[Preoperative diagnosis of parathyroid neoplasms by means of stepwise supraselective blood collection for the determination of the parathyroid hormone level].
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Over a 25-year period, two carcinomas of the parathyroid were observed in 67 cases of primary hyperparathyroidism. The most important signs and symptoms were bone disease, palpable neck metastases, renal stones, and hypercalceamia with high blood levels of parathyroid hormone. Histology revealed that in principle parathyroid carcinoma can be distinguished from adenoma by a trabecular pattern and thick fibrous bands. The presence of cellular atypia and variation or mitotic figures (regressive polymorphia) was not a useful criteria for carcinoma. Local recurrence occurred in both cases.
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The importance of superselective catheterization of the small jugular veins for PTH-radioimmunoassay is shown as an improvement of the preoperative localization technique. Since May 1976 in 20 patients the diagnosis of pHPT could be established. Our own experiences with the method of small vein sampling combined with the radioimmunoassay demonstrate that a reliable localization of overactive parathyroid tumours is possible.
Parathyroid carcinoma (PC) is a rare but clinically aggressive endocrine malignancy with limited treatment options and a poorly defined tumor microenvironment (TME). To elucidate its cellular heterogeneity and spatial architecture, we integrated single-cell and spatial transcriptomic profiling with whole-exome sequencing and multiplex immunohistochemistry on eight parathyroid neoplasm specimens, including PC, parathyroid adenoma, and atypical parathyroid tumor. We identified a distinct progenitor-like endocrine subpopulation (Ca-1) enriched in CDC73-mutant PC, exhibiting stem-like properties, elevated cell cycle activity, and pronounced genomic instability. Spatial mapping revealed that Ca-1 cells preferentially localize at the leading edge, forming a fibroinflammatory niche characterized by the enrichment of inflammatory cancer-associated fibroblasts (iCAFs) and SPP1+ macrophages. Within this niche, the dipeptidyl peptidase 4 (DPP4) is selectively expressed in Ca-1 cells and iCAFs, implicating a potential paracrine axis driving stromal remodeling and immunosuppression. These findings suggest that a spatially organized ecosystem may promote PC progression through TME remodeling and highlight the DPP4-CXCL2 axis as a candidate pathway for future investigation in aggressive parathyroid neoplasms.
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Big corticotropin (adrenocroticotropic hormone, ACTH), an immunoreactive form of ACTH with low biological activity and which elutes in the void volume on Sephadex G-50 gel filtration, is found in plasma and extracts of human pituitary and tumour. Controlled tryptic digestion of big ACTH releases a product with full corticotropic activity which is indistinguishable from the (1-39) ACTH with respect to size, charge and susceptibility to tryptic digestion. Immunoreactive ACTH, predominantly in the big form, is found in virtually all tissue extracts of carcinoma primary to or metastatic from the lung, but not of carcinoma metastatic to the lung, and even in precancerous lung lesions. The absence of clinical Cushing syndrome in patients with carcinoma of the lung and moderate elevation of plasma concentrations of ACTH is due to the low biological activity of big ACTH. Prolonged survival (for more than two years) of patients with lung carcinoma has been observed only in those whose plasma ACTH is low before therapy or after resection of the lung tumour. Rabbit, rat and mouse pituitaries contain an intermediate sized ACTH but the usual 1-39 peptide predominates in the pituitaries of monkey, sheep, dog, cat and guinea pig, as well as man. The hormonal form of ACTH appears to be an important factor regulating the cortisol/corticosterone ratio in mammalian adrenal corticoid secretion because administration of porcine ACTH to rabbits alters the adrenal secretory pattern so as to decrease corticosterone production and increase cortisol production.
The radiographic triad of a calcified pancreatic mass, duodenal ulcers, and nephrocalcinosis should suggest the Zollinger-Ellison syndrome, as part of the multiple endocrine adenomatosis complex. The clinical and radiographic features of the entity are described in this case report.
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Multiple endocrine neoplasia, type 2b, is a designation that has been proposed for the combination of medullary thyroid carcinoma, pheochromocytoma, mucosal neuromas, and somatic abnormalities. The neural lesions produce a characteristic diffuse or nodular enlargement of lips, tongue, and buccal mucosa. Microscopically, the lesions are composed of unencapsulated masses of hypertrophied and elongated nerves in which are found rare ganglion cells. The histologic similarity between the oral mucosal lesions and the alimentary tract ganglioneuromatosis in the syndrome suggests that the term ganglioneuromatosis is the most appropriate designation for the oral lesions. The oral lesions signal high risk for two potentially lethal neoplasms--medullary thyroid carcinoma and pheochromocytoma. Thus, patients having the characteristic findings are in urgent need of repeated evaluation of thyroidal C-cell and adrenal medullary function.
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The familial aggregation of peptic ulcer disease has been well established, as has its association with such clear-cut genetic factors as blood group O and nonsecretor status. However, the genetics of this disorder, or group of disorders, is still in question. Polygenic inheritance is the prevailing hypothesis that has been proposed for peptic ulcer. This hypothesis was based primarily on the exclusion of a simple mode of inheritance for all ulcer disease. Genetic heterogeneity is an alternative hypothesis that can explain both the familial aggregation of peptic ulcer disease and the lack of a simple Mendelian pattern of inheritance. The evidence for genetic heterogeneity in peptic ulcer disease is reviewed, and studies are proposed to test this hypothesis.
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