Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors report their experience with resection of small bowel carcinoid tumors associated with diffuse hepatic metastases. Nine patients were operated during the last ten years. A carcinoid syndrome was present biologically in every case and clinically in 8 cases. The tumor was removed in every case, either by right hemicolectomy (n = 6), or by small bowel resection (n = 3). Postoperative mortality and morbidity were nil. Adjuvant therapy included hepatic chemo-embolization and long-term therapy by a long-acting somatostatin analog. Five patients died during the period of follow-up and 4 patients were alive at the end of the study. Resection of small bowel carcinoid tumor is indicated even when the liver is involved by diffuse metastases to prevent the risk of small bowel occlusion, and to promote hepatic chemoembolization as subsequent therapy against the carcinoid syndrome and metastatic spread. Moreover, the procedure can participate in the selection of cases for orthotopic liver transplantation, when conservative therapy fails to control the carcinoid syndrome.
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PMID:[Resection of carcinoid tumors of the small intestine is still indicated in the presence of disseminated hepatic metastases]. 168 80

An example of the rare papillary cystic tumor of the pancreas was diagnosed cytologically by aspiration of the primary neoplasm. Subsequently, it metastasized, proving its low-grade malignant behavior. Diagnostic cytomorphologic features included abundant straight and branched papillary tissue fragments, and uniform, pale nuclei with folds or grooves. Although the primary tumor had a typical histologic appearance, metastases demonstrated increased nuclear pleomorphism and hyperchromasia, bizarre tumor giant cells, and an increased mitotic rate. Vimentin was diffusely positive, whereas neuron-specific enolase and somatostatin were focally and weakly reactive. Neurosecretory and zymogen granules were absent ultrastructurally. By flow cytometric study, the tumor was aneuploid (DNA Index = 1.3).
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PMID:Malignant papillary cystic tumor of the pancreas. 169 76

Ninety-nine carcinoid tumors of the duodenum were studied. Seventy-seven patients were followed up for a mean period of 65 months, 20 tumors were autopsy findings, and two patients were unavailable for follow-up. Sixteen tumors (21%) produced metastases, all discovered initially; 3 patients (4%) died from metastatic disease (mean survival, 37 months postoperatively). Features associated with metastatic risk were involvement of muscularis propria, size greater than 2 cm, and the presence of mitotic figures. For 51 tumors, there was no correlation between immunohistochemical somatostatin and history of diarrhea, cholelithiasis, or diabetes mellitus (somatostatin syndrome). Five tumors were associated with Zollinger-Ellison syndrome and had immunohistochemical gastrin, but in the others there was no correlation between ulcer disease and gastrin positivity. Duodenal carcinoids are indolent, especially when small and localized to the submucosa. Immunohistochemical identification of somatostatin and gastrin has little clinical relevance.
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PMID:Carcinoid tumors of the duodenum. A clinicopathologic study of 99 cases. 169 55

The therapeutic principles in the management of endocrine gastroenteropancreatic (GEP) tumours include surgical extirpation of the primary tumour in the absence of metastases and medical control of symptoms in the preoperative phase. In the presence of metastases only palliative procedures are available. Tumour growth might be controlled by surgical procedures as debulking of tumour masses, medically by chemotherapy and more recently by new developments as a long-acting somatostatin analogue (SMS 201-995) and alpha-interferon. Their efficacy is currently evaluated in prospective studies. In contrast to inhibition of growth symptoms derived from excessive hormone production by GEP tumours can be well controlled. SMS 201-995 effectively prevents or at least improves flush and diarrhoea in the carcinoid syndrome, disabling diarrhoea in the Verner-Morrison syndrome and migratory erythema in the glucagonoma syndrome. SMS acts by inhibition of hormone release from the tumour and by a direct mechanism at the site of the target cell via SMS receptors present on tumour and target cells. For control of acid hypersecretion in gastrinoma patients omeprazole is superior to all former and present alternatives and replaced total gastrectomy completely. A similarly effective drug to prevent hypoglycaemia due to uncontrolled insulin release from insulinomas is not available since neither SMS nor diazoxide are effective in every insulinoma patient.
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PMID:Therapeutic strategies in the management of endocrine GEP tumours. 170 88

A relationship between blood plasma levels of polypeptide growth factors and those of peptide and sex steroid hormones, as assayed radioimmunologically, was studied in 91 patients with bone tumors of various histology and 45 healthy donors. The levels of insulin-like growth factor (IGF-1) and somatotropic hormone were significantly higher in cases of chondrosarcoma and patients suffering osteogenic sarcoma in the late puberal period as compared to controls and cases of fibrous histiocytoma, giant-cell tumor, benign tumors and tumor-like lesions of the bone. The peak levels of IGF-1, somatotropic hormone and insulin were registered in osteogenic sarcoma patients who developed pulmonary metastases either in the course or after the completion of combined treatment. Somatostatin level was significantly lower in patients with osteogenic sarcoma aged 11-20 years as compared to healthy adolescents, the lowest level being observed in adolescents suffering osteogenic sarcoma with metastases to the lungs. No relationship was established between total testosterone level, on the one hand, and those of IGF-1 and epidermal growth factor, on the other. A reverse correlation was established between concentrations of IGF-1 and total estradiol. The role of polypeptide growth factor antagonists in combined treatment of bone sarcomas is discussed.
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PMID:[Polypeptide growth factors and their interrelation with hormones in the blood plasma of patients with primary bone tumors]. 184 44

A 39-year-old woman with a primary carcinoid of the common bile duct is presented. The tumor showed argyrophilia; and, by immunohistochemical studies, chromogranin, serotonin, and somatostatin were demonstrated. The patient has no symptoms 42 months after surgical treatment. To the authors' knowledge, this is the first report of a carcinoid of the common bile duct as studied by immunohistochemistry. When six choledochal carcinoids were reviewed, the following characteristics were observed: in none of the cases were endocrine symptoms present; women were affected more frequently than men; the carcinoids occurred in younger patients than did adenocarcinomas; metastases were present in half of the patients; and, as a group, carcinoids seemed to have a better prognosis than adenocarcinomas in this location.
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PMID:Primary carcinoid of the common bile duct. Immunohistochemical characterization of a case and review of the literature. 187 30

A 63-year-old Japanese man complained of hematuria and pollakisuria for several months. Computed tomography and cystography disclosed an infiltrative tumor mass in the irregularly thickened apical and posterior walls of the urinary bladder. Narrowing of the vesical lumen and posterior extension of the tumor into the pelvic cavity were also noted. After palliative ureterocutaneostomy, 60 Gy irradiation was given locally. The patient died of cachexia seven months later. Autopsy revealed neuroendocrine carcinoma of the urinary bladder with extensive invasions and metastases to the pelvic and peritoneal cavities, liver, lungs, vertebrae, left kidney and retroperitoneal lymph nodes. Histologically, atypical tumor cells with eosinophilic cytoplasm formed solid nests and anastomosing cords with pseudoglandular structures. No other histologic tumor components were included. An intact urachal remnant was found at the vesical apex while features of metaplastic cystitis were absent. In addition to positive carcinoembryonic antigen and cytokeratin, the argyrophilic cancer cells were immunoreactive for neuron-specific enolase, chromogranin A, serotonin, neuropeptide Y, glicentin, somatostatin, neurotensin and calcitonin. Ultrastructurally, neurosecretory-type granules, with a mean diameter of 166 nm, were identified in the cytoplasm of the tumor cells. To discuss the histogenesis of the tumor, 44 previously reported cases of neuroendocrine carcinoma of the urinary bladder were reviewed.
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PMID:Neuroendocrine carcinoma of the urinary bladder: case report and review of the literature. 194 51

Serotonin-producing pancreatic endocrine tumours are rare neoplasms which in most cases exhibit malignant biological behaviour. These tumours, in the majority of the well-documented cases, are composed of argyrophil- and argentaffin-positive cells which contain large pleomorphic neurosecretory granules. In contrast, argyrophilic non-argentaffin pancreatic endocrine tumours with tumour cells containing round neurosecretory granules are exceptional. In this study we describe such a tumour not associated with clinical evidence of carcinoid syndrome in a 60-year-old woman. Histological examination revealed tumour extension in pancreatic lymphatic vessels and veins but no evidence of locoregional or distant metastases. Ten months after surgery the patient showed no recurrence of the disease. Immunohistochemistry revealed cytoplasmic serotonin production in the tumour cells which were negative for anti-gastrin, insulin, glucagon, somatostatin, pancreatic polypeptide (PP), vasoactive intestinal peptide (VIP) and ACTH. This study emphasizes the usefulness of combined ultrastructural and immunohistochemical investigations in order to identify and characterize the rare pancreatic endocrine tumours with serotonin production.
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PMID:Serotonin-producing pancreatic endocrine tumour. Histological, ultrastructural and immunohistochemical study of a case. 196 80

In 15 patients with insulinoma, six patients after successful removal of this tumour, two patients with previous pancreas resection because of hypoglycaemia elsewhere, and 10 control subjects, the diagnostic usefulness of euglycaemic clamp procedures (without exogenous insulin) was assessed in comparison with prolonged starvation. Only insulinoma patients developed sustained hypoglycaemia (less than or equal to 2.3 mmol l-1) within 2-44 h without caloric intake, because of inappropriately elevated immunoreactive insulin (IR-insulin) concentrations. IR-proinsulin values were elevated in most (7 out of 10), but not in all insulinoma patients. The steady-state glucose infusion rate necessary to maintain a stable plasma glucose concentration of 4.4-5.0 mmol l-1 was significantly (P less than or equal to 0.001) higher in insulinoma patients (2.5 +/- 0.6 mg kg-1 min-1) than in pancreas resected patients (0.6 +/- 0.2 mg kg-1 min-1), or in control subjects (0.5 +/- 0.1 mg kg-1 min-1). Due to a considerable degree of overlap, sensitivity (0.44) and specificity (0.95) were too low for such a procedure to qualify as a diagnostic test. There was no correlation of glucose infusion rates to IR-insulin values (r = 0.024, P = 0.461). One reason for this was the development of insulin resistance in some, but not in all insulinoma patients. When, in analogy to insulin/glucose ratios, a diagnostic index was derived by multiplying the steady state glucose infusion rate by the steady state IR-insulin concentration, the diagnostic accuracy was greatly increased (sensitivity and specificity 0.94, respectively), but still lower than that of 'amended' insulin/glucose ratios in fasting plasma or at the time of discontinuation of prolonged fasts (1.00). Somatostatin infusions inhibited insulin secretion (IR-C-peptide plasma concentrations) by 52-88% in subjects without insulinoma and in those insulinoma patients whose tumour cells ultrastructurally contained plenty of normal secretory granules, and to a lesser degree when only abnormal or virtually no secretory granules were present, i.e. in more de-differentiated tumours. In contrast to this significant (P = 0.036) association, malignancy, i.e. the presence of metastases, could not be predicted from whether or not insulin secretion was resistant to the inhibitory action of somatostatin. In conclusion, euglycaemic clamp experiments are less reliable for detecting or excluding a functioning insulinoma than the relation of glucose and insulin values during starvation. The inhibition of insulin secretion by somatostatin depends on the presence of normal beta-granules, and does not distinguish adenomas from carcinomas.
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PMID:Evaluation of a euglycaemic clamp procedure as a diagnostic test in insulinoma patients. 196 48

Somatostatin is a regulatory hormone or tissue factor which plays an inhibitory role in the normal regulation of several organ systems, including the central nervous system, hypothalamus and pituitary gland, the gastrointestinal tract and the exocrine and endocrine pancreas. Sandostatin is an analogue of somatostatin which has characteristics which makes it a better compound for clinical use than native somatostatin: it inhibits GH preferentially over insulin. It has a long half-life in the circulation, causing a prolonged inhibitory effect in somatostatin-responsive target organs. It is active after subcutaneous administration and rebound hypersecretion does not occur. Sandostatin is very well tolerated by most patients. Somatostatin receptors remain present on a variety of tumours which arise in tissues that contain these receptors normally. High numbers of somatostatin receptors have been found on GH-secreting pituitary tumours and on most metastatic endocrine pancreatic tumours and carcinoids. Sandostatin treatment ameliorates clinical symptoms in most acromegalic patients while GH hypersecretion and elevated concentrations of circulating IGF-I are well controlled. In most patients hormonal hypersecretion from endocrine pancreatic tumours and carcinoids is also suppressed during Sandostatin therapy. This results in an instant improvement in the quality of life. There is preliminary evidence of control of tumour growth. The presence of high numbers of somatostatin receptors on tumours enables in vivo receptor-imaging, with 123iodine coupled to a somatostatin analogue. This newly developed technique provides for the first time the possibility of localization of the primary tumours and their metastases and a prediction of which patients may respond to treatment with Sandostatin. Theoretically this somatostatin-receptor imaging technique represents a new approach which may be extended to other receptor-containing tumours. Therefore it may provide a new, powerful alternative to tumour localization performed with monoclonal antibody technology. Another potential development is the use of beta-emitting isotopes coupled to somatostatin analogues for therapeutic irradiation. Somatostatin analogues exert potent inhibitory effects on the growth of a variety of experimental tumour models in animals. Several mechanisms of action have been proposed including the direct antiproliferative effects of somatostatin and its analogues in a variety of tumour cell cultures. Most well-differentiated human brain tumours like meningiomas and low-grade astrocytomas contain somatostatin receptors, while undifferentiated brain tumours mainly contain EGF receptors. Fifteen percent of human breast carcinomas contain somatostatin receptors; those which do have a better prognosis. It can be concluded that somatostatin is an endogenous, naturally occurring inhibitory growth factor.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:The clinical use of somatostatin analogues in the treatment of cancer. 197 66


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