Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0031511 (pheochromocytoma)
 14,622 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Multiple endocrine neoplasia, type IIb (MEN IIb) is a rare syndrome characterized by the occurrence of medullary thyroid carcinoma (MTC), pheochromocytoma and mucosal neuroma. A 35-year-old male patient with MEM IIb having megacolon, marfanoid habitus and no family history of the disease underwent surgery. Because MTC was present in both lobes, total thyroidectomy and modified neck dissection were performed. Pheochromocytoma was found bilaterally and bilateral adrenalectomy with adrenal autotransplantation in the rectus abdominis muscle was carried out. Postoperative course was satisfactory except for transient hypocalcemia and mild ileus. After the slow corticosteroid weaning process, his adrenocortical function was at the lower level within a normal range. In August 1986 (24 postoperative months), he was maintained by the administration of 10mg of hydrocortisone every three days, and calcitonin and CEA levels in sera were normal. We collected 15 cases reported in Japanese literatures. MTC and mucosal neuroma were found in all cases, whereas pheochromocytoma was present in 9 cases. Bilateral and multicentric occurrences were usual, and total thyroidectomy and bilateral adrenalectomy were, warranted. We believe that autotransplantation following bilateral adrenalectomy is a worthy alternative.
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PMID:[An operated case of multiple endocrine neoplasia type IIb and review of the Japanese literature]. 289 22

Fifty meta-iodobenzylguanidine (MIBG) scintiscans were performed in three groups of medullary thyroid cancer (MTC) patients. Group 1 (n = 11) included treated patients with normal calcitonin levels; Group 2 (n = 24) included patients with elevated calcitonin levels due to sporadic and isolated MTC; Group 3 (n = 15) included patients with elevated calcitonin levels due to familial MTC or multiple endocrine neoplasia Type IIA syndrome (MEN). In Group 1 three pheochromocytoma were depicted by MIBG scintiscan. In Group 2 MTC was seen in a small number of patients (3 of 24). In Group 3, besides adrenal hyperplasia and pheochromocytoma four patients, MIBG scintigraphy showed where MTC had localized and spread in almost half of patients (7 of 15). MIBG uptake occurred in patients with relatively high calcitonin level (greater than 0.6 nmol/l). These data indicate that in patients with familial MTC or MEN syndrome, MIBG scintiscan can be useful not only in detecting associated pheochromocytoma, but also in showing MTC.
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PMID:Radioiodinated meta-iodobenzylguanidine uptake in medullary thyroid cancer. A French cooperative study. 289 39

We have studied the clinical and thyroid immunohistological features of 19 patients with sporadic medullary thyroid carcinoma and 16 patients with the hereditary syndrome multiple endocrine neoplasia 2a (MEN 2a). Both groups were identified by family screening using serum calcitonin determinations before and after pentagastrin stimulation. Pheochromocytoma and hyperparathyroidism were associated both with multiple endocrine neoplasia 2a and some cases of sporadic medullary thyroid carcinoma. Hereditary medullary thyroid carcinoma was invariably associated with C-cell hyperplasia, but C-cell hyperplasia was also associated with some sporadic tumours. All tumours were positive for calcitonin and carcinoembryonic antigen (by immunohistological staining) (CEA) and most tumours stained for somatostatin. C-cell hyperplasia also stained for calcitonin, CEA and somatostatin. We conclude that sporadic and familial medullary thyroid carcinoma cannot always be discriminated by clinical or immunohistological methods. Family screening is essential in the diagnosis of hereditary medullary thyroid carcinoma.
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PMID:Familial and sporadic medullary thyroid carcinoma: clinical and immunohistological findings. 290 73

Medullary Ca of the thyroid (MCT) occurs in sporadic and familial form. When familial MCT is associated with pheochromocytoma and parathyroid adenoma or hyperplasia it is called multiple endocrine syndrome type IIa (MEN type IIa). When the syndrome is associated with mucosal neuromata or marfanoid habitus it is called MEN type IIb or type III. When familial MCT is not associated with the above tumors, it is called type IV. Measurement of serum calcitonin is the diagnostic method of choice. CEA is a better prognostic marker than calcitonin. When MCT is confined to the thyroid, total thyroidectomy is recommended. If lymph nodes are involved, lymph node neck dissection is required. I131 therapy and radiotherapy are ineffective. Optimal chemotherapy has not yet been established.
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PMID:Medullary carcinoma of the thyroid: differentiating the types and current management. 290 13

A 65-year-old woman presenting with back pain, difficulties in walking and watery diarrhea. A right adrenal tumor and high excretion of catecholamines were found. Laboratory examinations showed raised levels of vasoactive intestinal polypeptide, pancreatic polypeptide, gastrin and calcitonin. Histology showed a combined pheochromocytoma-ganglioneuroma. The neoplastic cell population was immunohistochemically shown to contain tyrosine hydroxylase, neuropeptide Y, met-enkephalin, substance P, vasoactive intestinal polypeptide, calcitonin and calcitonin gene-related peptide. Postoperatively, the patient recovered fully and the hormone levels returned to normal.
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PMID:Adrenal pheochromocytoma-ganglioneuroma producing catecholamines and various neuropeptides. 318 92

Evident progress has been made in the treatment of pheochromocytoma. The results of hormone analysis became very accurate, the method for tumor localisation are non invasive and safe: with 131-I-MIBG, ultrasonography and CT-scan an exact preoperative localisation is possible without serious risks. Patients are prepared for the operation with alpha- and beta-blocking agents. Modern methods of anaesthesia with continuous monitoring of blood pressure, pulmonary pressure and cardiac output and a standardized operative procedure are essential. From 1965 to 1987 71 patients with a total of 87 catecholamine producing tumors have been operated. In all cases a transabdominal access was chosen. Biadrenal tumors were removed in 8 patients, multiple (7) tumors in 2 patients. The comparison of the 2 time intervals 1965 to 1976 and 1977 to 1987 showed a significant decrease of serious intra- and postoperative complications. Surgical specimens of 36 patients with pheochromocytoma were used for immunohistologic evaluation. Marked positivity was found in 44% of cases for calcitonin. The reaction for vasoactive intestinal polypeptide (VIP) was positive in 28% of cases. Somatostatin was not detected in any case, neuron-specific enolase (NSE) in all cases. 6 patients with malignant pheochromocytoma were treated with high doses of 131-I-MIBG, 4 other patients received a combined chemotherapy.
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PMID:[Treatment of pheochromocytoma: changes in diagnosis and therapy]. 321 83

Synthesis and secretion of calcitonin and calcitonin gene-related peptide (CGRP) were studied in medullary thyroid carcinomas (MTC) by hybridization histochemistry on tissue sections and by Northern gel analysis of mRNA. Five patients with MTC and elevated serum levels of calcitonin and CGRP were studied. Surgically obtained tumor samples (four primary and three lymph node metastases) were extracted after freezing, and the RNA was fractionated on Northern gels. Hybridization was carried out with 32P-labeled synthetic oligodeoxyribonucleotides coding specifically for calcitonin and CGRP. Calcitonin- and CGRP-specific mRNAs approximately 1000 nucleotides in length were demonstrated in all 7 tumor samples. However, neither calcitonin nor CGRP mRNA was detected in a pheochromocytoma from 1 of the patients who had multiple endocrine neoplasia type II. A series of unselected lung carcinomas yielded the same result. Hybridization histochemistry was carried out on sections from the same tumors using the same probes. The mRNAs for calcitonin and CGRP were located in all cells of neoplastic MTC appearance, with CGRP mRNA at significantly lower levels. This demonstrated that both calcitonin and CGRP mRNA were present within the same tumor cells. The lung tumors and pheochromocytoma were negative with both probes. Hybridization histochemistry is likely to be of use in diagnosis of medullary thyroid cancer and in studying the calcitonin-CGRP mRNA processing mechanism in whole cells.
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PMID:Identification of calcitonin and calcitonin gene-related peptide messenger ribonucleic acid in medullary thyroid carcinomas by hybridization histochemistry. 348 48

Expression of the calcitonin (CT)/calcitonin gene related peptide (CGRP) gene and the proopiomelanocortin (POMC) gene has been demonstrated by Northern blot hybridization analysis of RNA extracted from human medullary thyroid carcinoma (MTC), pheochromocytoma and lung carcinoma. CT mRNA in these tumors could not be distinguished in size from CT mRNA isolated from normal human thyroid tissue. CGRP mRNA (previously demonstrated in 12 out of 12 lung tumor cell lines investigated) could not be detected in 13 primary lung tumors or 10 metastases thereof. The length of POMC mRNA in MTCs (present in all 4 metastases investigated but not in 7 primary tumors) and pheochromocytomas is about 100 nucleotides more than pituitary POMC RNA. In lung tumors 2 POMC RNA species can be detected, one of the same size as in pituitary tissue and one about 100 nucleotides larger.
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PMID:Detection of mRNA encoding calcitonin, calcitonin gene related peptide and proopiomelanocortin in human tumors. 348 30

Serum calcitonin was determined by RIA in 59 healthy subjects (Group 1), 49 randomly selected patients with treated or untreated thyroid disorders (Group 2), and in 12 kindred of a pheochromocytoma index case (Group 3). Although most subjects in Group 2 had normal calcitonin levels, there were significant (p less than 0.001) differences between all three groups. Of the five patients in Group 2 with high serum calcitonin, one had medullary cancer of the thyroid, one had multiple endocrine neoplasia, one had acromegaly, and two remained undiagnosed. Increased serum calcitonin levels were also found in seven of 12 normotensive relatives of a patient with pheochromocytoma. It is therefore concluded that high serum calcitonin levels in patients with thyroid disorders strongly suggest the presence of C-cell neoplasia or medullary cancer of the thyroid.
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PMID:Serum calcitonin in thyroid disorders and in pheochromocytoma kindred. 396 31

A family with MEN IIa (medullary thyroid carcinoma (MCT), pheochromocytoma and hyperparathyroidism) was identified. Three relatives had been treated for MCT earlier. Eleven asymptomatic family members had elevated pentagastrin (PG)-stimulated serum immunoreactive calcitonin (i-CT) concentrations, including one who earlier had a pheochromocytoma removed. Nine of these subjects underwent thyroidectomy, and histological examination revealed multifocal MCT in all. Although surgery was judged complete in all, elevated PG-stimulated serum i-CT levels postoperatively indicated residual disease in 5. The natural history of MCTs in the present family varied, with most cases behaving benignly. Occasionally, however, the disease pursued an aggressive course. As MCT often metastasizes before being clinically evident, high cure rates can only be obtained by early diagnosis and treatment, possibly in the pre-metastasizing phase of C-cell hyperplasia, detectable only by elevated PG-stimulated serum i-CT levels.
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PMID:Familial medullary thyroid carcinoma in multiple endocrine neoplasia (MEN) IIa: diagnosis and problems in treatment. 612 16


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