title: Cystic parathyroid glands in MEN1: A rare entity?
abstract: Approximately 300 cases of sporadic parathyroid cyst (PCs) have been reported to date. Only two cases have been described in MEN1 so far. Detection by imaging could be challenging, especially in multiglandular primary hyperparathyroidism (HPT) and clinical outcome could be different. During the period 1990-2014, 71 MEN1 patients were operated for primary hyperparathyroidism in our centre. We report three cases of PCs in MEN1 patients affected by HPT, who underwent a total or subtotal parathyroidectomy with transcervical thymectomy. In our series, all three patients had an unsatisfactory postoperative course, at variance with the high percentage (over 90 %) of long-term success in MEN1 patients operated at our centre. One patient affected by cystic degeneration of all the four parathyroid glands reported persistent hypoparathyroidism, despite autografts of parathyroid tissue. For the other two cases, surgery failed to cure hyperparathyroidism, perhaps because of the presence of undetected ectopic parathyroid tissue. In the context of a multiglandular disease such as MEN1 syndrome, PCs seem rare but our experience shows about a 4 % incidence. Furthermore their presence, even in expert hands, could affect the preoperative identification of the parathyroid glands due to the difficult differential diagnosis between PC and other cystic lesions of the neck, and intraoperative detection of the glands as well as the postoperative outcome.
authors.affiliation: Department of Surgery and Translational Medicine, University of Florence, Largo Brambilla 3, 50134, Florence, Italy. tiziana.cavalli@gmail.com.
authors.forename: Tiziana
authors.initials: T
authors.lastname: Cavalli
authors.affiliation: Department of Surgery and Translational Medicine, University of Florence, Largo Brambilla 3, 50134, Florence, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Giudici
authors.affiliation: Department of Surgery and Translational Medicine, University of Florence, Largo Brambilla 3, 50134, Florence, Italy.
authors.forename: Gabriella
authors.initials: G
authors.lastname: Nesi
authors.affiliation: Institute of Pathology, Department of Health Sciences, University "Magna Graecia" of Catanzaro, Catanzaro, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Amorosi
authors.affiliation: Department of Surgery and Translational Medicine, University of Florence, Largo Brambilla 3, 50134, Florence, Italy.
authors.forename: Raffaella
authors.initials: R
authors.lastname: Santi
authors.affiliation: Department of Surgery and Translational Medicine, University of Florence, Largo Brambilla 3, 50134, Florence, Italy.
authors.forename: Maria Luisa
authors.initials: ML
authors.lastname: Brandi
authors.affiliation: Department of Surgery and Translational Medicine, University of Florence, Largo Brambilla 3, 50134, Florence, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Tonelli
mesh_terms: D000328:Adult; D003560:Cysts / Q000175:diagnosis* / Q000453:epidemiology / Q000473:pathology / Q000601:surgery; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D049950:Hyperparathyroidism, Primary / Q000150:complications / Q000601:surgery*; D007011:Hypoparathyroidism / Q000209:etiology / Q000601:surgery; D015994:Incidence; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000150:complications / Q000235:genetics / Q000601:surgery*; D010280:Parathyroid Glands / Q000473:pathology* / Q000601:surgery; D010282:Parathyroid Neoplasms / Q000175:diagnosis* / Q000453:epidemiology / Q000473:pathology / Q000601:surgery; D016105:Parathyroidectomy; D013934:Thymectomy
keywords: HPT; MEN1; PTH; Parathyroid cysts; Surgery