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Query: UMLS:C0027627 (
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Parathyroid carcinoma
has always been difficult to diagnose pathologically. In fact, most parathyroid tumors which are classified as carcinoma do not recur after excision, and most parathyroid tumors which actually
metastasize
or recur repeatedly in the neck are not recognized as malignant at first presentation. In 2002, germline HRPT2 (also known as CDC73) mutation was reported as the cause of hyperparathyroidism-jaw tumor (HPT-JT) syndrome, an autosomal dominant hereditary tumor syndrome associated with a lifetime risk of parathyroid carcinoma approaching 15 %. Subsequently, bi-allelic inactivation or mutation of HRPT2 has been reported in the majority of parathyroid carcinomas that actually behave in a malignant manner but very rarely in sporadic benign parathyroid disease. Furthermore, germline testing for HRPT2 mutation in patients presenting with parathyroid carcinoma often identifies occult HPT-JT syndrome even in the absence of a family history or other syndromic manifestations. HRPT2 mutation testing is not readily available, and loss of expression of parafibromin (the protein encoded by HRPT2) as determined by immunohistochemistry has been used as a surrogate marker of HRPT2 mutation. Immunohistochemistry for parafibromin can be technically difficult and has been deployed by different investigators with variable enthusiasm and success. However, proponents have found immunohistochemistry for parafibromin useful to definitively confirm a pathological diagnosis of parathyroid carcinoma, predict a worse outcome in definite parathyroid carcinomas, triage formal genetic testing for HPT-JT syndrome, and predict the outcome of histologically atypical parathyroid adenomas.
...
PMID:Understanding the genetic basis of parathyroid carcinoma. 2440 36
A 45 year-old woman who presented with non-specific neck and shoulder pain, was found to have mild hypercalcaemia, markedly elevated parathyroid hormone levels, and an irregular parathyroid gland on imaging. The patient underwent a parathyroidectomy and the pathology report came back positive for parathyroid carcinoma with muscular invasion.
Parathyroid carcinoma
is an exceptionally rare cause of primary hyperparathyroidism and can have a poor prognosis due to
metastases
and a high propensity to recur after resection. Reports of non-functioning parathyroid carcinomas tend to behave even more aggressively. Repeat imaging on this patient showed residual cancer present, so the patient underwent a second surgery with radical neck dissection and has since been doing very well postoperatively. Diagnosis and treatment is challenging and it is critical to continuously follow-up for recurrent disease.
...
PMID:Parathyroid carcinoma, a rare cause of primary hyperparathyroidism. 2513 18
Parathyroid carcinoma
(
PCA
), accounting for less than one per cent of all endocrine malignancies, is a rare cause of primary hyperparathyroidism. A diagnosis of parathyroid carcinoma may be challenging in the presence of localised disease and involves a histological diagnosis based on capsular, vascular, or perineural invasion or the presence of metastasis. Distant metastasis remains a rare presentation, with the lung being the most common site. Surgery remains the treatment of choice as radiotherapy and chemotherapy have proved to be of limited benefit in
metastatic disease
. This case reports suggests that radiofrequency ablation has the potential to be a novel and effective treatment option in these patients.
...
PMID:Metastatic parathyroid carcinoma treated with radiofrequency ablation: A novel therapeutic modality. 2532 2
Parathyroid carcinoma
is an uncommon malignancy and a rare cause of primary hyperparathyroidism. Although this tumor is capable of metastasis,
metastatic disease
is very uncommon intracranially, with only seven cases reported in the literature. When intracranial
metastases
occur, they typically present months to years following the diagnosis of the primary tumor with hypercalcemia refractory to medical conservative treatment. Aggressive surgical resection of all
metastases
is necessary for control of the disease. We report a case of metastatic parathyroid carcinoma with two intracranial metastatic foci (in the left frontal lobe and left cerebellar hemisphere) identified at the time of the primary tumor diagnosis in a patient who presented with symptomatic hypercalcemia.
...
PMID:Parathyroid carcinoma with intracranial metastasis at diagnosis in a patient with uncontrolled hypercalcemia. 2536 37
Parathyroid carcinoma
is a malignant neoplasm affecting 0.5 to 5.0% of all patients suffering from primary hyperparathyroidism. This cancer continues to cause challenges for diagnosis and treatment because of its rarity, overlapping features with benign parathyroid disease, and lack of distinct characteristics. The third/second generation PTH assay ratio provides valuable information to distinguish between benign parathyroid disease and parathyroid carcinoma. An abnormal ratio (>1) could indicate a high suspicion regarding carcinoma and
metastatic disease
. Early en bloc surgical resection of the primary tumour with clear margins remains the best curative treatment. Although prolonged survival is possible with recurrent or
metastatic disease
, cure is rarely achievable. The efficacy of classical adjuvant therapies, such as radiotherapy and chemotherapy, in management of persistent, recurrent, or
metastatic disease
has been disappointing. In
metastatic disease
the goal of therapeutic support is to control the PTH-driven hypercalcemia that represents the primary cause of mortality. Calcimimetics, which are allosteric modulators of the calcium sensing receptor, have a sustained effect in lowering serum calcium levels. Bone anti-resorptive therapy, like intravenous bisphosphonates (pamidronate and zolendronate), or more recently denosumab (fully human monoclonal antibody with high affinity to bind RANK ligand) might be temporarily useful. In a small number of cases treated with anti-PTH immunotherapy, inducing anti-PTH antibodies, promising results have been seen with clinical improvements and decrease of calcemia. In one case metastasis shrinkage has been observed.
...
PMID:Parathyroid carcinoma: Challenges in diagnosis and treatment. 2591 Sep 97
Parathyroid carcinoma
(PC) is a rare endocrine malignancy and the cause of primary hyperparathyroidism. It is usually associated with a high rate of local and distant recurrence. Laboratory findings and clinical symptoms may be similar to those in parathyroid adenoma. The histological features of PC may also be non-specific and the affected gland is often indistinguishable from a benign lesion. The proper diagnosis is commonly made months to years later when the disease recurs or
metastases
are present. Therefore, parathyroid carcinoma still remains a diagnostic and management challenge for many physicians. However, there are some features that, in combination, may help in diagnosis. Surgery still remains the only curative treatment, even in
metastatic disease
. In advanced, non-operable subjects, managing hypercalcaemia and controlling a tumour are the main goals. Morbidity is caused by hypercalcaemia rather than
metastases
. A multidisciplinary approach with experienced endocrinologists, pathologists, radiologists, nuclear medicine doctors, oncologists, and surgeons is needed to optimize patient outcome.
...
PMID:[Primary hyperparathyroidism due to parathyroid cancer - a diagnostic and management challenge]. 2593 Oct 46
Parathyroid carcinoma
(PC) is a rare endocrine malignancy. The tumor is mostly functioning, causing severe primary hyperparathyroidism, with high serum calcium and parathyroid hormone (PTH) levels. Nonfunctioning PC is extremely rare. We report a 50-year-old male patient who was referred to our Department for a right thyroid nodule, incidentally detected on carotid Doppler ultrasound scan, with a fine-needle aspiration cytology showing a follicular lesion. At the time of our evaluation, neck ultrasound showed a 1.3 cm right hypoechoic thyroid nodule with irregular margins and the absence of enlarged bilateral cervical lymph nodes. Thyroid function tests were normal. Serum calcium was normal and plasma PTH slightly above the upper limit of the normal range. The patients underwent right lobectomy. The intraoperative frozen-section pathological examination raised the suspicion of a PC. Definitive histology showed a markedly irregular infiltrative growth of the tumor with invasion of the thyroid tissue and cervical soft tissues. Immunostaining for thyroglobulin was negative, whereas staining for chromogranin A and PTH showed a strong reactivity. Based on the microscopic findings and the immunohistochemical profile, the tumor was diagnosed as a PC. Postoperative serum calcium and phosphate levels were in the normal range. One month after surgery, serum calcium and PTH were normal. Neck ultrasound and total body computed tomography scan were negative for local and
metastatic disease
. Eight months later, serum calcium was normal and plasma PTH level remained around the upper limit of normal range. Neck ultrasound did not show any pathological lesions. This is the first case of a nonfunctioning sporadic PC misdiagnosed prior of surgery as a follicular thyroid nodule. The parathyroid nature of the neck lesion could not be suspected before surgery. Fine-needle aspiration cytology (FNAC) may fail to distinguish a parathyroid tumor from a benign thyroid nodule because at FNAC, parathyroid and thyroid lesions have some morphological similarities. Histological criteria are not always sufficient for the differential diagnosis, which can definitely be established using immunohistochemistry.
...
PMID:A nonfunctioning parathyroid carcinoma misdiagnosed as a follicular thyroid nodule. 2635 Apr 18
Parathyroid carcinoma
(PC) is an uncommon endocrine malignancy and constitutes a rare cause of hyperparathyroidism. The current study presents the clinical features, laboratory findings, sensitivity of imaging modalities, surgical treatment and the long-term outcome of six patients, who were diagnosed with PC and treated in the Department of Head and Neck Surgery, Zhejiang Province Cancer Hospital (Hangzhou, China) over 13 years (February 1999-January 2012). Pre-operative recognition and intraoperative identification of this rare endocrine malignancy is extremely important, but require a high index of clinical suspicion. The primary treatment is surgical en bloc resection of the tumor and any involved surrounding structures, and it is of great importance, as the prognosis depends on the initial surgery. Radiation therapy and chemotherapy showed no evidence of effectiveness on PC, although certain data show a decreased risk of localized disease recurrence with the addition of radiation therapy. The prognosis of PC is variable and post-operative parathyroid hormone levels that do not decrease often indicate a poor prognosis or presence of other
metastases
.
...
PMID:Parathyroid carcinoma: A report of six cases with a brief review of the literature. 2678 36
Parathyroid carcinoma
only represents <1% of all cases of primary hyperparathyroidism (PHPT). Even rare, chronic PHPT may lead to excessive osteoclast activity, and the increased resorption leads to destruction of cortical bone and formation of fibrous cysts with deposits of hemosiderin-so-called brown tumors. These benign, osteolytic lesions may demonstrate FDG-avidity on (18)F-FDG PET/CT, and as such are misinterpreted as skeletal
metastases
. Regression of the lesions may occur following successful treatment. We present a case demonstrating the diagnostic work-up and follow-up of a patient with PHPT due to parathyroid carcinoma and with presence of brown tumors on (18)F-FDG PET/CT, visualizing the possible role of this imaging modality in the evaluation of treatment response in these patients.
...
PMID:Brown Tumors Due to Primary Hyperparathyroidism in a Patient with Parathyroid Carcinoma Mimicking Skeletal Metastases on (18)F-FDG PET/CT. 2685 55
Parathyroid carcinoma
is a rare cause of primary hyperparathyroidism. Clinical indicators of carcinoma include the severity of hyperparathyroidism and presence of a palpable neck mass. Definitive diagnosis requires surgical resection and specimen histology, or documentation of
metastatic disease
. We present a case of parathyroid carcinoma in a young female patient with severe hyperparathyroidism, who was also found to have subclinical hyperthyroidism in the setting of an intercurrent, solitary, functioning thyroid adenoma. A diagnostic approach to parathyroid disease, combining multimodality imaging and clinicopathologic features, is discussed.
...
PMID:Multimodality imaging in parathyroid carcinoma: A tale of two nodules. 2740 50
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