ESPE2024 Poster Category 1 Pituitary, Neuroendocrinology and Puberty 4 (9 abstracts)
Somatotropinomas in Pediatric Endocrinology Practice: Single Center Experience
Gözde Gürpınar 1 , Ayşe Uzuner 2 , Seda Duman Öztürk 3 , Selen Hürmüzlü Közler 1 , Esra Koçyiğit 1 , Gizem Böke Koçer 1 , Sibel Tuğçe Aygün 1 , Hakan Gürkan 4 , Durdugül Emecen Ayyıldız 5 , Jeremy Huw Jones 6 , Savaş Ceylan 2 & Filiz Mine Çizmecioğlu Jones 1
1Division of Pediatric Endocrinology, Department of Pediatrics, Kocaeli University School of Medicine, Kocaeli, Turkey. 2Department of Neurosurgery, Pituitary Research Center, Kocaeli University School of Medicine, Kocaeli, Turkey. 3Department of Pathology, Kocaeli University School of Medicine, Kocaeli, Turkey. 4Department of Medical Genetics, Faculty of Medicine, Trakya University, Edirne, Turkey. 5Department of Pediatric Genetics, Dr. Behçet Uz Children's Education and Research Hospital, İzmir, Turkey. 6Department of Academic Writing, Kocaeli University, Kocaeli, Turkey
Introduction: Pituitary gigantism patients are characterized by male predominance and large tumors that are difficult to control. In most cases, history, physical examination, and adjunctive biochemical, imaging, and/or molecular genetic testing will ultimately reveal the diagnosis.
Methods: In this single-center study, demographic, clinical, endocrinological data of patients <19 years old who were followed up with the diagnosis of somatotropinoma between 2014-2024 were retrospectively reviewed.
Results: Seventeen patients with a median age of 15.3(11.5–18.6) years at the time of diagnosis were included, of whom nine were female (53%). All patients had macroadenomas with a median tumor diameter of 23(10–45)mm. The median height SDS of all patients was 3.32(-0.02-5.79). All patients were diagnosed with acromegaly/gigantism. At the time of diagnosis symptoms included headache n = 8(39%), visual impairment n = 4(24%), delayed puberty n = 7(41%). The median preoperative serum IGF-1 level was 1002 (572–1094) ng/mL, and the growth hormone(GH) level was 13.55 ng/mL (5-107). Four patients underwent a glucose tolerance test, but no suppression of GH concentrations was observed (mi n = 5.22 ng/ml). Hormonal evaluation revealed hypogonadotropic hypogonadismin seven (41%), central hypothyroidism in three (18%), and central adrenal insufficiency in four (24%). Sixteen of 17(94%) underwent surgery. The patient who did not accept surgery had concurrent hyperprolactinemia and was diagnosed with McCune Albright Syndrome, received medical treatments. His analysis of the GNAS gene revealed a pathogenic variant. Two other patients molecular genetic analysis revealed negative for AIP variant in one, whereas the results for the GNAS and MEN1 genes are pending. In the case of the adult with a family history of tall individuals, a fresh pituitary tissue sample was investigated for GNAS variants but none was found. Peripheral blood samples were taken for AIP, MEN1 testing, but the results are pending. Histopathology reported a median Ki-67 index of 2%(1-7). Twelve (70.6%) were positive for GH, two(11.8%) were positive for PIT1, CAM5.2, and GH. The other two patients findings were consistent with somatomammotroph cells (positive for GH, PRL).
Conclusion: Pediatric somatotropinoma is uncommon, no treatment guidelines are avaiable. With experienced pituitary surgeons, transsphenoidal surgery results in a remission rate of 70–85% for microadenomas and 40-50% for macroadenomas. Medical treatment and radiotherapy are recommended for those who have persistent disease after surgery and those with a residual mass. In our series, the gender ratio was almost same, all had macroadenomas, and all those who underwent surgery achieved remission without requiring medical treatment.
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