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Postgraduate Medical Journal Aug 1978A patient is described who presented with the classical symptomatology and profound electrolyte disturbance of the Verner-Morrison syndrome due to a pancreatic apudoma...
A patient is described who presented with the classical symptomatology and profound electrolyte disturbance of the Verner-Morrison syndrome due to a pancreatic apudoma secreting vasoactive intestinal polypeptide (VIP). Diagnosis was confirmed by plasma VIP as measured by a radio-immunoassay technique now available. It is suggested that the cyclical nature of the symptoms in this case was due to cyclical release of VIP from the tumour in response to an unknown stimulus. Perfusion studies confirmed the excess secretory state of water, sodium and chloride in the small intestine. Symptoms were completely abolished by surgery and the progress is being monitored by means of serial plasma VIP estimations to detect any early recurrence of metastatic disease.
Topics: Adenoma, Islet Cell; Aged; Apudoma; Female; Gastrointestinal Hormones; Humans; Pancreatic Neoplasms; Syndrome; Time Factors; Vasoactive Intestinal Peptide
PubMed: 215990
DOI: 10.1136/pgmj.54.634.566 -
Gut Jun 1981A female patient is described with a single pancreatic tumour producing vasoactive intestinal polypeptide (VIP), insulin, and pancreatic polypeptide. The initial...
A female patient is described with a single pancreatic tumour producing vasoactive intestinal polypeptide (VIP), insulin, and pancreatic polypeptide. The initial presentation was with diarrhoea and hypokalaemia and a raised plasma VIP was demonstrated. Her symptoms improved with metoclopramide administration and absolute concentrations of 28 aminoacid (peak IV) VIP were found to have fallen. She then developed hypoglycaemia with hyperinsulinism. All symptoms resolved after surgical excision. This case emphasises the potential of these tumours to contain more than one endocrine cell type synthesising different biologically active peptides.
Topics: Apudoma; Diarrhea; Female; Gastrointestinal Hormones; Humans; Insulin; Insulin Secretion; Metoclopramide; Middle Aged; Pancreatic Neoplasms; Pancreatic Polypeptide; Vasoactive Intestinal Peptide
PubMed: 7021331
DOI: 10.1136/gut.22.6.505 -
Iodine-131 MIBG scintigraphy of neuroendocrine tumors other than pheochromocytoma and neuroblastoma.Journal of Nuclear Medicine : Official... Jun 1987Metaiodobenzylguanidine (MIBG) locates most pheochromocytomas and neuroblastomas. The tracer is concentrated in intracellular storage vesicles by an active process. Many...
Metaiodobenzylguanidine (MIBG) locates most pheochromocytomas and neuroblastomas. The tracer is concentrated in intracellular storage vesicles by an active process. Many other neuroendocrine tumors of the amine precursor uptake and decarboxylation (APUD) series have hormonal storage vesicles and, thus, the potential to take up [131I]MIBG. A variety of neuroendocrine tumors in 57 patients were studied 1, 2, and 3 days after 0.5 mCi [131I]MIBG. Views from skull to pelvis were obtained. Results of MIBG scans were compared with all available imaging modalities (including plain radiography, liver scan, ultrasound, computed tomography, and angiography) and surgical exploration. The neuroendocrine nature of the tumor was determined by histology, immunohistochemistry, electron microscopy, and the assay of appropriate biogenic amines and peptide hormones. Results were (positive/total cases): carcinoids (four of ten), nonsecreting paragangliomas (three of three), sporadic medullary carcinomas of the thyroid (MCT) (one of five), familial MCT (one of 26), chemodectomas (two of five), oat cell carcinomas (zero of four), choriocarcinoma (one of one), atypical schwannoma (with storage granules) (one of one), Merkel cell skin cancer (one of one), islet cell carcinoma (zero of one). We conclude that a wide range of neuroendocrine tumors show [131I]MIBG uptake; tumors other than pheochromocytomas and neuroblastomas are less often seen scintigraphically, but in certain cases (e.g., carcinoid and nonsecreting paragangliomas) scintigraphy may be useful in depicting the extent and location of disease and may indicate therapeutic potential. Iodine-131 MIBG shows promise in the diagnosis and staging of tumors of varied types.
Topics: 3-Iodobenzylguanidine; Aged; Apudoma; Carcinoid Tumor; Carcinoma; Carotid Body Tumor; Choriocarcinoma; Female; Humans; Iodine Radioisotopes; Iodobenzenes; Male; Middle Aged; Neurilemmoma; Paraganglioma; Paraganglioma, Extra-Adrenal; Pregnancy; Radionuclide Imaging; Thyroid Neoplasms
PubMed: 3035114
DOI: No ID Found -
Journal of Clinical Pathology Aug 1992To determine retrospectively the relative usefulness of electron microscopy and immunocytochemistry for tumour diagnosis; to monitor the influence of new antibodies and... (Comparative Study)
Comparative Study
AIMS
To determine retrospectively the relative usefulness of electron microscopy and immunocytochemistry for tumour diagnosis; to monitor the influence of new antibodies and antisera on the use of these techniques in one laboratory.
METHODS
During 1980 to 1989 inclusive, 726 tumours were examined by electron microscopy, 862 by immunocytochemistry, and 286 by both techniques. The choice of techniques and, for immunocytochemistry, the range of antibodies used were compared between each category of final diagnosis.
RESULTS
During the study period there was a sharp fall in the use of electron microscopy and a corresponding rise in immunocytochemistry. These trends applied to all categories of final tumour diagnosis, but the use of electron microscopy was sustained longer for lesions suspected or eventually confirmed to be melanomas or amine precursor uptake decarboxylation cell carcinoma (APUDomas)--for example, carcinoid tumours. The immunocytochemistry:electron microscopy use ratios ranged from 2.07:1 to 0.44:1 for the categories in which lymphoma and APUDoma, respectively, were the final diagnoses. The abandonment of electron microscopy for suspected or confirmed lymphomas and carcinomas corresponded to the increasing availability of relevant antisera and antibodies.
CONCLUSIONS
The wider application of immunocytochemistry for tumour diagnosis is endorsed, but electron microscopy should be retained for selected cases in which the results of immunocytochemistry might be predictably ambiguous or otherwise unhelpful.
Topics: Apudoma; Humans; Immunohistochemistry; Melanoma; Microscopy, Electron; Neoplasms; Retrospective Studies; Time Factors
PubMed: 1401179
DOI: 10.1136/jcp.45.8.693 -
British Medical Journal Nov 1980
Topics: Achlorhydria; Apudoma; Diarrhea; Female; Gastrointestinal Hormones; Humans; Hypokalemia; Kidney Neoplasms; Middle Aged; Syndrome
PubMed: 7437784
DOI: 10.1136/bmj.281.6251.1323 -
Proceedings of the National Academy of... Mar 1983Two human neuroendocrine tumor cell lines derived from a colon carcinoma contain either numerous double minute chromosomes (COLO 320 DM) or a homogeneously staining...
Homogeneously staining chromosomal regions contain amplified copies of an abundantly expressed cellular oncogene (c-myc) in malignant neuroendocrine cells from a human colon carcinoma.
Two human neuroendocrine tumor cell lines derived from a colon carcinoma contain either numerous double minute chromosomes (COLO 320 DM) or a homogeneously staining marker chromosome (COLO 320 HSR). We found amplification and enhanced expression of the cellular oncogene c-myc in both COLO 320 DM and HSR cells, and we were able to show that the homogeneously staining regions of the COLO 320 HSR marker chromosome contain amplified c-myc. From previous and present karyotypes, it appears that the homogeneously staining regions reside on a distorted X chromosome. Therefore, amplification of c-myc has been accompanied by translocation of the gene from its normal position on chromosome 8 (8q24). Because double minute chromosomes were features of primary cultures from the original tumor, it seems reasonable to suspect that amplification of c-myc may have contributed to tumorigenesis.
Topics: Apudoma; Chromosome Mapping; Chromosomes; Colonic Neoplasms; Gene Amplification; Humans; Karyotyping; Neoplasms, Nerve Tissue; Oncogenes; Translocation, Genetic
PubMed: 6300869
DOI: 10.1073/pnas.80.6.1707 -
CA: a Cancer Journal For Clinicians 1980A total of 44 carotid body tumors were observed for over 40 years at Memorial Sloan-Kettering Cancer Center in New York. The true nature of th paraganglioma can remain...
A total of 44 carotid body tumors were observed for over 40 years at Memorial Sloan-Kettering Cancer Center in New York. The true nature of th paraganglioma can remain obscure in those patients without prior tissue biopsy or sophisticated diagnostic studies. Eighteen patients came to surgery without a primary working diagnosis of the conditions, 43 percent of the seris. Significant advances in the preoperative workup of a cervical mass attached to the carotid sheath are now recommended: ultrasonography, radioisotope perfusion scanning, and selective carotid arteriography. Catecholamine determination should be considered for any paraganglioma exhibiting vasomotor instability or hypertension. Prior to 1945 the carotid bifurcation was sacrificed eight times with a 50 percent mortality occurring in relatively youthful subjects. In the 35 years since then, a more sophisticated surgical technique preserving the carotid circulation has resulted in over 30 resections with but one fatality. Multicentricity, both in the sporadic and the familial form, is characteristic of this rare tumor, also called "APUDoma." (APUD is an acronym derived from three of the most important characteristics of these cells: a high amine content, amine precursor uptake, and decarboxylation. Its malignant potential is beyond debate.
Topics: Adult; Aged; Carotid Body Tumor; Female; Humans; Male; Middle Aged; Paraganglioma, Extra-Adrenal
PubMed: 6250680
DOI: 10.3322/canjclin.30.5.260 -
British Journal of Cancer Nov 1992Malignant carcinoid tumours, islet cell tumours and medullary carcinomas of the thyroid are tumours with similar clinical features. In patients with unresectable or... (Clinical Trial)
Clinical Trial
Malignant carcinoid tumours, islet cell tumours and medullary carcinomas of the thyroid are tumours with similar clinical features. In patients with unresectable or metastatic tumours leukocyte interferon (IFN) and recombinant human (rh) IFN have demonstrated efficacy. Twenty-four evaluable patients with progressive tumours were treated with 2.5 megaunits rh IFN alpha-2b, administered once daily subcutaneously, for a median duration of 7 months (range 0.5-37+). Two carcinoid patients demonstrated a response in tumour size, 80% showed stable disease (SD). Sixty percent of the carcinoid patients with elevated urinary 5-hydroxyindoleacetic (5-HIAA) levels reached a biochemical partial response of the urinary 5-HIAA levels (median duration 13.5 months). In the patients with an islet cell or medullary tumour and an elevated tumour marker, the marker did not further increase. Of the 12 carcinoid patients evaluable for a symptomatic response, ten (83%) experienced a relieve of symptoms. IFN alpha-2b dose reduction or discontinuation due to toxicity was necessary in three and ten patients, respectively. No neutralising IFN alpha-2b antibodies developed despite prolonged treatment. In conclusion, IFN alpha-2b had a beneficial effect in patients with progressive tumours, while long-term IFN alpha-2b treatment did not augment neutralising antibodies. In view of the IFN alpha-2b-related toxicity, administration of IFN alpha-2b on alternating days may be preferable.
Topics: Adenoma, Islet Cell; Adult; Aged; Apudoma; Biomarkers, Tumor; Blood Platelets; Carcinoid Tumor; Catecholamines; Drug Administration Schedule; Female; Histamine; Humans; Hydroxyindoleacetic Acid; Interferon alpha-2; Interferon-alpha; Male; Middle Aged; Neoplasm Metastasis; Pancreatic Neoplasms; Phosphopyruvate Hydratase; Recombinant Proteins; Serotonin; Thyroid Neoplasms
PubMed: 1384643
DOI: 10.1038/bjc.1992.372 -
British Journal of Cancer Jan 1995We performed a phase II study with mitoxantrone in patients with carcinoid tumours, islet cell tumours and medullary carcinomas of the thyroid. Thirty-five eligible... (Clinical Trial)
Clinical Trial
We performed a phase II study with mitoxantrone in patients with carcinoid tumours, islet cell tumours and medullary carcinomas of the thyroid. Thirty-five eligible patients received mitoxantrone 12 mg m-2 i.v. every 3 weeks. Among 18 previously untreated patients, three responded (17%, 95% CI = 4-41%); no responses were achieved in 17 previously treated patients. Of the 21 patients who had carcinoid tumours, 11 were previously untreated and two achieved a response (18%, 95% CI = 2-52%). Overall response rate was 9% (95% CI = 2-23%). At a median follow-up of 43 months, median overall survival was 16 months. The median survival of 21 patients with a normal alkaline phosphatase was 29 months and 9 months for 14 patients with elevated serum levels (P = 0.005). A similar observation was noticed for gamma-glutamyltransferase (P = 0.007). We concluded that mitoxantrone is not active in APUD tumours. Elevated alkaline phosphatase and gamma-glutamyltransferase are associated with a poor prognosis.
Topics: Adenoma, Islet Cell; Adult; Aged; Alkaline Phosphatase; Apudoma; Carcinoid Tumor; Carcinoma, Medullary; Female; Humans; Male; Middle Aged; Mitoxantrone; Neoplasm Metastasis; Pancreatic Neoplasms; Survival Rate; Thyroid Neoplasms
PubMed: 7819024
DOI: 10.1038/bjc.1995.21 -
Annals of Surgery Jan 1977
Topics: Adenoma; Adrenal Gland Neoplasms; Carcinoma; Cushing Syndrome; Gastrointestinal Neoplasms; Hormones, Ectopic; Humans; Hyperplasia; Multiple Endocrine Neoplasia; Neoplasms; Neurosecretory Systems; Pancreatic Neoplasms; Paraneoplastic Endocrine Syndromes; Pituitary Neoplasms; Thyroid Neoplasms; Zollinger-Ellison Syndrome
PubMed: 12724
DOI: 10.1097/00000658-197701000-00001