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[Primary hyperparathyroidism with severe skeletal involvement in one patient with parathyroid carcinoma].

Parathyroid carcinoma is an uncommon cause of primary hyperparathyroidism; however, when this condition is severe, cancer must be suspected. We report on a 28-year-old male with severe hypercalcemia, cachexia, acute pancreatitis, urolithiasis, anemia and a severe skeletal involvement with multiple fractures. The patient had a 4-cm parathyroid tumor, that was surgically excised, along with the ipsilateral thyroid lobe. During the postoperative period, he had a severe and prolonged hungry bone syndrome, with a slow recovery of fractures, with functional and anatomical sequelae in the extremities. PTH levels were adequate for the serum calcium during the 16 months of follow-up.

Adult↗

Parathyroid carcinoma.

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.

Adenoma↗

Identification of parathyroid hormone messenger ribonucleic acid in an apparently nonfunctioning parathyroid carcinoma transformed from a parathyroid carcinoma with hyperparathyroidism.

mRNA coding for pre-pro-PTH, a precursor of PTH, was sought in an apparently nonfunctioning parathyroid carcinoma that had transformed from one that was previously functioning. Total poly(A+) RNA was prepared by phenol-chloroform-isoamyl alcohol extraction and oligo-dT-cellulose affinity chromatography from the tumor tissue and bovine parathyroid glands. In the rabbit reticulocyte lysate cell-free translation system, total poly(A+) RNA from the tumor as well as that from bovine parathyroid glands directed the translation of a product which was specifically precipitated by an anti-PTH serum and which migrated at the same position as pre-pro-PTH on sodium dodecyl sulfate-polyacrylamide gel electrophoresis. These results indicated the presence of mRNA coding for pre-pro-PTH (PTH mRNA) in an apparently nonfunctioning parathyroid carcinoma, suggesting that PTH synthesis is not always absent in parathyroid carcinomas which are not accompanied by hyperparathyroidism.

Animals↗

Providing optimal preoperative localization for recurrent parathyroid carcinoma: a combined parathyroid scintigraphy and computed tomography approach.

PURPOSE: The incidence of parathyroid carcinoma is approximately 0.5% to 5% in patients with primary hyperparathyroidism. Recurrent parathyroid carcinoma is treated with surgical resection of all sites of disease to ameliorate systemic manifestations of hyperparathyroidism, primarily hypercalcemia. This study investigates the role of parathyroid scintigraphy and computed tomography (CT) imaging in recurrent parathyroid carcinoma. MATERIALS AND METHODS: A retrospective chart review was performed on 8 patients diagnosed with recurrent parathyroid carcinoma at our tertiary care institution between 1975 and 2001. Surgical reports, histopathology, parathyroid scintigraphy, and CT findings were recorded. Surgical reports and radiologic studies were compared for concordance of recurrence sites. RESULTS: There were 32 imaging studies before reoperation: 15 parathyroid scintigraphy and 17 CTs. Of 15 sites of recurrence potentially seen on scintigraphy, 10 were true-positive (67%). Of 17 sites of recurrence potentially seen on CT, 9 were true-positive (53%). Of the 8 false-negatives on CT, 7 of these recurrences were in the surgical bed (88%). There were 9 instances in which CT and scintigraphy were performed preoperatively for comparison and correlation. CT and scintigraphic findings were incongruent in 7 of 9 of these cases (78%). CONCLUSION: Successful surgical intervention for recurrent parathyroid carcinoma requires accurate preoperative localization studies and complete excision of metastases. Our data supports combined analysis of parathyroid scintigraphy and CT for patients with recurrent disease before reoperation. Additionally, our review suggests that sensitivity may be optimized with SPECT parathyroid scintigraphy and close correlation with CT.

Aged↗

Parathyroid carcinomas: can clinical outcomes for parathyroid carcinomas be determined by histologic evaluation alone?

The Fifth Annual Meeting of the Japan Endocrine Pathology Society was held in September 2001. A highlight of the meeting was the participation of Japanese and American endocrine pathologists in a conference to consider the topic "The Determination of Clinical Outcomes for Parathyroid Carcinomas by Histological Evaluation of Invasive Growth." The discussion was centered around three cases of parathyroid carcinomas selected by the coordinator, Dr. Kameyama, and discussed by Drs. Ronald DeLellis, Ricardo Lloyd, and Kennichi Kakudo. In this article, we summarize their diagnoses and comments.

Adult↗

Parathyroid carcinoma.

Although parathyroid neoplasms are common and cause primary hyperparathyroidism, parathyroid carcinoma is a rare entity. At times it can be difficult to diagnose. Patients with parathyroid carcinoma usually present with profound symptoms of hyperparathyroidism and highly elevated serum calcium and parathyroid hormone (PTH) levels. At the time of neck exploration, a large, gray-white, locally invasive tumor is commonly encountered. The course of patients with parathyroid carcinoma is variable; unfortunately, more than 50% have persistent or recurrent disease due to regional or distant disease. Surgical resection is the principal treatment for patients with parathyroid carcinoma. The optimal surgical treatment is en bloc tumor resection with ipsilateral thyroid lobectomy when the diagnosis is suspected and until it is proven otherwise. Patients who have persistent or recurrent parathyroid carcinoma should have localizing studies to identify loco-regional or distant tumor sites. Reoperation in patients with localized parathyroid carcinoma is recommended because it relieves symptoms of hypercalcemia, and it normalizes serum calcium and PTH levels in most patients. For patients who have unresectable parathyroid carcinoma, a protocol-based treatment with chemotherapy and external radiotherapy should be considered. Additionally, second-generation bisphosphonates and the NPS R-568 calcimimetic agent may be useful in normalizing the serum calcium and improving symptoms of hypercalcemia. However, they do not treat the tumor and are rarely effective in the long term.

Adenocarcinoma↗

Parathyroid carcinoma.

INTRODUCTION: Parathyroid carcinoma is a rare tumor with an incidence of 0.5 to 4%. Preoperative differentiation from benign adenoma is very difficult. Several features, though nonspecific, have been used in differentiating parathyroid carcinoma from the adenoma. MATERIAL AND METHOD: A retrospective analysis during the period of nine years, four cases of parathyroid carcinoma were diagnosed on the basis of per-operative local invasion and on histological evidence of capsular invasion and vascular invasion. RESULT: We have different observations to make. Diagnosis of parathyroid carcinoma which was done on the basis of intra operative suspicion only. One patient died on 6th post operative day due to development of pancreatitis. At the follow-up of two years there was no local recurrence or distant metastasis. CONCLUSION: It is preferable to have a high index of suspicion for parathyroid carcinoma when these features are present than to miss the opportunity for surgical cure by failing to consider it in the differential diagnosis.

Adenoma↗

[Parathyroid carcinoma].

BACKGROUND: Parathyroid carcinoma is the least frequent malignancy among endocrine tumors. In the most reported series of patients with primary hyperparathyroidism the incidence of carcinoma is less than 1%. Racognition by a surgeon that the parathyroid tumor is malignant, and the performance of an adequate en bloc removal of primary lesion, with histologic diagnosis offer the best treatment of a patient with this unusual malignancy. CASE REPORT: We reported a 30-year-old patient with parathyroid carcinoma, primary hyperparathyroidism, and recurrent nephrocalcinosis. Marked hypercalcemia, low serum phosphorus, and substantial elevation of serum parathyroid hormone indicated a diagnosis of primary hiperparathyroidism. General symptoms were anorexia, muscle weakness, back pain and depression. Ultrasonography done before the surgery revealed a 2 cm upper left parathyroid gland with solid and cystic areas. The neck exploration was done with en block resection of the tumor. A histopathological evaluation confirmed the diagnosis of parathyroid carcinoma. Over more than a three-year-follow-up, the patient had no evidence of the disease recurrence and his serum PTH and calcium levels remained within the normal. CONCLUSION: Parathyroid carcinoma is a rare cause of primary hyperparathyroidism. Preoperative diagnosis remains a challenge. Radical en bloc resection of the tumor is the treatment of choice for this malignancy.

Adult↗

Parathyroid carcinoma.

BACKGROUND: Parathyroid carcinoma is a rare malignancy affecting 0.5-5 per cent of all patients with primary hyperparathyroidism. This article reviews the literature on the pathogenesis, pathology, clinical features, diagnosis and management of parathyroid carcinoma. METHODS: A Medline search was performed and all relevant English language articles published between 1970 and 2005 were retrieved. The search words included 'parathyroid carcinoma', 'pathology', 'genetics', 'management' and 'radiotherapy'. Secondary references were obtained from key articles. RESULTS AND CONCLUSION: The exact aetiology of parathyroid carcinoma remains obscure. Recently, the HRPT2 gene has been implicated in its pathogenesis and may prove to be a genetic target in future. Surgical resection is the accepted 'gold standard'. There is now a growing consensus on the use of adjuvant radiotherapy as it has been shown to provide a survival benefit.

Combined Modality Therapy↗

Positive effect of radiotherapy and surgery on hormonally active pulmonary metastases of primary parathyroid carcinoma.

OBJECTIVE: Parathyroid carcinoma is a rare cause of primary hyperparathyroidism. Surgery is the primary treatment in recurrent or metastatic disease. Radiotherapy has been used as an adjuvant to control subclinical local disease but is otherwise considered ineffective. DESIGN: We report on a patient with parathyroid carcinoma with hypercalcaemia and pulmonary metastases, treated with pamidronate and radiotherapy and later with surgery. METHODS: The treatment was evaluated using serial analysis of serum parathyroid hormone (PTH) and calcium, clinical evaluation and chest radiographs. RESULTS: Intravenous pamidronate alone had limited effect on hypercalcaemia. Following irradiation of the pulmonary lesions (34 Gy in ten fractions), serum levels of calcium and PTH decreased and pamidronate could be discontinued. The patient's general condition improved parallel to a radiological response. At clinical relapse 18 months following radiotherapy, the pulmonary metastases were resected and serum PTH was normalised. CONCLUSIONS: The results indicate that parathyroid carcinoma can be radiosensitive. Thus radiotherapy may be an alternative to palliate symptoms of hypercalcaemia in patients not suited for surgery.

Antineoplastic Agents↗

Parathyroid carcinoma.

BACKGROUND: Parathyroid carcinoma is rare and represents 0.1% to 5% of the cases of hyperparathyroidism. New accounts are important to the understanding of these tumors. Experience in 9 cases is reported. METHODS: From 1970 to 1995, 10 patients with parathyroid carcinoma demonstrated by clinical course or pathologic features of malignancy were treated. The patient's clinical data, laboratory and imaging examinations, surgical findings, pathology, recurrences, and survival were analyzed. RESULTS: One male patient was excluded because of insufficient data. Average age was 51 years, with female:male ratio of 2:1. Average calcium level was 14,3 mg/dL. Palpable mass was found in 55%, bone disease in 89%, and renal disease in 78%. Four patients were reoperations. Five were operated on for hyperparathyroidism (1/tertiary). Capsular invasion was the most incident pathologic feature. Local recurrence occurred in 55%; neck node and bone metastasis in 11%, and lung in 33%. Two patients are alive and 5 died of disease. CONCLUSIONS: Parathyroid carcinoma has clinical and laboratory features that can help diagnosis at the first surgery. It seems to have variable malignancy.

Adenoma↗

A human parathyroid carcinoma that produces parathyroid hormone: long term maintenance in tissue culture.

Parathyroid carcinoma cells from a pulmonary metastasis of a patient with a serum Ca of 17 mg/dl and an immunoreactive parathyroid hormone (PTH) of 6.4 ng eq bovine (b) PTH/ml (normal 40--400 pg/ml) have been maintained in tissue culture for more than 2 1/2 years. The cells secrete PTH into the culture media that 1) during immunoassay dilutes in parallel to human hyperparathyroid serum, 2) has a molecular weight similar to intact highly purified bPTH, and 3) stimulates bone resorption in a manner that is equivalent and additive to synthetic bPTH-(1--34).

Adult↗

Hyperfunctioning intrathyroidal parathyroid carcinoma.

Intrathyroidal parathyroid carcinoma is an exceedingly rare cause of primary hyperparathyroidism with difficulties in the diagnosis and management. We report a case of hypercalcemia from intrathyroidal parathyroid carcinoma in a 63-year-old Saudi female. She was diagnosed 2 years earlier with osteoporosis in a primary care clinic and was on alendronate since then. This year she was noted to have hypercalcemia, but in retrospect she had more than 10 years history of multiple medical problems related to hypercalcemia. Parathyroid 99mTc-SestaMIBI scintigraphy revealed parathyroid adenoma in the left inferior parathyroid gland. She had successful video-assisted parathyroidectomy that relieved most of her symptoms. The extreme rarity of such a case, the interesting clinical presentation and review of the literature are discussed.

Carcinoma↗

[Parathyroid carcinoma].

The parathyroid carcinoma is a rare cause of primary hyperparathyroidism. It deserves special attention in regard to diagnosis and treatment. Within a period of 10 years we observed 117 patients with primary hyperparathyroidism (pHPT) at the University Hospitals of Ulm and Heidelberg. In five patients a parathyroid carcinoma was found, corresponding to an incidence of 4.4%. In four of five patients no clinical signs were evident preoperatively indicating a parathyroid malignancy.

Adult↗

[Hyperfunctional parathyroid carcinoma].

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.

Carcinoma↗

Parathyroid carcinoma: an overview.

Parathyroid carcinoma is a rare tumor that is responsible for <1% of cases of hyperparathyroidism in most parts of the world. An increased incidence of this tumor has been reported in patients with the hyperparathyroidism-jaw tumor (HPT-JT) syndrome, but the etiology of most other cases is unknown. Parathyroid carcinomas tend to occur a decade earlier than adenomas, and the sex ratio approaches unity in contrast to the female preponderance of adenomas. Most patients with carcinomas present with marked hypercalcemia and are more likely to have associated bone and renal disease than those with adenomas. Although fibrosis and mitotic activity are common in carcinomas, these features are not specific for malignancy. The diagnosis of carcinoma should be restricted to those tumors that show invasion of blood vessels, perineural spaces, soft tissues, thyroid gland, or other adjacent structures or to tumors with documented metastases. Mutations of the HRPT2 gene (1q21-q32), which are responsible for the HPT-JT syndrome, have been implicated in the development of a high proportion of parathyroid carcinomas. A subset of patients with mutation-positive carcinomas have germline mutations of the HRPT2 gene. This finding suggests that some patients with apparent sporadic parathyroid carcinomas may have the HPT-JT syndrome or a variant of this syndrome. Because of the high frequency of local recurrence following incomplete excision, an en bloc resection is the preferred surgical approach for treatment of parathyroid carcinomas.

Carcinoma↗

Parathyroid carcinoma: case report.

Parathyroid carcinoma is a rare entity, representing less than 1% to 5% of patients with primary hyperparathyroidism. Parathyroid carcinomas are often symptomatic, have markedly elevated calcium levels, have palpable masses, are equally distributed between the sexes, and present 10 years younger than their benign counterparts. Parathyroid carcinoma is often undiagnosed preoperatively, suspected intraoperatively, and only confirmed postoperatively. When diagnosed, the treatment of choice is an en-bloc resection of the tumor.

Aged↗