Current and Future Treatments for Parathyroid Carcinoma

Long, Kristin L.; Sippel, Rebecca S.

doi:10.2217/ije-2017-0011

Cited by 16 publications

(16 citation statements)

References 55 publications

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“…In this system, the extent of local invasion, but not size, is considered. For the nodal stage, only the site: central (pN1a) vs. lateral (pN1b) neck is considered [10,23].…”

Section: Discussionmentioning

confidence: 99%

Parathyroid Carcinoma: Incidence, Survival Analysis, and Management: A Study from the SEER Database and Insights into Future Therapeutic Perspectives

Ullah

Khan

Waheed

et al. 2022

Cancers

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Introduction: Parathyroid carcinoma (PC) is an extremely rare entity, with a frequency of 0.005% of all malignancies. Most data related to this rare disease are limited to case series and a few database studies. We present a large database study that aims to investigate the demographic, clinical, and pathological factors, prognosis, and survival of PC. Methods: Data of parathyroid carcinoma were extracted from the Surveillance, Epidemiology, and End Results (SEER) diagnosed between 1975 and 2016. Results: PC had a slightly higher incidence in men (52.2%, p < 0.005), the majority of cases affected Caucasians (75.4%, p < 0.005), and the mean age at diagnosis was 62 years. Histologically, 99.7% were adenocarcinomas not otherwise specified (p < 0.005), well-differentiated (p < 0.005), and 2–4 cm (p < 0.001) in size among the patients with available data. In cases with staging provided, most PC were organ-confined (36.8%, p < 0.001). Lymph nodes were positive in 25.2% of cases where lymph node status was reported. The main treatment modality was surgery (97.2%), followed by radiation alone (2%), and very few received chemotherapy alone (0.8%), p < 0.005. Five-year follow-up was available for 82.7% of the cases. Those who underwent surgery only or radiation alone had 5-year survivals of 83.8% and 72.2%, respectively (p < 0.037). Multivariable analysis identified tumor size >4 cm, age > 40 years, male sex, Caucasian race, distant spread, and poorly differentiated grade as independent risk factors for mortality (p < 0.001). Conclusion: PC is a very rare tumor mostly affecting Caucasian individuals in the fifth decade. Older age, poor histologic differentiation, and distant metastasis are associated with a worse prognosis. Surgical resection offers the best survival outcome. To better understand the pathogenesis and factors affecting survival, all PC patients should be enrolled in national and international registries.

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“…In this system, the extent of local invasion, but not size, is considered. For the nodal stage, only the site: central (pN1a) vs. lateral (pN1b) neck is considered [10,23].…”

Section: Discussionmentioning

confidence: 99%

Parathyroid Carcinoma: Incidence, Survival Analysis, and Management: A Study from the SEER Database and Insights into Future Therapeutic Perspectives

Ullah

Khan

Waheed

et al. 2022

Cancers

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“…The great majority of PCs are functioning tumors over-secreting parathyroid hormone (PTH), responsible for primary hyperparathyroidism (PHPT) and elevated serum levels of calcium; only 2% of PCs are non-functioning variants characterized by normal serum calcium and PTH values, manifesting through compressive signs and symptoms of local invasion ( 1 ). Contrary to parathyroid hyperplasia and adenomas, PCs are associated with poor prognosis due to more severely elevated PTH, and higher, commonly unmanageable, hypercalcemia, which accounts for death in a majority of cases, with an overall survival rate of 78-85% and 49-70% at 5 and 10 years after diagnosis, respectively ( 2 ). Moreover, 10-30% patients with PC already have metastatic disease at diagnosis, most commonly in the lungs, bone, or liver ( 3 ).…”

Section: Parathyroid Carcinomamentioning

confidence: 99%

Genetics and Epigenetics of Parathyroid Carcinoma

et al. 2022

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Parathyroid carcinoma (PC) is an extremely rare malignancy, accounting less than 1% of all parathyroid neoplasms, and an uncommon cause of primary hyperparathyroidism (PHPT), characterized by an excessive secretion of parathyroid hormone (PTH) and severe hypercalcemia. As opposed to parathyroid hyperplasia and adenomas, PC is associated with a poor prognosis, due to a commonly unmanageable hypercalcemia, which accounts for death in the majority of cases, and an overall survival rate of 78-85% and 49-70% at 5 and 10 years after diagnosis, respectively. No definitively effective therapies for PC are currently available. The mainly employed treatment for PC is the surgical removal of tumoral gland(s). Post-surgical persistent or recurrent disease manifest in about 50% of patients. The comprehension of genetic and epigenetic bases and molecular pathways that characterize parathyroid carcinogenesis is important to distinguish malignant PCs from benign adenomas, and to identify specific targets for novel therapies. Germline heterozygote inactivating mutations of the CDC73 tumor suppressor gene, with somatic loss of heterozygosity at 1q31.2 locus, account for about 50-75% of familial cases; over 75% of sporadic PCs harbor biallelic somatic inactivation/loss of CDC73. Recurrent mutations of the PRUNE2 gene, a recurrent mutation in the ADCK1 gene, genetic amplification of the CCND1 gene, alterations of the PI3K/AKT/mTOR signaling pathway, and modifications of microRNA expression profile and gene promoter methylation pattern have all been detected in PC. Here, we review the current knowledge on gene mutations and epigenetic changes that have been associated with the development of PC, in both familial and sporadic forms of this malignancy.

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“…Owing to the limited available data on tumor characteristics and prognosis, a formal staging system does not exist at this time. However, the newest release of the Tumor, Node and Metastases (TNM) cancer manual from the combined American Joint Committee on Cancer (AJCC) as well as the Union for International Cancer Control (UICC) have proposed and defined specific variables to be recorded prospectively in order to develop a formal staging system in the future [42].…”

Section: Stagingmentioning

confidence: 99%

“…The mechanism of action of cinacalcet is the decrease in PTH production, which makes it an apt choice for parathyroid cancer related hypercalcemia [77]. The initial dose is 30 mg twice daily, which can be increased after 2–4 week intervals, based on how well the patient tolerates it [42]. Gallium nitrate has been described in the literature as an option for treatment of hypercalcemia due to similar osteoclastic inhibition.…”

Section: Adjuvant Therapy For Parathyroid Cancermentioning

confidence: 99%

Parathyroid Cancer: A Review

Machado¹,

Wilhelm

2019

Cancers

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Parathyroid cancer is one of the rarest causes of primary hyperparathyroidism and tends to present with more severe symptoms than its more benign counterparts. This article details various aspects of the disease process, including epidemiology, clinical presentation, and a step-wise diagnostic process for parathyroid cancer. This includes laboratory assessments as well as a proposed staging system. The en bloc principle of surgical intervention is detailed, as well as the current role of adjuvant treatments. A general guide to surveillance and the natural history of the disease is also outlined.

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Current and Future Treatments for Parathyroid Carcinoma

Cited by 16 publications

References 55 publications

Parathyroid Carcinoma: Incidence, Survival Analysis, and Management: A Study from the SEER Database and Insights into Future Therapeutic Perspectives

Parathyroid Carcinoma: Incidence, Survival Analysis, and Management: A Study from the SEER Database and Insights into Future Therapeutic Perspectives

Genetics and Epigenetics of Parathyroid Carcinoma

Parathyroid Cancer: A Review

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