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Aortopulmonary window parathyroid gland causing primary hyperparathyroidism in men type 1 syndrome

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Abstract

Primary hyperparathyroidism (HPT) is the most common endocrinopathy in Multiple Endocrine Neoplasia type 1 (MEN1) syndrome. Supernumerary and/or ectopic parathyroid glands, potentially causes of persistent or recurrent HPT after surgery, have been previously described. However, this is the first ever described case of ectopic parathyroid gland localized in the aortopulmunary window causing HPT in MEN1. After a consistent concordant pre-operative imaging assessment the patient, a 16 years old male affected by a severe hypercalcemia, underwent surgery. The parathyroid was found very deeply near the tracheal bifurcation, hidden by the aortic arch itself and for this reason not visible at the beginning of the dissection but only after being identified by palpation for its typical consistence. The intraoperative PTH decreased at normal level 10 min after removal of the ectopic gland. The patient remained with normal value of calcemia and PTH during the 10 months of follow-up.

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Authors’ contribution

All authors contributed equally to this work; F.T., F.G., C.B. and F.C. collected the data, F.T., F.G. and C.B. analyzed data, F.T., F.G. wrote the manuscript; M.L.B supervised the manuscript.

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Correspondence to Francesco Tonelli.

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Authors certify that there is no actual or potential conflict of interest in relation to this article and they state that there are no financial interests or connections, direct or indirect, or other situations that might raise the question of bias in the work reported or the conclusions, implications, or opinions stated—including pertinent commercial or other sources of funding for the individual author(s) or for the associated department(s) or organization(s), personal relationships, or direct academic competition. The Authors certify also that the manuscript, including related data and figures, has not been previously published and that it is not under consideration elsewhere.

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Tonelli, F., Biagini, C., Giudici, F. et al. Aortopulmonary window parathyroid gland causing primary hyperparathyroidism in men type 1 syndrome. Familial Cancer 15, 133–138 (2016). https://doi.org/10.1007/s10689-015-9840-x

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