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Query: UMLS:C0221002 (primary hyperparathyroidism)
 4,921 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the kindred with familial isolated hyperparathyroidism with parathyroid cancer. The proband was diagnosed as having primary hyperparathyroidism at age 43. The same disorder was also found in his daughter who had low bone mass. His son was found to have primary hyperparathyroidism by family screening. The pathological diagnosis of the resected parathyroid in both father and daughter was parathyroid cancer, and that in son was parathyroid adenoma. The right lower gland of the proband and the left lower gland of the son were present in thymus. No mutations were found in the sequences of MEN1 gene, hence gene(s) other than MEN1 gene may have contributed to the malignant potency in our cases.
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PMID:A case of familial isolated hyperparathyroidism with ectopic parathyroid cancer. 1160 67

Familial hyperparathyroidism and parathyroid carcinoma are rare diseases. A case of parathyroid carcinoma in an 8-year-old girl whose mother had previously undergone parathyroidectomy for primary hyperparathyroidism is reported. Parathyroid carcinoma in a preadolescent child has not been described previously, and may have a familial basis.
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PMID:Familial parathyroid carcinoma in a child. 1204 5

The parathyroid cancer is mentioned in literature with an incidence of 0.5-5% in the etiology of the primary hyperparathyroidism. The authors present the case of a 45 year old female with diagnosis of "primary hyperparathyroidism" based on clinical, ultrasonographic and biochemical investigations. A right superior adenoma of 3 x 2 x 1 cm (150 mg) has been found and extirpated. The histological examination showed an adenoma with predominant "chief" cells. Three years after surgery the patient had a recurrence of the clinical and hypercalcemie syndrome (with more severe damages). The surgical reexploration showed the right thyroid lobe with a nodular aspect and in its inferior pole a enlarged parathyroid gland of one cm size was found. An en-block exeresis including the right thyroid lobe with isthmus as well as the mentioned lesion and the half of left superior parathyroid, the left inferior one together with retrosternal fat tissue have been performed. Frozen sections completed by paraffin examination established the diagnosis of hyperfunctioning parathyroid carcinoma. We have emphasize the issues that could suggest the initially preoperative true diagnosis: the large size of excised adenoma and the relatively quick recurrence of the phenomena of parathyroid hyperfunction. The surgical principles and strategies in the treatment of parathyroid cancer are also discussed.
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PMID:[Hyperfunctional parathyroid carcinoma]. 1273 Nov 81

Parathyroid carcinoma accounts for 0.5 to 4.0% of cases of primary hyperparathyroidism. The prognosis depends largely on the extent of successful resection at the time of initial operation. Therefore, early diagnosis before surgery is important. We report 3 cases of primary hyperparathyroidism. The first patient, a 20-year-old uremic female, had refractory hypercalcemia after 5 years of hemodialysis treatment. Hypercalcemia persisted despite repeated parathyroidectomy. Pathology revealed diffuse hyperplasia of the parathyroid glands with focal adenomatous changes. Multiple pulmonary metastases were found later. The second patient, a 45-year-old female with history of nephrolithiasis, presented with severe osteoporosis. She underwent repeated parathyroidectomy for local recurrence. Pathology disclosed typical features of parathyroid carcinoma with adjacent lymph node metastasis. The third patient, a 34-year-old male, had recurrent episodes of extremity fracture and hypercalcemia with palpable neck mass. He underwent resection of the parathyroid tumor. Vascular and capsular invasions were noted microscopically. All three patients were relatively young and had extremely high intact parathyroid hormone (iPTH) level (15 to 31 times the upper limit of normal). The first patient died of hypercalcemia and respiratory failure and the other 2 were treated successfully with surgical excision and, in case 2, combined chemotherapy and radiotherapy. The latter 2 patients had no recurrence during 18 months and 14 months of follow-up, respectively. Our experience with these cases suggests that the combination of the following characteristics are highly suggestive of parathyroid carcinoma: young age, palpable neck mass, concomitant renal and skeletal disease, and extremely high iPTH level in patients with PTH-dependent hypercalcemia.
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PMID:Variability of clinical presentations in three cases of parathyroid carcinoma. 1283 92

In primary hyperparathyroidism, calcium homeostasis is disrupted by excessive synthesis and secretion of parathyroid hormone (PTH), which is usually caused by a solitary adenoma, or less often by nodular hyperplasia or carcinoma of the parathyroid glands. So far, the distinction between these forms of primary hyperparathyroidism has been made by histological examination. In this report clinical and histological findings, including PTH immunohistochemistry, are described in five dogs with primary hyperparathyroidism, three dogs with secondary hyperparathyroidism due to chronic renal failure, and eight control dogs. In the dogs with primary hyperparathyroidism, nodular adenomatous hyperplasia was found in two animals and parathyroid adenoma in three. The dogs with chronic renal failure had diffuse parathyroid gland hyperplasia. The parathyroid glands of the control dogs and the inactive cells surrounding the hyperplastic nodules showed slight to moderate, localized, paranuclear PTH immunolabelling. In the primary nodular and secondary diffuse hyperplasia, all parathyroid cells had a diffuse cytoplasmic PTH labelling pattern, sometimes in combination with localized paranuclear labelling. In parathyroid adenoma, areas with either paranuclear labelling or diffuse cytoplasmic labelling were observed. As both parathyroid adenoma and primary nodular parathyroid gland hyperplasia have characteristics of intrinsic autonomy (i.e., suppression of the remaining endocrine tissue), there would seem to be no functional difference between the two abnormalities. It is argued that primary (multi)nodular hyperplasia is a multiple form of parathyroid adenoma.
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PMID:Parathyroid hormone immunohistochemistry in dogs with primary and secondary hyperparathyroidism: the question of adenoma and primary hyperplasia. 1285 9

Parathyroid carcinoma is a rare cause of primary hyperparathyroidism and these tumours are usually hyperfunctional as opposed to other malignant endocrine tumors. Surgery is the only effective treatment while nonsurgical modalities yield poor results. We report a patient, who presented with palpable mass in the neck and severe hypercalcemia. He underwent debulking surgery and received allendronate, calcitonin, dacarbazine followed by in- situ alcohol instillation with some success.
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PMID:Parathyroid carcinoma: difficult management options. 1292 68

Parathyroid cancer presents a diagnostic challenge as a rare endocrine malignancy usually not recognized preoperatively and often not conclusively identified intraoperatively. We examined a cluster of parathyroid cancers treated at University of Louisville-affiliated hospitals during a 5-year interval. Clinical and histologic data from patient records at University Surgical Associates, the University of Louisville Hospital, Norton Hospital, and the Louisville Veterans Administration Medical Center in Louisville, KY were retrospectively reviewed. During the study interval surgical exploration of the neck was undertaken on 175 patients with primary hyperparathyroidism; four parathyroid malignancies (2%) were identified. Three of the four patients exhibited symptomatic hyperparathyroidism with very high diagnostic calcium and parathormone levels. All patients had multiple coexisting diseases and two had undergone previous parathyroid surgery. Sestamibi scan localized the lesion in two patients, ultrasound was used in one patient, and a positron emission tomography scan was needed to identify the lesion in the fourth. Intraoperative findings varied from multiple nodules involving the thyroid and paratracheal nodules to otherwise normal-appearing enlarged parathyroid gland. External pathologic review was needed to conclusively establish the diagnosis in all cases, even though initial histologic analysis was suggestive of malignancy. All patients are alive, well, and free of disease. This rarely occurring malignancy may be suggested by very high preoperative calcium parathormone levels. Intraoperative and histologic findings are often inconclusive resulting in therapeutic decisions made by the operating surgeon on the basis of limited or incomplete information.
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PMID:Parathyroid cancer, a continued diagnostic and therapeutic dilemma: report of four cases and review of the literature. 1295 30

Parathyroid cancer is rare but relatively frequent in Japan compared to Western countries. Surgical parathyroidectomy is the primary choice for radical treatment of primary hyperparathyroidism (pHPT), hence it is important to distinguish malignant from benign tumor in the determination of surgical indication as well as method of operation. However, it is not easy to diagnose parathyroid cancer prior to operation. In the present study, we analyzed the background data, biochemical data and bone mineral density (BMD) of 131 patients with pHPT (111 benign and 20 malignant). BMD of the lumbar spine and mid-radius was measured by dual-energy X-ray absorptiometry. Serum levels of calcium, alkaline phosphatase (ALP), and parathyroid hormone (PTH) were significantly higher in malignant group compared to benign one. The extent of elevation of mid PTH seemed to be higher than that of intact PTH in malignant group. Age-, gender-, and race-adjusted BMD of distal one-third of radius was significantly decreased in malignant group compared to benign one, although that of lumbar spine was not significantly different between the two groups, indicating that osteopenia was marked in the region which was rich in cortical bone in malignant group. On the other hand, serum levels of calcium, ALP, and mid PTH as well as age were selected as predictors of malignancy in univariate logistic regression analysis, while serum level of intact PTH was not selected. In conclusion, radial BMD was lower in malignant group compared to benign one in pHPT. Serum levels of calcium, ALP and mid PTH were useful to predict malignancy of affected parathyroid glands in pHPT patients.
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PMID:Trial to predict malignancy of affected parathyroid glands in primary hyperparathyroidism. 1461 8

Parathyroid carcinoma is a rare entity with an 0,5-1% of incidence on primary hyperparathyroidism (HPP) in literature. The very high values of calcium and parathormone (PTH) and the clinical aspects of hypercalcemia are the characteristics findings in these patients. We present our experience on 6 pts on 153 cases with HPP treated (3,9%). The clinical and diagnostic suspects are frequently intraoperative findings and the decision making for the surgeon is not always easy (parathyroidectomy with or without hemithyroidectomy, lymphadenectomy, surgical resection of other tissues). The mortality rate is high and we have registered three deaths at 8,14 and 64 months.
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PMID:[Parathyroid carcinoma: clinical aspects and therapy]. 1501 6

Recent progresses in the ability to obtain a secure diagnosis and preoperative localisation have resulted in a lower threshold for surgery of primary hyperparathyroidism. We questioned whether these trends have been accompanied by an improvement in surgical results, or changes in the profile of the disease among operated patients. From a total of 511 operations (499 patients), we retrospectively investigated the data from three successive periods of 10 years each: (1973-1982: 73 operations; (1983-1992): 155 operations; (1993-2002): 283 operations. Rates of surgical failure, defined as persistent hypercalcaemia at six months, have progressively declined: 6.8%, 1.3% and 0.7% respectively. There also has been a decline in the rates of permanent hypoparathyroidism or laryngeal nerve injury. However, these complications were highly influenced by the underlying pathology (surgery for single adenoma versus surgery for multiple gland disease) and by the need for concomitant thyroid surgery. Considering signs and symptoms, the frequency of kidney stones has declined from 50% to 29.7%, while the rate of patients diagnosed at routine screening has increased from 19% to 39%. The prevalence of parathyroid cancer among operated patients has successively declined from 6.8% to 1.3%, then 0% during the last period. Our data suggest that present improvement in the success rate of parathyroid surgery be partly due to improvement in preoperative localisation. Among imaging techniques, subtraction scintigraphy, based on the simultaneous recording of technetium-99m-sestamibi and iodine-123, provided the highest rate of accurate location (92.6%). Because this imaging technique depicted a majority of cases of multiple parathyroid gland disease at prospective evaluation (14 out of 15), we now use it to select appropriate cases for a focussed surgery under local anaesthesia, without the additional need for intraoperative PTH monitoring. The present good surgical results would justify surgery even for elderly or asymptomatic patients. Surgery carried out before appearance of symptoms seems beneficial. Only asymptomatic patients with a short life expectancy may be denied surgery.
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PMID:[Primary hyperparathyroidism: three decades of evolution in diagnostic and imaging techniques and advantages of early surgery]. 1514 84


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