Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0001430 (adenoma)
 21,222 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A father and son each presented with severe watery diarrhea. The son was found to have a pancreatic islet-cell tumor associated with the pancreatic cholera syndrome, as well as a parathyroid adenoma. The father was found to have multiple islet-cell adenomas and the Zollinger-Ellison syndrome. Pancreatic tumor tissue from each patient contained detectable gastrin and vasoactive intestinal peptide; however, a much higher gastrin concentration was found in the tumor tissue from the father and a much higher vasoactive intestinal peptide content in the tumor tissue from the son. Thus, watery diarrhea may be mediated by different hormones in families having multiple endocrine neoplasia; the precise cause of the diarrheal syndrome should be defined to ensure the proper therapy.
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PMID:Hormone-mediated watery diarrhea in a family with multiple endocrine neoplasms. 22 Aug 98

Investigations are presented on the occurrence of tumour products during combination chemotherapy of a 49-year-old female with an APUDoma metastatic to the liver. Calcitonin was demonstrated in high concentration in the tumour tissue. Serum calcitonin, serum histaminase and 5-HIAA in a 24-hour urine sample increased immediately after the administration of cytotoxic agents, falling subsequently below the pretreatment level. These findings indicate a therapeutic effect with lysis of tumour cells. Continuous determination of the three tumour substances showed an increase in these products before clinical suspicion of progression. Electron microscopic examination during the initial course disclosed the tumour to be an APUDoma. Autopsy failed to disclose a primary site outside the liver. Further autopsy findings were an adenoma of the thyroid and a chromofobe adenoma of the pituitary, thus assigning the patient to type 1 multiple endocrine neoplasia.
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PMID:Combination chemotherapy of an APUDoma. With special reference to the therapeutic value of monitoring hormonal substances. 40 2

Thirty-two patients with medullary carcinoma of the thyroid (MCT) were studied before and after therapy. Sixteen patients had familial and 16 had the sporadic type of disease. The groups differed in several features: Patients with sporadic disease were older at diagnosis and presented with enlargements in the thyroid or lymph nodes, and one patient had neuromata; in the familial group the disease was more often bilateral and was associated at times with the MEA syndrome (parathyroid adenoma or pheochromocytomas, or both). The immunoreactive serum calcitonin (CT) level was measured before and after some form of therapy in all 32 patients. Our investigations showed: 1) Delaying treatment was clearly detrimental in this progressive disease; 2) The most effective therapy was surgery, while radiotherapy and chemotherapy were less effective; 3) Postoperatively, the CT level usually fell sharply, when the disease was thought extirpated, but the lowest nadir might be reached at from 1 month to 6 years; 4) In all such patients the CT level eventually rose from the postoperative nadir; 5) Patients with no clinical or radiological evidence of disease, had high CT levels for a mean of 3 years of observation; 6) A marked rise of CT levels in three patients preceded metastases and death; 7) Patients with abdominal, particularly liver disease had the highest CT levels; and 8) Patients who died had relatively high CT levels, corresponding in general with the extent of disease.
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PMID:Medullary thyroid carcinoma: importance of serial serum calcitonin measurement. 42 24

A 62 year old female died of a chromophobe pituitary adenoma of the sphenoid bone. A normal pituitary gland was present within the sella turcica. She also had a chief cell parathyroid adenoma, a papillary thyroid carcinoma, and an ovarian thecoma (multiple endocrine neoplasia syndrome).
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PMID:Sphenoidal pituitary adenoma. 42 97

Of 51 patients with primary hyperparathyroidism (2 patients with MEN, Type 1 clinical symptomatology, diagnostic procedures, differential diagnosis, operative strategy and long-term results are being reported. Aside from clinical findings and radiologic signs in our hands determination of the ionized serum calcium fraction, results of chrest bone biopsies and parathormone determinations are best parameters to substantiate the diagnosis of PHPT. Parathormone radioimmunassay determination is very helpful in localizing the adenoma, especially in cases of reoperations. Five patients were seen in acute hypercalcemic crises, in which emergency operations are absolutely indicated. Postoperative hypercalcemia and recurrencies were observed in 3.9%. Successful extirpation of parathyroid adenomas (15% multiple adenomas were found) is the therapy of choice in PHPT, only in cases with hyperplasia subtotal parathyroidectomy is indicated.
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PMID:[Diagnosis and therapy of primary hyperparathyroidism (author's transl)]. 72 76

In 82 patients, a preoperative diagnosis of primary hyperparathyroidism has been established by means of transfemoral neck vein catheterization and measurement of serum immunoreactive parathyroid hormone (iPTH). Twenty-five of these patients have had cancer in other parts of the body but with no evidence of recurrence or metastasis. One patient had carcinoma of the colon with metastases, and four were members of families with multiple endocrine adenomatosis (MEA, Types I and II). In six other hypercalcemic patients, high levels of iPTH were found also in the effluent blood from cancer sites other than the parathyroid gland, secondary to ectopic hormone production or pseudohyperparathyroidism. In addition, a high serum level of iPTH was found in the superior vena cava of a seventh patient who had carcinoma of the breast but no clinical or radiological signs of recurrence or metastasis with the exception of an enlarged liver. This iPTH finding was interpreted as being, probably, the result of parathyroid adenoma in either the neck or the mediastinum. At the time of operation, a transcervical mediastinal search was made. Four normal cervical parathyroid glands were found; three were removed. Hypercalcemia persisted after operation, and the patient died. At postmortem examination, microscopic study revealed that the disease had metastasized to lungs and hilar lymph nodes. There was massive metastasis in the liver; the liver contained a large amount of iPTH. The results of these investigations suggest that (1) venous catheterization of the neck veins and the effluent blood from extraparathyroid tumors aid in identifying and localizing iPTH production; (2) primary benign hyperparathyroidism is not uncommon in patients with cancer, and its co-existence must be recognized; (3) high serum iPTH level in the superior vena cava may be found in patients with metastatic or primary cancer of the thoracic cavity; and (4) hyperparathyroidism may be the first hint of a familial multiple endocrine syndrome.
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PMID:Hypercalcemia in patients with known malignent disease. 96 5

1. Three members of a kindred and a fourth unrelated individual demonstrating the syndrome of mucosal neuroma, bumpy lips, marfanoid habitus, medullary thyroid carcinoma and pheochromocytoma are reported, and the literature pertaining to this syndrome has been reviewed. 2. There are currently 41 definite and 16 additional probable reported cases manifesting the syndrome of mucosal neuroma, bumpy lips, pheochromocytoma and medullary thyroid carcinoma. Mucosal neuroma was present in all patients. Medullary thyroid carcinoma was histologically proved in 38 cases. Pheochromocytoma was documented in 19 patients with a probable diagnois in another 4 patients. Only one patient was noted to have hypercalcemia associated with parathyroid adenoma. Associated abnormalities seen in this syndrome include hypertrophied corneal nerves, skeletal defects and gastrointestinal tract abnormalities. 3. The relationship of this syndrome to other types of multiple endocrine neoplasia syndromes and the phakomatoses is also discussed. This syndrome appears to be distinct from the entity of multiple endocrine neoplasia type 2. We suggest that this syndrome be called multiple endocrine neoplasia type 3, following the classification originally proposed by Steiner et al. 4. MEN type 3 appears to be inherited as an autosomal dominant disorder. Many apparently non-familial cases have been reported, but due to inadequate information regarding family history it may be possible that some of these cases also had other affected family members. 5. This syndrome most likely results from a dysplasia of neuroectodermal tissue. The pathogenesis of this syndrome is discussed and evidence supporting the hypothesis is reviewed. 6. The importance of diagnosing the syndrome at an early stage and of investigating the relatives of a patient manifesting this potentially fatal syndrome are stressed. 7. Plasma calcitonin measurement following calcium infusion is extremely useful as a screening procedure for the diagnosis of medullary thyroid carcinoma, when the patients are completely asymptomatic and routine thyroid function tests are normal. Affected individuals should also be periodically screened for the development of pheochromocytoma.
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PMID:Mucosal neuroma, pheochromocytoma and medullary thyroid carcinoma: multiple endocrine neoplasia type 3. 111 36

In a 20 year period, from 1971 through 1991, 105 chromaffin tumours--excluding cervical ones--were operated by the same surgeon: 50 during the first 15 years and 55 during the last 5 years. Pheochromocytomas are defined as intra-adrenal chromaffin tumours, and paragangliomas--or ectopic pheochromocytomas--as of extra-adrenal location. Among those tumours, 30 were malignant (i.e. metastatic) and 75 benign. Among the 30 malignant tumours, 14 were ectopic, 2 occurred in a MEN II A setting and were bilateral, 2 were associated with liver adenoma and liver hemangioma respectively suggesting Von Hippel-Lindau syndrome, and one case was associated with a seemingly sporadic primary hyperparathyroidism. 9 out of those 30 malignancies were not associated with hypertension. Among 75 benign pheochromocytomas or paragangliomas, 10 were ectopic, 7 occurred in a MEN setting (6 type II, 1 type I). 3 patients without evidence of MEN or other neuroectodermal abnormalities presented bilateral pheochromocytoma, either synchronous (2) or metachronous (1). 7 cases occurred in a Von Hippel-Lindau syndrome (3 bilateral) and 4 in a neurofibromatosis setting (1 bilateral). 3 other cases were familial without evidence of MEN (including a case of triple tumour: bilateral and ectopic and another ectopic case). 2 other cases were associated with seemingly sporadic hyperparathyroidism. As a whole, in 34 of 75 benign pheochromocytomas or paragangliomas, the tumour was not intra-adrenal, unilateral and sporadic. Among those 75 tumours, 22 were not overtly hypertensive, including 10 out of the 41 seemingly intra-adrenal, solitary and sporadic. The pheochromocytoma, benign, intra-adrenal sporadic, hypertensive accounts for no more than 30% of the subphrenic catecholamine-secretin chromaffin tumours.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Can pheochromocytoma be considered a benign unilateral intra-adrenal sporadic hypertensive tumor ? Reflections on a series of 105 surgically treated subdiaphragmatic chromaffin tumors]. 134 86

Strumal carcinoid is an unusual form of monodermal ovarian teratoma with thyroid-like follicles admixed with typical carcinoid tumor patterns. We encountered a case of this neoplasm in a patient with multiple endocrine neoplasia, type IIA (Sipple's syndrome), including a medullary thyroid carcinoma diagnosed 24 years previously. During evaluation of bilateral adrenal pheochromocytomas, a unilateral left ovarian strumal carcinoid was discovered. Subsequently, the patient had a parathyroid adenoma excised. The ovarian tumor was immunohistochemically reactive for neuron-specific enolase, chromogranin, synaptophysin, and serotonin, but did not stain for calcitonin. The follicular structures stained for thyroglobulin. This unusual case shows that ovarian strumal carcinoid, like carcinoid tumors at other sites, may arise in association with multiple endocrine neoplasia.
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PMID:Ovarian strumal carcinoid in association with multiple endocrine neoplasia, type IIA. 134 63

We report the characterization of a dense cluster of CpG islands at D10S94 in proximal 10q11.2. D10S94 is tightly linked to the gene responsible for multiple endocrine neoplasia type 2A (MEN 2A), a dominantly inherited tumor syndrome characterized by medullary thyroid carcinoma (MTC), pheochromocytoma, and/or parathyroid adenoma. To date, no recombinants between D10S94 and MEN2A have been identified. The gene(s) responsible for two additional dominantly inherited disorders involving cancer of the medullary thyroid, MEN 2B (MEN2B), and dominantly inherited MTC without additional clinical features (MTC1), also map to this region. The gene or genes responsible for these disorders may be located at or near the D10S94 locus. A 570-kb long-range restriction map has been generated by pulsed-field gel electrophoresis using probes developed during a 160-kb bidirectional cosmid walk at D10S94. Six CpG islands are clustered within a 180-kb region; five fall within a 145-kb NotI restriction fragment that is contained in its entirety in our cosmid contig. The SacII, SfiI, and NotI restriction maps for lymphoblast and cloned DNA are concordant. These CpG islands may represent the 5' ends of candidate genes for MEN2A, MEN2B, and/or MTC1. One gene designated mcs94-1, which is associated with one of the CpG islands in this cluster, has been isolated and characterized in detail.
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PMID:A cluster of CpG islands at D10S94, near the locus responsible for multiple endocrine neoplasia type 2A (MEN2A). 135 67


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