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01-01-2016 | Short Communication

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

Authors: Francesco Tonelli, Carlo Biagini, Francesco Giudici, Federica Cioppi, Maria Luisa Brandi

Published in: Familial Cancer | Issue 1/2016

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.

Brandi ML, Gagel RF, Angeli A, Bilezikian JP, Beck-Peccoz P, Bordi C et al (2001) Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab 86:5658–5671PubMedCrossRef

Burgess JR, David R, Greenaway TM, Parameswaran V, Shepherd JJ (1999) Osteoporosis in multiple endocrine neoplasia type 1. Arch Surg 134:119–123CrossRef

Marks SJ, Menczel J, Campbell G, Aurbach GD, Spiegel AM, Norton JA (1991) Heterogenous size of the parathyroid glands in familial multiple endocrine neoplasia type 1. Clin Endocrinol 35:521–526CrossRef

Tonelli F, Marcucci T, Fratini G, Tommasi MS, Falchetti A, Brandi ML (2007) Is total parathyroidectomy the treatment of choice for hyperparathyroidism in multiple endocrine neoplasia type 1? Ann Surg 246:1057–1082CrossRef

Arnalsteen LC, Alesina PF, Quiereux JL, Farrel SG, Patton FN, Carnaille BM et al (2002) Long-term results of less than total parathyroidectomy for hyperparathyroidism in multiple endocrine neoplasia type 1. Surgery 132:1119–1125PubMedCrossRef

Kraimps JL, Duh QY, Demeure M, Clark OH (1992) Hyperparathyroidism in multiple endocrine neoplasia syndrome. Surgery 112:1080–1088PubMed

Kivlen MH, Bartlett DL, Libutti SK, Skarulis MC, Marx SJ, Simonds WF et al (2001) Reoperation for hyperparathyroidism in multiple endocrine neoplasia type 1. Surgery 130:991–998PubMedCrossRef

Nilubol N, Weinstein L, Simonds WF, Jensen RT, Phan GQ, Hughes MS et al (2012) Preoperative localizing studies for initial parathyroidectomy in MEN1 syndrome: is there any benefit? World J Surg 36:1368–1374PubMedCrossRef

Montenegro FL, Lourenço DM Jr, Tavares MR, Arap SS, Nascimento CP Jr, Massoni Neto LM et al (2012) Total parathyroidectomy in a large cohort of cases with hyperparathyroidism associated with multiple endocrine neoplasia type 1: experience from a single academic center. Clinics (Sao Paulo) 67:131–139CrossRef

10.

Arnault V, Beaulieu A, Lifante JC, Sitges Serra A, Sebag F, Mathonnet M et al (2010) Multicenter study of 19 aortopulmonary window parathyroid tumors: the challenge of embryologic origin. World J Surg 34:2211–2216PubMedCrossRef

11.

Doppman JL, Skarulis MC, Chen CC, Chang R, Pass HI, Fraker DL et al (1996) Parathyroid adenomas in the aortopulmonary window. Radiology 201:456–462PubMedCrossRef

12.

Akerström G, Malmaeus J, Bergström R (1984) Surgical anatomy of human parathyroid glands. Surgery 95:14–21PubMed

13.

Henry JF, Defechereux T, Raffaelli M, Lubrano D, Iacobone M (2000) Supernumerary ectopic hyperfunctioning parathyroid gland: a potential pitfall in surgery for sporadic primary hyperthyroidism. Ann Chir 125:247–252PubMedCrossRef

14.

Mariette C, Pellissier L, Combemale F, Quievreux JL, Carnaille B, Proye C (1998) Reoperation for persistent or recurrent primary hyperparathyroidism. Langenbecks Arch Surg 383:174–179PubMedCrossRef

15.

Schwarzlmüller T, Brauckhoff K, Løvås K, Biermann M, Brauckhoff M (2014) High cardiac background activity limits 99mTc-MIBI radioguided surgery in aortopulmonary window parathyroid adenomas. BMC Surg 23:14–22

16.

Iihara M, Suzuki R, Kawamata A, Horiuchi K, Okamoto T (2012) Thoracoscopic removal of mediastinal parathyroid lesions: selection of surgical approach and pitfalls of preoperative and intraoperative localization. World J Surg 36:1327–1334PubMedCrossRef

17.

Hunter GJ, Schellingerhout D, Vu TH, Perrier ND, Hamberg LM (2012) Accuracy of four-dimensional CT for the localization of abnormal parathyroid glands in patients with primary hyperparathyroidism. Radiology 264:789–795PubMedCrossRef

18.

Hunter GJ, Ginat DT, Kelly HR, Halpern EF, Hamberg LM (2014) Discriminating parathyroid adenoma from local mimics by using inherent tissue attenuation and vascular information obtained with four-dimensional CT: formulation of a multinomial logistic regression model. Radiology 270:168–175PubMedCrossRef

19.

Hoang JK, Sung WK, Bahl M, Phillips CD (2014) How to perform parathyroid 4D CT: tips and traps for technique and interpretation. Radiology 270:15–24PubMedCrossRef

20.

Profanter C, Schmid T, Prommegger R, Bale R, Sauper T, Bodner J (2004) Robot-assisted mediastinal parathyroidectomy. Surg Endosc 18:868–870PubMedCrossRef

21.

Ismail M, Maza S, Swierzy M, Tsilimparis N, Rogalla P, Sandrock D et al (2010) Resection of ectopic mediastinal parathyroid glands with the da Vinci robotic system. Br J Surg 97:337–343PubMedCrossRef

22.

Lairmore TC, Govednik CM, Quinn CE, Sigmond BR, Lee CY, Jupiter DC (2014) A randomized, prospective trial of operative treatments for hyperparathyroidism in patients with multiple endocrine neoplasia type 1. Surgery 156:1326–1334PubMedCrossRef

23.

Van Heerden JA, Kent RB 3rd, Sizemore GW, Grant CS, ReMine WH (1983) Primary hyperparathyroidism in patients with multiple endocrine neoplasia syndromes. Surgical experience. Arch Surg 118:533–536PubMedCrossRef

24.

O’Riordain DS, O’Brien T, Grant CS, Weaver A, Gharib H, van Heerden JA (1993) Surgical management of primary hyperparathyroidism in multiple endocrine neoplasia types 1 and 2. Surgery 114:1031–1039PubMed

25.

Goudet P, Dalac A, Le Bras M, Cardot-Bauters C, Niccoli P, Lévy-Bohbot N et al (2015) MEN1 disease occurring before 21 years old: a 160-patient cohort study from the Groupe d’étude des Tumeurs Endocrines. J Clin Endocrinol Metab 100:1568–1577PubMedCrossRef

26.

Locchi F, Cavalli T, Giudici F, Brandi ML, Tonelli F (2014) Intraoperative PTH monitoring: a new approach based on the identification of the “true” time origin of the decay curve. Endocr J 61:239–247PubMedCrossRef

27.

Tonelli F, Spini S, Tommasi M, Gabbrielli G, Amorosi A, Brocchi A, Brandi ML (2000) Intraoperative parathormone measurement in patients with multiple endocrine neoplasia type I syndrome and hyperparathyroidism. World J Surg 24:556–562PubMedCrossRef

28.

Udelsman R, Donovan P, Shaw C (2014) Cure predictability during parathyroidectomy. World J Surg 38:525–533PubMedCrossRef

29.

Yavuz S, Simonds WF, Weinstein LS, Collins MT, Kebebew E, Nilubol N et al (2012) Sleeping parathyroid tumor: rapid hyperfunction after removal of the dominant tumor. J Clin Endocrinol Metab 97:1834–1841PubMedPubMedCentralCrossRef

Title: Aortopulmonary window parathyroid gland causing primary hyperparathyroidism in men type 1 syndrome
Authors: Francesco Tonelli
Carlo Biagini
Francesco Giudici
Federica Cioppi
Maria Luisa Brandi
Publication date: 01-01-2016
Publisher: Springer Netherlands
Published in: Familial Cancer / Issue 1/2016
Print ISSN: 1389-9600
Electronic ISSN: 1573-7292
DOI: https://doi.org/10.1007/s10689-015-9840-x

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