ECE2020 Audio ePoster Presentations Pituitary and Neuroendocrinology (217 abstracts)
Multi-hormonal secretion in a young male with a large cystic pancreatic neuroendocrine neoplasm
Georgios Boutzios 1 , Eleni Koukoulioti 1 , Nikolaos Garmpis 2 , Ilias Giovannopoulos 1 , Konstantinos Bourgalis-Michalopoulos 1 & Dimitrios Mantas 2
1National and Kapodistrian University of Athens, Pathophysiology, Athens, Greece; 2National and Kapodistrian University of Athens, Second Department of Propedeutic Surgery, Athens, Greece
Introduction: The molecular pathogenesis of pancreatic neuroendocrineneoplasms (PNEN) is increasingly yet incompletely understood. PNEN typically occur sporadically or may occur within the context of hereditary tumor predisposition syndromes. PNEN can also be associated with endocrinopathy of hormonal hypersecretion. We present a young male with a multi-hormonal neuroendocrine neoplasm of pancreas.
Case presentation: A 24-year-old male, non-smoker, with a history of epileptic seizures, hypophyseal Rathke cyst and tonsillectomy presented with hypoglycemias. Family history was unremarkable. Prolonged fasting test was indicative for insulinoma. Abdomen CT was performed and showed a 4 cm cystic lesion of the pancreatic tail. EUS with FNAB was performed and the cytological result was compatible with PNEN. The patient underwent distal pancreatectomy. Histology revealed a well differentiated PNEN, Grade 2 (Ki67 6%), CgA (+), Syn (+), SSTR2a (+), insulin (+), Glucagon (–) with insulin production. Moreover, another small lesion of well differentiated PNEN (1.3 mm) Grade 1 (Ki67 1%), CgA (+), Syn (+), SSTR2a (+), Glucagon (+), insulin (–) with glucagon production was histologically found. Due to the young age of the patient and multi-hormonal appearance of the neoplasm, a gene analysis for MEN1 and VHL was performed and was negative.
Conclusion: This is a rare case of functional PNEN with two different pancreatic lesions producing competitive hormones. The clinical symptoms are compatible with the larger producing neoplasm.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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