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)

In the present study we characterized and compared the different molecular forms of glucagon-like immunoreactivity in extracts of peripheral plasma and hepatic metastases of a patient with pancreatic alpha-cell tumor. Plasma and tissue extracts were chromatographed on Sephadex G-50 columns. Immunoreactivity in the eluting fractions was assayed with an anti-glucagon antiserum that specifically recognizes the C-terminal region of the pancreas glucagon molecule. Total plasma glucagon-like immunoreactivity prior to surgery was 26.64 nmol/l and consisted of four peaks of immunoreactivity of apparent 9,000 mol wt, 5,800-5,400 mol wt, and 4,000 mol wt. Total glucagon-like immunoreactivity extracted from the hepatic metastasis was 47.41 nmol/g wet weight and eluted as two major peaks of immunoreactivity as follows: peak I, mol wt 3,800, corresponding to "true" 3,500 mol wt glucagon; peak II, mol wt 1,400, probably consisted of glucagon degradation products. The results clearly demonstrated that both plasma and glucagon-like immunoreactivity extracted from hepatic metastases were heterogeneous and comprised species corresponding not only to "true" glucagon but also to higher mol wt forms. The lack of higher mol wt forms of immunoreactivity in the hepatic metastases of the alpha-cell tumor suggests that this metastatic tumor tissue may contain an enzyme capable of converting the higher mol wt forms to smaller glucagon-like components whereas this degradative system seems to be defective in the primary tumor.
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PMID:Differences in molecular heterogeneity of glucagon-like immunoreactivity in plasma and liver metastases of a patient with alpha-cell tumor. 298 14

A woman with a multiple-hormone-producing pancreatic islet cell tumor with hepatic metastases and with recurrent hypoglycemic attacks, was treated with streptozotocin. After this treatment, the elevated serum levels of insulin, C-peptide, glucagon and serotonin fell markedly and the low level of fasting blood glucose returned to normal. In accordance with these hormonal changes, scintiscan and CT scan revealed marked regression of the metastatic tumors in the liver. She is alive at this writing, five years after the streptozotocin treatment. Streptozotocin should thus be considered for treatment of malignant islet cell carcinoma with liver metastases and which is not amenable to surgery.
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PMID:Streptozotocin effective for treating multiple-hormone-producing malignant islet cell tumor. 298 54

A case of a 58-year-old woman with an unusual variant of malignant islet-cell tumor showing oncocytic features is described. Using the light microscopy technique, the tumor appeared comprised of solid nests of uniform cells with abundant, eosinophilic cytoplasm and round nuclei with granular chromatin. Ultrastructurally, the cells contained numerous abnormal mitochondria, dilated rough endoplasmic reticulum, and scattered dense-core neurosecretory granules, often associated with cytoplasmic filaments. Tumor cells were focally immunoreactive for insulin, glucagon, and somatostatin and diffusely immunoreactive for alpha 1-antitrypsin as assayed by the avidin--biotin technique. The tumor was immunonegative for human chorionic gonadotropin, gastrin, adrenocorticotropic hormone, and serotonin. The patient exhibited some of the clinical features associated with glucagonoma syndrome, including diabetes mellitus and chronic diarrhea. The tumor behaved in a malignant fashion, with widespread lymphatic involvement and bony metastases at the time of presentation. This report of an oncocytic islet-cell carcinoma supports the concept of oncocytic differentiation in islet-cell tumors in a fashion analagous to oncocytic carcinoids.
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PMID:Functioning oncocytic islet-cell carcinoma. Report of a case with electron-microscopic and immunohistochemical confirmation. 300 44

The clinical features of eight women and three men with nonfunctioning islet cell carcinoma of the pancreas were reviewed. The mean patient age was 58 years (range 44 to 75 years). Weight loss and abdominal pain were the most frequent presenting symptoms. An abdominal mass was palpable in five patients. At operation regional or distant metastases were present in 82% of patients. Only 18% of patients underwent resection for potential cure. All tumors proved histologically to be neuroendocrine in origin. Immunohistochemical staining showed positive reactivity for neuron-specific enolase and chromogranin in all tumors studied but was negative for insulin, glucagon, and somatostatin. Focal positivity for pancreatic polypeptide was seen in one tumor. Nine patients with unresectable disease at operation were available for follow-up. Mean survival for the entire group was 23 +/- 7.2 months (range 4 to 72 months). Survival differences between women and men appeared to favor women but were not statistically significant. Postoperative regional or systemic chemotherapy also had no significant effect on patient survival although two of the longest survivors (36 and 72 months) had received adjunctive chemotherapy. Nonfunctioning islet cell neoplasms are locally aggressive, have a propensity for early metastases, and are rarely resectable for cure. Unlike pancreatic exocrine carcinomas, endocrine malignancies may respond favorably to adjunctive chemotherapy.
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PMID:Nonfunctioning malignant neuroendocrine tumors of the pancreas. 302 43

A patient with metastatic islet cell carcinoma demonstrated multiple clinical syndromes simultaneously with secretion of ACTH, gastrin, glucagon, and serotonin. Hepatic arterial embolization resulted in an initial decrease in all secretory products, which was sustained for glucagon and serotonin. Recrudescence of the Cushings and Zollinger-Ellison syndrome was managed by surgical extirpation of the primary tumor and regional metastases as well as bilateral adrenalectomy. Electron microscopy and immunocytochemistry of the primary tumor and the metastatic lesions revealed the presence of multiple types of granules within single cells and, different patterns of secretory profiles in different tumor sites.
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PMID:Metastatic islet cell tumor with ACTH, gastrin, and glucagon secretion. Clinical and pathologic studies with multiple therapies. 303 1

We describe a patient with a pancreatic islet carcinoma presenting with spinal cord compression owing to vertebral metastases. Subsequent studies demonstrated a typical islet cell carcinoma by light microscopy. By electron microscopy, the neurosecretory granules were morphologically suggestive of glucagon production. Radioimmunoassay studies revealed markedly elevated levels of serum glucagon. Notably, the patient did not exhibit the characteristic glucagonoma syndrome. This case exemplifies clearly that elevated levels of immunoreactive neuropeptide hormones are not necessarily associated with overt hormonal syndromes. Possible mechanisms for explaining this apparent discrepancy include the production of immunoreactive molecules with weak or absent systemic biological activity. Nevertheless, the determination of immunoreactive hormone levels in neuroendocrine neoplasms is an extremely effective adjunct method for their diagnosis and monitoring.
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PMID:A glucagon-secreting pancreatic alpha islet cell tumor presenting as spinal cord compression. 303 55

The author reviews a personal and other reported cases of necrolytic migratory erythema, a useful marker of glucagon-secreting islet cell tumours of the pancreas. The lesions extend and migrate as the centres form either a bulla or a parakeratotic crust. The eruption involves particularly the body folds, feet and thighs; evolves in waves. The tongue may be smooth and firey red. Histologically, the striking feature is an eosinophilic degeneration and death of the upper part of the epidermis. Ablation of the tumour is followed by cure of the rash but in many cases the presence of metastases makes cure impossible. The exact cause of the eruption remains unknown.
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PMID:[Necrolytic migratory erythema: glucagonoma syndrome. Clinical and histological aspects]. 609 68

Eighty-one primary ovarian carcinoids and intraovarian metastases from six mid-gut carcinoids were examined for the presence of tumor cells immunoreactive with antisera raised against various neurohormonal peptides, mostly of gastroenteropancreatic (GEP) origin. Twenty of the primary and two of the metastatic carcinoids contained such tumor cells. The incidence of tumors with any kind of neurohormonal peptide immunoreactive tumor cells was 53% in the trabecular carcinoids, and 42% in the strumal carcinoids, whereas the incidence was much lower (7%) in the insular type. Immunoreactive pancreatic polypeptide (PP), glucagon, enkephalin, and somatostatin were those neurohormonal peptides most commonly observed in the tumor cells of the primary carcinoids. Those less commonly found were substance P, calcitonin, VIP, neurotensin, beta-endorphin, and ACTH. Four metastatic carcinoids were nonreactive with all the antisera used. Cells storing immunoreactive insulin, glucagon, PP, VIP, gastrin, substance P, or enkephalin were found in one of the two remaining metastatic carcinoids; in the other only gastrin-immunoreactive tumor cells were observed. The occurrence and distribution of tumor cells storing the neurohormonal peptides in ovarian carcinoids are discussed in relation to their possible origin in the ovary and to carcinoids in the gut.
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PMID:Neurohormonal peptides in ovarian carcinoids: an immunohistochemical study of 81 primary carcinoids and of intraovarian metastases from six mid-gut carcinoids. 611 50

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

A 58-year-old white woman with known metastatic glucagonoma had widespread necrolytic migratory erythema characteristic of the glucagonoma syndrome. She did not respond to conventional chemotherapy with streptozocin. After one course of dacarbazine therapy, she had remission of the glucagonoma clinically with regression of tumor metastases as defined by liver scanning. After 10 months and additional courses of dacarbazine therapy, she remains in clinical remission. Plasma glucagon levels have decreased although they remain at two to four times the upper limit of normal. On several occasions there was resolution of this patient's rash after intravenous glucose in the absence of supplemental amino acids. We conclude that dacarbazine is an effective mode of chemotherapy for malignant glucagonoma.
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PMID:Malignant glucagonoma syndrome: response to chemotherapy. 625 16


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