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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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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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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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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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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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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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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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Jessica Svedlund, Elham Barazeghi, Peter Stålberg, Per Hellman, Göran Åkerström, Peyman Björklund, and Gunnar Westin

, benign adenoma in ∼85% of cases and parathyroid hyperplasia or multiple adenomas in ∼15%, and very rarely by parathyroid carcinoma (<1%). In renal failure, secondary hyperparathyroidism (sHPT) occurs with multiple tumor development as a result of calcium

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Yulong Li, Jianhua Zhang, Poorni R Adikaram, James Welch, Bin Guan, Lee S Weinstein, Haobin Chen, and William F Simonds

identified and are presumed to cause partial or complete inactivation of this gene ( Newey et al. 2010 ). It is estimated that at least two thirds of seemingly sporadic parathyroid carcinomas harbor somatic or germline mutations in the CDC73 gene

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

and carcinoma, in contrast, are rarer being the latter the rarest parathyroid tumor (<1%) ( Cetani et al. 2016 , Saponaro et al. 2018 ). PHPT occurs as sporadic (90%) or as familial (10%) disease. Among the latter, multiple endocrine neoplasia