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Endocrine Abstracts (2018) 56 P851 | DOI: 10.1530/endoabs.56.P851

ECE2018 Poster Presentations: Pituitary and Neuroendocrinology Pituitary - Clinical (101 abstracts)

Pituitary adenomas in childhood and the transition period - clinical and genetic characterization of 49 patients at one tertiary care endocrine institution in Romania

Andreea Vladan 1 , Serban Radian 1, , Ionela Baciu 1, , Iuliana Gherlan 1, , Antonia Lefter 1 , Simona Galoiu 1, , Cristina Dumitrescu 1 , Camelia Procopiuc 1 , Corin Badiu 1, & Catalina Poiana 1,

1C.I. Parhon National Institute of Endocrinology, Bucharest, Romania; 2Department of Endocrinology, C. Davila University of Medicine and Pharmacy, Bucharest, Romania.

Introduction: Pituitary adenomas (PAs) are rare in childhood and the transition period, can result from AIP/MEN1 mutations, are difficult to manage and severely impair quality-of-life.

Aim: To describe the clinical and genetic characteristics of patients with PA onset before 21 years old.

Patients and methods: Retrospective study (1980–2015). Clinical, imaging and hormonal data, AIP/MEN1 sequencing.

Results: We identified 49 patients (33 F/16 M), with onset age 18 (15–19) years, median (25th–75th percentile): 27 prolactinomas (5 micro, 20 macro, 2 giant adenomasn [>4 cm]; 3 clinical MEN1), 11 somatotropinomas (9 macro, 2 giant adenomas; 2 somatolactotropinomas; 2 patients with gigantism), 8 corticotropinomas (7 microadenomas), 2 nonfunctional PAs (NFPA, 1 mesoadenoma, 1 macroadenoma), 1 giant GH co-secreting, thyrotropinoma. From 34 patients tested, two (both with gigantism) had AIP mutations: c.940C>T (M-18yrs.), c.895dup (F-13yrs.). One patient with prolactinoma and primary hyperparathyroidism had a MEN1 mutation: c.1446delC. Therapy included Dopamine agonists (DA): 27 prolactinomas, 8 somatotropinomas, 2 corticotropinomas, 1 NFPA, 1 thyrotropinoma; Somatostatin analogues: 11 somatotropinomas and 1 thyrotropinoma; Pegvisomant: 5 somatotropinomas; pituitary surgery: 10 somatotropinomas, 6 corticotropinomas, 1 NFPA, 1 thyrotropinoma; radiotherapy: 8 somatotropinomas, 3 corticotropinomas, 8 prolactinomas, 1 NFPA, 1 thyrotropinoma; Temozolomide: 2 giant PAs, multiply operated and irradiated (1 thyrotropinoma+1 DA-resistant prolactinoma). AIP mutations were associated with gigantism, giant adenomas (rsquare=0.59, P<0.01) and the absence of tumour growth control (rsquare=−0.39, P<0.05). The number of treatment agents per patient was 1 (1–2), for prolactinomas and 5 (3–6) for somatotropinomas. At the last follow-up visit, 7 (2–10.5) years after diagnosis, 33/47 (70.21%) functional PAs were controlled biochemically.

Conclusions: Results of therapy in patients with PA onset before 21 years are suboptimal, despite aggressive therapy. Somatotropinomas are particularly resistant, partly due to AIP mutations.

Volume 56

20th European Congress of Endocrinology

Barcelona, Spain
19 May 2018 - 22 May 2018

European Society of Endocrinology 

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