Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neuroblastoma originates in the adrenal medulla or anywhere in the body that sympathetic tissue normally is present. It may present with a variety of symptoms due to primary tumor, metastatic disease, or unusual signs and symptoms such as opsoclonus-myoclonus or severe diarrhea. Despite the fact that this neoplasm responds to a variety of therapeutic modalities, it remains one of the most frustrating and difficult childhood tumors to treat and cure.
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PMID:Neuroblastoma. 388 4

The intestinal carcinoid tumors of 26 patients were stained for the presence of serotonin, gastrin, somatostatin, motilin, secretin, glucagon, pancreatic polypeptide, ACTH, and neurotensin. Argentaffin and argyrophil stains were also performed in all cases. Thirty-five separate tumors (counting metastases and multiple primaries) from the 26 patients were studied. Serotonin was present in 30 of the 35 tumors. Nineteen tumors contained serotonin only. Fourteen tumors contained multiple neuroendocrine products. One tumor contained gastrin only. One tumor did not stain immunohistochemically, but was argyrophilic. Metastatic deposits were studied in nine patients. Some metastases produced the identical neuroendocrine products as the primary tumor, whereas others produced either additional or fewer hormones than the primary tumor. Moreover, different metastases from the same primary tumor were observed to produce different hormones. Argyrophilic cells were present in all cases and were much more numerous than cells staining by immunohistochemistry. Argyrophilic cells probably contain monoamines and polypeptide hormones in addition to those studied in this series. The argyrophil stain was the best general stain in this study for the demonstration of neuroendocrine cells. Argentaffin staining was negative in ten cases that were serotonin positive and two argentaffin positive cases were serotonin negative. The carcinoid syndrome, as clinically defined by the presence of flushing and diarrhea, was noted in five patients, all of whom had serotonin-containing small bowel carcinoids. Endocrine-related symptoms were not clinically appreciated in the remaining patients.
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PMID:The neuroendocrine products of intestinal carcinoids. An immunoperoxidase study of 35 carcinoid tumors stained for serotonin and eight polypeptide hormones. 618 28

Eighty-two symptomatic patients with carcinoid tumors of the small intestine were examined and treated over a 20-year period. Common clinical features included weight loss, diarrhea, and symptoms of intermittent bowel obstruction; 25 patients (30%) exhibited the carcinoid syndrome. Multiple carcinoid tumors occurred in 23 patients (28%), and hepatic metastases were present in 30 (37%). All patients underwent operation. The overall mortality was 7%, and the cumulative five-year survival rate was 59%. Two factors influenced prognosis after operation: hepatic metastases and incomplete resection. Other variables, including the sex and age of the patient and the size of the primary tumor, were of no additional prognostic value. Wide resection of the tumor, including regional lymph nodes, is indicated, regardless of the size of the primary tumor.
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PMID:Surgical therapy for small-bowel carcinoid tumors. 683 Apr 29

Although patients with bronchial and ovarian carcinoid tumors can develop the carcinoid syndrome (diarrhea and/or flushing) in the absence of hepatic metastasis, it is believed that development of the carcinoid syndrome in patients with carcinoid tumors of gastrointestinal origin occurs only after the patient has hepatic metastasis. This is explained by hepatic inactivation of most of the serotonin in the portal circulation or by the fact that hepatic metastases are larger than the primary tumor in the gastrointestinal tract. Three patients with ileal and jejunal carcinoid tumors who developed the carcinoid syndrome without obvious hepatic metastasis are described. Two of the patients had intra-abdominal, but extrahepatic, metastasis that probably drained directly into the systemic circulation. The third patient had an ileal carcinoid with clinical involvement limited to adjacent mesenteric lymph nodes. Following resection of her tumor, her urinary 5-HIAA excretion and platelet serotonin level returned to normal, and her attacks of carcinoid flushing virtually ceased. She has occasional spells of "blushing" that are thought to be benign; however, further close follow-up study will be needed to be certain that she is free of disease. It is suggested that each patient with the carcinoid syndrome be evaluated with CT and technetium-99 pertechnetate liver scans. If there is no liver involvement detected with these studies, one should consider hepatic arteriogram or laparotomy to determine if the patient's tumor might be totally resectable.
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PMID:Carcinoid syndrome from gastrointestinal carcinoids without liver metastasis. 709 50

Twenty-six cases of carcinoid-related mesenteric angiopathy and intestinal infarction (three from our institution and 23 previously reported cases) were reviewed. Twenty patients presented with acute abdominal findings, including peritonitis (13 cases), intestinal obstruction (five cases), and bleeding per rectum (two cases). Fifteen patients (75%) experienced antecedent symptoms of abdominal pain and/or diarrhea, averaging 2.5 years in duration. Twelve patients (46%) exhibited symptoms of carcinoid syndrome. Mesenteric angiography in three cases demonstrated encasement and segmental branch narrowing or occlusion of major mesenteric vessels. Eleven patients underwent resection and primary bowel anastomosis with an early survival rate of 91%. Four additional patients who underwent lesser surgical procedures and five patients who did not undergo operation all died. Elastic vascular sclerosis (EVS) was identified in 19 of 22 cases with available histologic material (86%). These changes were observed in proximity to as well as distant to the primary tumor. In general, the severity of EVS did not correlate with the likelihood of gut ischemia. Although not the sole cause of intestinal gangrene in patients with midgut carcinoids, EVS may contribute significantly to the evolution of these ischemic changes.
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PMID:Mesenteric angiopathy, intestinal gangrene, and midgut carcinoids. 728 Oct 10

A 53-year-old woman presented with symptoms of weight loss, diarrhea, and melena. A barium enema with endoscopy revealed multiple colonic polyps which were shown histologically to be metastatic deposits of poorly differentiated adenocarcinoma. The primary tumor, a poorly differentiated gastric adenocarcinoma, had been resected 11 years earlier. This appears to be only the second published report of polypoid colonic metastases from gastric adenocarcinoma.
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PMID:Metastases from gastric adenocarcinoma presenting as multiple colonic polyps: report of a case. 751 12

The prognosis and the quality of life of patients with carcinoid tumors is related either to symptoms from the substances secreted or to progressive tumor growth. Medical treatment with cytotoxic agents is of marginal value for increasing life expectancy and reducing clinical symptoms. Recent studies with interferon have shown interesting results. In the present investigation, 22 patients with carcinoid tumors and syndrome were treated with recombinant interferon alpha-2a (r-IFN alpha-2a) at the dose of 6 x 10(6) IU intramuscularly daily for 8 weeks and three times weekly thereafter. The primary tumor was localized in the foregut (n = 11), midgut (n = 7), hindgut (n = 1), and unknown site (n = 3). Most cases had liver metastasis. Seventeen patients had elevated 5-hydroxyindoloacetic acid (5-HIAA) excretion and 5 had flushing and/or diarrhea as the only clinical manifestation. Six cases presented a complete syndrome (flushing, diarrhea and 5-HIAA excretion). Control of symptoms was obtained in 80% and a 5-HIAA level reduction in 58% of the patients. The interferon treatment was more effective for control of the carcinoid syndrome than for control of tumor growth. The treatment was well tolerated and fever, myalgia, anorexia and fatigue were the most frequent side-effects.
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PMID:Treatment of carcinoid syndrome with recombinant interferon alpha-2a. 768 66

A 78-year old male came to our observation presenting and enlargement of bilateral inguinal lymph nodes and a tumor of the mesogastric abdominal wall. Three years before the patient had been operated on for a primary tumor of the umbilicus with concomitant longstanding diarrhea. No histological examination was performed at that time. We performed a lymph node biopsy which demonstrated carcinoid metastasis. We went on to perform radical resection of the abdominal wall, regional lymphadenectomy and right hemicolectomy for malignant villous adenoma of the right colon. The abdominal defect was repaired by using Goretex mesh. Cyclic adjuvant alpha-interferon therapy was continued for more than 1 year, followed by long term therapy with longastatin. Twenty months after the operation the patient is in good clinical conditions and disease-free. On the basis of literature review our case appears to be the first primary carcinoid of the umbilicus.
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PMID:[Primary carcinoid of the umbilicus. Description of the 1st case]. 770 43

Two cases of neuroendocrine tumor in the liver, positive for VIP, without evidence of a primary tumor outside the liver is presented. One patient had a VIPoma syndrome with diarrhea, hypokalemia, and hypercalcemia, all symptoms were reversible after treatment consisting of somatostatin analogue and arterial liver embolization followed by liver resection. The other patient showed no endocrine symptoms. To the best of our knowledge, VIPomas apparently primary in the liver have not been previously described.
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PMID:Liver VIPoma: report of two cases and literature review. 795 Aug 21

The clinical evolution of type I multiple endocrine neoplasia (MEN I) was studied in 45 patients among a consecutive series of 172 with Zollinger-Ellison syndrome (ZES). These 172 patients were seen in our hospital between 1959 and 1989. Diarrhea was half as frequent in ZES-MEN I as in sporadic ZES cases. At diagnosis, mean basal acid output and serum gastrin levels in MEN I patients (28.8 +/- 6.6 mmol/h and 587 +/- 487 pg/ml, respectively) were not different from those observed in the others with sporadic ZES. Laparotomy was performed in all 36 patients with no diffuse liver involvement to attempt the removal of gastrinomas. Twenty-nine patients had adenomas, located in the pancreas in 21, in the duodenal wall in 3, and in both in 5. Adenomas were multiple in 23 cases (78%). No tumor was found in seven patients. Twenty-nine of the 36 operated patients were tumor-free after surgery; 7 died in the postoperative period between 1959 and 1970. Median follow-up of the 38 other patients was 95 months (range 17-278 months). Among the 24 patients without residual tumor at discharge (group I), biological and/or morphological evidence of a persistent or recurrent source of gastrin was obtained in 22. Among the 14 patients with residual tumor (group II), an increase in tumor size was seen in 5 after a median of 27 months (range 9-36 months), while no change occurred in 9 after 54 months (3-100 months). Actuarial survival curves were not different, either in group I versus group II patients (67 and 72% at 5-year follow-up, respectively) or in MEN I versus sporadic ZES patients. Apparently, complete resection of primary tumor did not reduce the incidence of subsequent liver metastases. In all, 21 of the 45 patients had malignant gastrinomas (47%), consisting of liver metastases in 14 (31%), metastatic lymph nodes in 11 (24%), and lung metastases in 2 (4%). Monitoring of fundic argyrophil cells disclosed hyperplasia in 13 of the 14 MEN I patients (92%), and 5 had invasive carcinoid tumors. Taken together, these results prompt us to recommend that in ZES-MEN I patients, surgery should be avoided and oxyntic mucosa regularly monitored.
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PMID:Clinical, anatomical, and evolutive features of patients with the Zollinger-Ellison syndrome combined with type I multiple endocrine neoplasia. 809 74


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