Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0221002 (primary hyperparathyroidism)
 4,921 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Parathyroid carcinoma is a rare etiology of primary hyperparathyroidism characterized by severe clinical symptoms of hypercalcemia and markedly elevated serum parathormone levels. Nonfunctioning parathyroid carcinoma is an inactive form of the disease. There are less than 30 reports of nonfunctioning parathyroid carcinoma in the English literature and the association with parathyroid adenoma has not been described. This report describes a patient with concurrent nonfunctioning parathyroid carcinoma and a functioning parathyroid adenoma.
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PMID:Concurrent nonfunctioning parathyroid carcinoma and parathyroid adenoma. 1664 86

Parathyroid carcinoma usually develops in association with primary hyperparathyroidism. Only 18 cases have been reported in patients with secondary or tertiary renal hyperparathyroidism. We present a case of parathyroid carcinoma arising on transplanted parathyroid tissue after total parathyroidectomy for renal hyperparathyroidism.
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PMID:[Parathyroid carcinoma arising on transplanted parathyroid tissue after total parathyroidectomy for renal hyperparathyroidism]. 1676 5

Parathyroid carcinoma constitutes less than 1 % of primary hyperparathyroidism. The male to female ratio is approximately equal and the mean age at presentation is 40 years. In about half of the patients there is a palpable cervical mass, and serum calcium level is usually above 14 mg/dl. In a case report we present a 21-year-old man with a non-tender, non-mobile bulging mandibular mass. He suffered from fatigue, recent weight loss, hoarseness, polydipsia, polyuria, hematuria, recurrent renal stones and bouts of constipation and diarrhea. A mandibular biopsy confirmed brown tumor. Serum calcium level was 15.4 mg/dl. Cervical ultrasound revealed a hypoecho area suspicious of parathyroid adenoma. Parathyroid carcinoma was later confirmed and en bloc resection was performed. During a two-year follow up there has been no evidence of recurrence or hypercalcemia. This unique case of parathyroid carcinoma in conjunction with brown tumor is the second reported case worldwide.
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PMID:Parathyroid carcinoma: A rare case with mandibular brown tumor. 1677 84

We present case report of primary hyperparathyroidism treated surgically as well as a review of literature concerning this subject. The disease of not well known etiology presents with elevated parathormon levels and hypercalcemia. Primary hyperparathyroidism which states 85% percent of all kinds of hyperparathyroidism is usually parathyroid adenoma, in 11-15% glandular hyperplasia and in 1-4% parathyroid cancer. Clinical symptoms are muscle weakness and fatigue, nephrolithiasis, occasionally peptic ulcers, pancreatitis, hypertension. Laboratory test reveal increased level of PTH, hypercalcemia, elevated alkaline phosphatase levels and decreased phosphorus levels. Diagnostic imaging techniques such as ultrasonography, MRI or CT have sensitivity about 52-75%. Highest sensitivity in localization of ectopic parathyroid adenoma has sestamibi scintigraphy with technetium-99. Skeleton x-rays show typical changes in distal parts of bones and osteopenia. Treatment of choice is surgical excision of adenoma. Normalization of PTH and calcium levels after surgery and improvement of renal, musculoskeletal and circulatory system function could be achieved in 95%. Most common complications are recurrent laryngeal nerve injury, hypo- or hyperparathyroidism, bleeding or stridor.
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PMID:[Primary hyperparathyroidism--case report and review of the literature]. 1682 51

Parathyroid carcinoma is a very rare cause of primary hyperparathyroidism and these tumors are usually hyper-functioning as compared to other malignant endocrine tumors. Surgery is the only effective primary treatment. We report a patient, who presented with pathological fracture of femur, hypercalcemia, bilateral renal stones, markedly raised Parathormone levels and palpable mass in the neck. Parathyroid adenoma was initially diagnosed and localized at left lower gland by Sestamibi scan and ultrasonography. She underwent surgery and enlarged parathyroid gland was removed. Intra operatively there was no evidence of local invasion or lymph nodes involvement but biopsy report suggested malignancy.
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PMID:Parathyroid carcinoma. 1697 24

Primary hyperparathyroidism is a common disorder of mineral metabolism characterized by incompletely regulated, excessive secretion of parathyroid hormone from one or more of the parathyroid glands. In adults with the disease, a single, benign adenoma is seen approximately 80 percent of the time, with multiple gland involvement comprising most of the remaining patients. Very rarely, a parathyroid cancer is responsible but it is seen in less than 0.5 percent of patients with primary hyperparathyroidism. In this article, we will review important clinical and diagnostic features of asymptomatic primary hyperparathyroidism as well as considerations for surgical or medical management of the disease.
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PMID:Asymptomatic primary hyperparathyroidism. 1711 90

Parathyroid carcinoma (PC) is an uncommon finding, accounting for only 1-2% of patients with primary hyperparathyroidism (HPT), but a relatively higher incidence has been reported in Italy and Japan. The etiology of the tumour remains unclear, but molecular analysis studies have hypothesised the involvement of mutations of several genes in the pathogenesis of PC, including the oncogene cyclin Dl or PRADI located at the chromosome 13, the retinoblastoma and the p53 tumour suppressor gene. The clinical presentation of patients with PC is mainly related to the increased secretion of PTH rather than to the tumour burden. The pre-operative diagnosis of malignancy is very difficult to obtain, and, thus, intra-operative recognition of PC is mandatory. However, reliable signs of malignancy are rarely detectable. Probably, only vascular invasion, that correlates with tumour recurrence and metastases, should be considered useful in confirming malignancy, although both Ki-67 and Cyclin D1 have been recently used to aid in the definitive diagnosis. The en bloc resection of the tumour, together with ipsilateral thyroid lobe and adjacent structures, only if involved, avoiding any capsular rupture of the mass, represents the gold standard of surgical treatment of patients. Although the PC has traditionally been considered as a radioresistant tumour, there are some retrospective data holding a possible benefit from post-operative irradiation. No cytotoxic regimen with proven efficacy is currently available for patients with PC, but since hypercalcemia is ultimately the most frequent cause of death, several studies have suggested the usefulness of bisphosphonates (i.e., clodronate, pamidronate and zoledronate), calcitonin, and calcimimetic agents (i.e., cinacalcet) in patients with PC and severe hypercalcemia. In conclusion, PC is a rare malignancy and the NCDB survey reports an overall five- and ten-year survival rate of 85% and 49%, respectively. However, it is very difficult to predict the clinical behaviour of patients with PC and probably the ultimate prognosis depends on successful resection of the tumour at the initial surgery.
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PMID:Parathyroid cancer: etiology, clinical presentation and treatment. 1721 44

Measurement of bioactive parathyroid hormone (PTH) is essential for optimal management of bone abnormalities in dialysis patients. This can be accomplished by PTH measurements using third-generation PTH assays, which detect more or less of the first six amino acids of the PTH structure. Such assays do not detect non-(1-84) PTH fragments, such as human PTH (7-84), which are recognized by the second-generation PTH assays that use a detection antibody that recognizes an epitope within the 13-34 region of the PTH structure. Therefore, third-generation PTH results are expected to be lower than those that are obtained with second-generation PTH assays. Rare exceptions to this rule have been reported for patients with severe primary hyperparathyroidism or parathyroid cancer. Sera and gland extracts were analyzed from a dialysis patient with high bone turnover disease and with surprising higher PTH levels by a third-generation assay than by a second-generation assay. This finding normalized after the surgical removal of an enlarged gland with a single nodule, an advanced type of nodular hyperplasia. HPLC fractionation of sera and gland extracts revealed the overproduction and secretion of a PTH molecule with an intact amino-terminus structure distinct from (1-84) PTH. This form of PTH was readily detectable by third-generation PTH assays but was poorly reactive in second-generation PTH assays. Therefore, parathyroid glands with advanced uremic nodular hyperplasia may overproduce and secrete a novel, biologically active form of PTH with an intact 1-6 region but a presumably modified 12-18 region required for the detection in second-generation PTH assays.
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PMID:Overproduction and secretion of a novel amino-terminal form of parathyroid hormone from a severe type of parathyroid hyperplasia in uremia. 1769 55

Parathyroid carcinoma is a rare malignancy of the parathyroid glands, and is the cause of primary hyperparathyroidism in fewer than one percent of cases. Symptoms are mainly due to local compression or hypercalcaemia secondary to markedly elevated parathyroid hormone levels. A minority of patients remain asymptomatic. Mediastinal parathyroid cysts are infrequent and may or may not be functioning. We present an 84-year-old woman with a giant functioning cystic parathyroid carcinoma located in the middle mediastinum. We performed a thorough MEDLINE and LILACS database search on published cases of parathyroid carcinoma and functioning parathyroid cysts, and found no case report with identical features to the one presented here.
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PMID:Parathyroid carcinoma presenting as a giant mediastinal retrotracheal functioning cyst. 1797 83

Parathyroid carcinoma is a rare endocrine tumour accounting for only about one of every hundred cases of primary hyperparathyroidism. The aetiology of this cancer remains obscure but the recent studies have identified that some gene mutations may be involved in its pathogenesis. Most patients with parathyroid carcinoma suffer from symptoms related to marked hypercalcemia; the incidence of associated renal, bone, gastrointestinal, neuromuscular and psychological symptoms is much more frequent than in those with benign parathyroid adenomas. The course of patients with parathyroid carcinoma is variable. However, in more than 50% cases, patients experience persistent or recurrent disease due to regional or distant disease. The treatment of parathyroid malignancy is predominantly surgical, comprising an initial en bloc resection of the tumour and adjacent neck structures. Several studies have suggested the usefulness of pharmacotherapy in the palliative treatment of the debilitating symptoms of hypercalcemia. The aim of this paper is to summarise the present state of knowledge on the aetiology, clinical presentation, diagnosis and treatment of parathyroid carcinoma.
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PMID:[Parathyroid carcinoma]. 1804 48


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