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Parathyroid carcinoma in multiple endocrine neoplasm type 1 syndrome: case report and systematic literature review

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Abstract

The aim of this report was to illustrate a case of parathyroid carcinoma (PC) in a patient with multiple endocrine neoplasia type 1 (MEN1) along with a comprehensive literature review. A 61-year-old man presented with 9-cm PC causing primary hyperparathyroidism (PHPT). His pre-operative corrected calcium and intact PTH serum levels were 2.92 mmol/L and 391.7 pg/mL, respectively. The neoplastic gland was removed in bloc with thyroid and central compartment lymph nodes. A literature review was run by searching PubMed MEDLINE from 1977 to 2018 for studies of all types, in the English language only, using the terms “Parathyroid, carcinoma, Multiple endocrine neoplasia, type 1, (MEN1).” Pathology confirmed PC. Post-operative calcium and PTH levels were normal. A diagnosis of MEN1 was established post-operatively. Seventeen cases of PC in patients with MEN1 have been reported in the literature. 59% of patients were men, and median age at diagnosis was 50 years, with median serum PTH of 379 pg/mL and median serum calcium level of 3.2 mmol/L. The occurrence of PC in the context of MEN1 is extremely rare. Diagnosis and treatment may represent a challenge, so opportune identification or suspicion of malignancy and adoption of correct surgical approach may offer affected patients the best outcome.

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Abbreviations

PC:

Parathyroid cancer

PHPT:

Primary hyperparathyroidism

NET:

Neuroendocrine tumours

MEN1:

Multiple endocrine neoplasia type 1

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Funding

This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

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Authors and Affiliations

  1. Department of Biomedical Sciences and Human Oncology – Unit of Endocrine, Digestive and Emergency Surgery, University “A. Moro” of Bari. Polyclinic of Bari, Piazza Giulio Cesare, 1, 70124, Bari, Italy

    Giovanna Di Meo, Lucia Ilaria Sgaramella, Valentina Ferraro, Angela Gurrado & Mario Testini

  2. Department of Emergency and Organ Transplant, University “A. Moro” of Bari. Polyclinic of Bari, Piazza Giulio Cesare, 1, 70124, Bari, Italy

    Francesco Paolo Prete

Authors
  1. Giovanna Di Meo
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  2. Lucia Ilaria Sgaramella
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  3. Valentina Ferraro
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  4. Francesco Paolo Prete
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  5. Angela Gurrado
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  6. Mario Testini
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Contributions

GDM contributed to conception and design of the study, case and literature review, draft writing, manuscript review, and intellectual input. LIS and VF contributed to case and literature review, draft writing, and manuscript review. FPP and AG contributed to draft writing, manuscript review, and intellectual input. MT helped in design and coordination of the study, draft writing, manuscript review, and intellectual input. All the authors read and approved the final manuscript.

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Correspondence to Mario Testini.

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The authors declare that they have no conflicts of interest.

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Di Meo, G., Sgaramella, L.I., Ferraro, V. et al. Parathyroid carcinoma in multiple endocrine neoplasm type 1 syndrome: case report and systematic literature review. Clin Exp Med 18, 585–593 (2018). https://doi.org/10.1007/s10238-018-0512-7

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  • DOI: https://doi.org/10.1007/s10238-018-0512-7

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