ECE2022 Eposter Presentations Endocrine-Related Cancer (61 abstracts)
Multiple endocrine neoplasia type 1: A puzzle that builds over the years
Mnif Fatma 1 , Kawthar El Arbi 1 , Yosra Lajmi 2 , Asma Zargni 1 , Faten Haj Kacem Akid 1 , Hamdi Frikha 1 , Dhoha Ben Salah 1 , Nadia Charfi 1 , Mouna Mnif 1 , Nabila Rekik Majdoub 1 , Mouna Elleuch 1 , Hassen Kammoun 2 , Fatma Abdelhédi 2 & Mohamed Abid 1
1Hedi Chaker Hospital, Department of Endocrinology, Sfax, Tunisia; 2Hedi Chaker Hospital, Department of Medical Genetics, Sfax, Tunisia
Introduction: Multiple endocrine neoplasia type 1 (MEN1) is a genetic disease that predisposes to the development of both hyperplastic and tumorous lesions of the endocrine glands, in particular parathyroid, pancreatic and pituitary. Thus we report our case.
Observation: An 18-year-old male patient was hospitalized for severe hypoglycemia mistakenly treated as epileptic seizures. The etiological investigation concluded to a benign multiple insulinoma, confirmed by the anatomopathological study. The patient was lost to follow-up and was re-hospitalized 11 years later for an intracranial hypertension related to a macro-prolactinoma of 7 cm of diameter. Prolactin level was 11300µg/l. A MEN1 was suspected. The investigation of additional associated lesions revealed a primary hyperparathyroidism. The evolution was fatal with the death of the patient in a cerebral herniation. The genetic study was not performed.
Discussion: Parathyroid tumors are the first manifestation of MEN1 in over 85% of cases. Less than 15%, the first manifestation may be an insulinoma or prolactinoma, likewise our patient. The chronology of lesion onset could differ from patient to another. The clinical presentation is usually completed over time. Thus a continuous screening of the different lesions is necessary. For our patient, the discovery of a primary hyperparathyroidism as well as a prolactinoma 11 years later make it particular.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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