), and 4.5 on day 2, with a daily mean of 4.4 for the first week post-treatment (p < 0.001). Symptom frequency decreased further after the second and third injections, and reached a plateau after the fourth injection. By month 6, flushing and diarrhea had significantly decreased from baseline by a mean of 1.3 and 1.1 episodes/day, respectively (both p < or = 0.001); 65% of patients with flushing as the target symptom and 18% of diarrhea-target patients achieved > or =50% reduction from baseline. Median urinary 5-hydroxyindoleacetic acid and chromogranin A levels decreased by 24 and 38%, respectively. Treatment was well tolerated. 28-day PR lanreotide was effective in reducing the symptoms and biochemical markers associated with carcinoid syndrome.
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PMID:Rapid and sustained relief from the symptoms of carcinoid syndrome: results from an open 6-month study of the 28-day prolonged-release formulation of lanreotide. 1597 11

A primary testicular carcinoid tumor with teratoma metastasized to the para-aortic lymph node. After inguinal orchiectomy, serum and urinary levels of 5-hydroxyindoleacetic acid (5-HIAA) were found to be elevated. Chemotherapy consisting of cisplatin, etoposide and bleomycin was not effective on the metastatic lesions. Retroperitoneal lymphadenectomy normalized the levels of 5-HIAA. The patient is alive without recurrence 25 months after the retroperitoneal lymphadenectomy.
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PMID:Primary carcinoid tumor of the testis with teratoma metastatic to the para-aortic lymph node. 1582 68

Gastrointestinal (GI) carcinoids are ill-understood, enigmatic malignancies, which, although slow growing compared with adenocarcinomas, can behave aggressively. Carcinoids are classified based on organ site and cell of origin and occur most frequently in the GI (67%) where they are most common in small intestine (25%), appendix (12%), and rectum (14%). Local manifestations--mass, bleeding, obstruction, or perforation--reflect invasion or tumor-induced fibrosis and often result in incidental detection at emergency surgery. Symptoms are protean (flushing, sweating, diarrhea, bronchospasm), usually misdiagnosed, and reflect secretion of diverse amines and peptides. Biochemical diagnosis is established by elevation of plasma chromogranin A (CgA), serotonin, or urinary 5-hydroxyindoleacetic acid (5-HIAA), while topographic localization is by Octreoscan, computerized axial tomography (CAT) scan, or endoscopy/ultrasound. Histological identification is confirmed by CgA and synaptophysin immunohistochemistry. Primary therapy is surgical excision to avert local manifestations and decrease hormone secretion. Hepatic metastases may be amenable to cytoreduction, radiofrequency ablation, embolization alone, or with cytotoxics. Hepatic transplantation may rarely be beneficial. Chemotherapy and radiotherapy have minimal efficacy and substantially decrease quality of life. Intravenously administered receptor-targeted radiolabeled somatostatin analogs are of use in disseminated disease. Local endoscopic excision for gastric (type I and II) and rectal carcinoids may be adequate. Somatostatin analogues provide the most effective symptomatic therapy, although interferon has some utility. Overall 5-year survival for carcinoids of the appendix is 98%, gastric (types I/II) is 81%, rectum is 87%, small intestinal is 60%, colonic carcinoids is 62%, and gastric type III/IV is 33%.
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PMID:Current status of gastrointestinal carcinoids. 1588 61

A 43-year-old man presented with fluctuating symptoms of weight gain, shortness of breath, pretibial oedema, associated with anxiety and memory disturbances. Laboratory investigation revealed an adrenocorticotropin (ACTH)-dependent cyclical Cushing's syndrome characterised by remarkable variations in urinary cortisol excretions ranging from 27 to 28,050 nmol/ 24 h. Magnetic resonance imaging (MRI ) of the pituitary was normal and ectopic ACTH production was suspected. A tumour in the right anterior mediastinum was revealed on octreotide receptor scintigraphy, which had initially been overlooked on computed tomography (CT) scanning. A thymic carcinoid tumour was suspected, which was supported by increased levels of urinary serotonin, while platelet serotonin and urinary 5-hydroxyindoleacetic acid levels were normal. The tumour was removed surgically and histological examination revealed an atypical thymic carcinoid tumour. Postoperatively, the patient's symptoms disappeared rapidly. He underwent external radiotherapy and is still free of symptoms after almost two years of follow-up. For clinical practice, a cyclical Cushing's syndrome should be suspected in any patient with clinical signs of Cushing's syndrome but normal biochemistry. Repeated measurement of urinary cortisol excretion is then required to establish or rule out the diagnosis.
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PMID:Cyclical Cushing's syndrome due to an atypical thymic carcinoid. 1642 39

Type I gastric carcinoid tumors result from hypergastrinemia in 1%-7% of patients with pernicious anemia. We diagnosed pernicious anemia in a 48-year-old female patient with complaint of fatigue for three months. She had no gastrointestinal symptoms. Endoscopic examination ot the upper gastrointestinal tract revealed atrophic gastritis and a polypoid lesion in the corpus of 3-4 mm in size. Endoscopic polypectomy was performed. Histopathological examination of the specimen revealed positive chromogranin A and synaptophysin stainings compatible with the diagnosis of a carcinoid tumor. Serum gastrin level was increased, urinary 5-hydroxyindoleacetic acid was within the normal range. There was no other symptom, sign, or laboratory finding of a carcinoid syndrome in the patient. No metastasis was found with indium-111 octreotide scan, computed tomographies of abdomen and thorax. Type I gastric carcinoid tumors are only rarely solitary and patients with tumors < 1 cm in size may benefit from endoscopic polypectomy.
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PMID:Treatment of solitary gastric carcinoid tumor by endoscopic polypectomy in a patient with pernicious anemia. 1683 Mar 92

Several circulating or urinary tumour markers can be used for the diagnosis and follow-up of functioning and clinically non-functioning neuroendocrine tumours of the pancreatic islet cells and intestinal tract. Among the specific tumour markers are serotonin and its metabolites--e.g. 5-hydroxyindoleacetic acid (5-HIAA)--in carcinoid tumours and the carcinoid syndrome, insulin and its precursors or breakdown products in insulinoma, and gastrin in gastrinoma. Plasma vasointestinal polypeptide (VIP) determinations have been used in the diagnosis of VIPoma, plasma glucagon for glucagonoma, and serum somatostatin for somatostatinoma. Among the tumour-non-specific markers are: chromogranins, neuron-specific enolase (NSE), alpha-subunits of the glycoprotein hormones, catecholamines, pancreatic polypeptide (PP), ghrelin and adrenomedullin.
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PMID:Biochemistry of neuroendocrine tumours. 1738 64

The results for plasma 5-hydroxyindoleacetic acid (5-HIAA), whole blood serotonin and the chromogranins on three patients following their treatment for carcinoid disease with somatostatin analogues are presented. Two of the patients (a 56-year-old woman and a 55-year-old man) demonstrated a good clinical response following treatment, with initial high blood serotonin and plasma 5-HIAA concentrations showing a significant decline. However, blood serotonin concentrations remained above the reference range during the course of treatment, whereas plasma 5-HIAA remained borderline high or marginally elevated with respect to the clinical cut-off concentration. The third patient, a 79-year-old man, demonstrated saturation of platelets with serotonin and increasing elevation of plasma 5-HIAA, which later fell with the introduction of interferon into this patient's treatment regimen. Results for the plasma chromogranin fragments, pancreastatin (Chromogranin A) and GAWK (Chromogranin B), showed a much greater degree of variability in all three cases. This study shows promise for plasma 5-HIAA as a useful marker for monitoring carcinoid disease. The limitations of blood serotonin expressed to the platelet count are highlighted.
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PMID:Monitoring the treatment of carcinoid disease using blood serotonin and plasma 5-hydroxyindoleacetic acid: three case examples. 1745 1

Rectal carcinoids comprise 12.6% of all carcinoid tumors and represent the third largest group of the gut carcinoids. A 64-year-old woman was diagnosed as high-grade neuroendocrine carcinoma. She had liver, bone, and bone marrow metastasis. Carcinoid syndrome was diagnosed due to diarrhea, nausea, vomiting, tachycardia, and high level of 24-hour urinary 5-hydroxyindoleacetic acid (160 mg/24 hours). No response was obtained by octreotide treatment. Rectal carcinoid tumors usually show favorable prognosis; however, poorly differentiated tumors might have unusually aggressive behavior and resistance to treatment. Bone marrow involvement might be a poor prognostic factor in carcinoid tumor as has been the case in many other tumors.
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PMID:Rectal carcinoid tumor with bone marrow and osteoblastic bone metastasis: a case report. 1760 60

An 88-year-old woman presented with right heart failure, history of diarrhoea, abdominal pain, weight lost, dyspnoea over several weeks and a new pan-systolic murmur. Echocardiography showed retracted tricuspid leaflets with incomplete coaptation resulting in severe regurgitation. Subcostal view showed an adjacent hepatic cyst leading to biopsy, which revealed neoplastic neuroendocrine cells. Her 24-hour urinary 5-hydroxyindoleacetic acid level was elevated. The unifying diagnosis was carcinoid syndrome for which she was treated. Echocardiography is an important tool for diagnosis, management and prognosis of carcinoid heart disease.
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PMID:Isolated severe tricuspid regurgitation: the importance of identifying underlying mechanism. 1762 23


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