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Elham Barazeghi, Anthony J Gill, Stan Sidhu, Olov Norlén, Roberto Dina, F Fausto Palazzo, Per Hellman, Peter Stålberg, and Gunnar Westin

Introduction Parathyroid carcinoma (PC) is a slow-growing and rare parathyroid disease associated with highly elevated parathyroid hormone levels and hypercalcemia. PC occurs in less than 1 to <5% of primary hyperparathyroidism (pHPT) cases

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Xiang Zhang, Ya Hu, Mengyi Wang, Ronghua Zhang, PeiPei Wang, Ming Cui, Zhe Su, Xiang Gao, Quan Liao, and Yupei Zhao

Introduction Primary hyperparathyroidism (pHPT) is a common endocrine disorder that is typically caused by benign or malignant neoplasia. Parathyroid adenoma (PAd) is the leading cause of pHPT, while parathyroid carcinoma (PCa) is a rare

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Nancy D Perrier, Andrew Arnold, Jessica Costa-Guda, Naifa L Busaidy, Ha Nguyen, Hubert H Chuang, and Maria Luisa Brandi

Endocrine Neoplasia (MEN2019), whose main focus was on malignancy in hereditary endocrine tumor syndromes. Parathyroid carcinoma (PC), first described by Sainton & Millot (1933) , is a rare malignant neoplasm involving the parathyroid gland and one of the

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F Cetani, E Pardi, C Banti, P Collecchi, P Viacava, S Borsari, G Fanelli, A G Naccarato, F Saponaro, P Berti, P Miccoli, A Pinchera, and C Marcocci

Introduction Parathyroid carcinoma is a rare endocrine malignancy and accounts for <1% of cases of sporadic primary hyperparathyroidism (PHPT) ( Marcocci et al . 2008 ). Oncogenes and tumor suppressor genes have been linked to parathyroid

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F Lumachi, M Ermani, F Marino, A Poletti, SMM Basso, M Iacobone, and G Favia

with primary hyperparathyroidism, abnormal parathyroid glands have been characterized as being hyperplastic, adenomatous or malignant, but parathyroid carcinoma (PC) is an uncommon finding, accounting for only 1–2% of cases ( Arnaud 1994 , Shane

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S Corbetta, V Vaira, V Guarnieri, A Scillitani, C Eller-Vainicher, S Ferrero, L Vicentini, I Chiodini, M Bisceglia, P Beck-Peccoz, S Bosari, and A Spada

Introduction Parathyroid carcinoma (PaC) is a rare cause of primary hyperparathyroidism ( Marx 2000 ). It is associated with a poor prognosis, and no curative therapies are available. PaCs occur either sporadically or in family members

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Vincenzo Condello, Filomena Cetani, Maria Denaro, Liborio Torregrossa, Elena Pardi, Paolo Piaggi, Simona Borsari, Anello Marcello Poma, Lucia Anna Muscarella, Paolo Graziano, Maria Grazia Chiofalo, Andrea Repaci, Giovanni Tallini, Francesco Boi, Gabriele Materazzi, Fulvio Basolo, and Claudio Marcocci

Parathyroid carcinoma (PC) is one of the rarest and aggressive malignancies of the endocrine system. In some instances the histological diagnosis remains uncertain unless there is evidence of gross local invasion or secondary spread. The identification of molecular markers could improve the diagnostic accuracy of these lesions.

The expression of 740 genes involved in the tumor progression processes was assessed in 8 parathyroid adenomas (PAs), 17 non-metastatic and 10 metastatic PCs using NanoString technology. Clustering analysis and Ingenuity Pathway Analysis (IPA) were interrogated to compare the gene expression profiles among the three analyzed groups and to evaluate the potential role of differentially expressed genes, respectively. The 103 differentially expressed genes between metastatic PCs and PAs are able to discriminate perfectly the two groups from a molecular point of view. The molecular signatures identified in non-metastatic PCs vs. PAs and in metastatic PCs vs. non-metastatic PCs comparisons, although with some exceptions, seem to be histotype-specific IPA reveals that hepatic fibrosis/hepatic stellate cell activation and GP6 signaling pathway are involved in malignant behavior of parathyroid tumors, whereas the activation of the HOTAIR regulatory pathway are involved in the metastatization process.

Our investigation identified differentially expressed genes in non-metastatic PCs mainly encoding ECM proteins and in metastatic PCs driving endothelial-to-mesenchymal transition or encoding mediators of angiogenesis. The identified genes might be promising molecular markers potentially useful in the clinical practice for the early diagnosis and prognosis of PC.

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Filomena Cetani, Claudio Marcocci, Liborio Torregrossa, and Elena Pardi

. 2017 ). Most patients with HPT-JT are at increasing risk of parathyroid carcinoma (up to 37%) and develop single or multiple cystic parathyroid adenomas and occasionally atypical adenomas ( Marx & Goltzman 2019 ). Because of the underlying genetic basis

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Kelly Brewer, Jessica Costa-Guda, and Andrew Arnold

. Table 2 Selected genetic lesions of established or potential pathogenic importance in sporadic parathyroid carcinoma. Gene Established or hypothesized role Primary mechanism Role in tumorigenesis CDC73 (parafibromin

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Anna Angelousi, Georgios K Dimitriadis, Georgios Zografos, Svenja Nölting, Gregory Kaltsas, and Ashley Grossman

). Approximately, 0.2% of pituitary tumours can develop cranial and extra-cranial metastases ( Kaltsas et al. 2005 a , Heaney et al. 2011 ), whereas the incidence of parathyroid carcinomas is estimated at less than 1% in patients with primary