Clinical and Experimental Medicine

, Volume 18, Issue 4, pp 585–593 | Cite as

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

  • Giovanna Di Meo
  • Lucia Ilaria Sgaramella
  • Valentina Ferraro
  • Francesco Paolo Prete
  • Angela Gurrado
  • Mario TestiniEmail author
Review Article


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.


Parathyroid carcinoma Multiple endocrine neoplasia type 1 MEN1 Primary hyperparathyroidism Cancer Surgery 



Parathyroid cancer


Primary hyperparathyroidism


Neuroendocrine tumours


Multiple endocrine neoplasia type 1


Authors’ 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.


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

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflicts of interest.


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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Biomedical Sciences and Human Oncology – Unit of Endocrine, Digestive and Emergency SurgeryUniversity “A. Moro” of Bari. Polyclinic of BariBariItaly
  2. 2.Department of Emergency and Organ TransplantUniversity “A. Moro” of Bari. Polyclinic of BariBariItaly

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