EYES2024 ESE Young Endocrinologists and Scientists (EYES) 2024 Adrenals and Neuroendocrine Tumors (17 abstracts)
Adrenocortical carcinoma: new insights from a tunisian cohort
Hamdi Frikha 1 , Fatma Mnif 1 , Dhoha Ben Salah 1 , Yesmine Abdelkafi 1 , Khouloud Boujelben 1 , Faten Hadjkacem 1 , Nadia Charfi 1 , Mouna Mnif 1 , Mohamed Abid 1 , Mouna Elleuch 1 & Nabila Mejdoub Rekik 1
1Endocrinology Department, Hedi Chaker University Hospital, Sfax, Tunisia
Introduction: Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with poor prognosis despite diagnostic and therapeutic advances. Tailoring personalized management strategies of ACC in low and middle income countries is hindered by data scarcity. This study aims to specify clinico-biological presentation and therapeutic outcomes of patients with ACC in the context of a developing country.
Methods: We reviewed demographic, clinical, pathology and follow-up data of 11 patients followed for ACC from 2010 to 2022 in a referral endocrine center of the region of Sfax, Tunisia.
Results: Our population was remarkably feminine (8/11 patients) with a mean age at diagnosis of 43 ±3 years. Compression signs and hyperandrogenism were the commonest initial presentation features. Autonomous secretion was present in 7 cases: hyperaldosteronism (3/11), hyperandrogenism (3/11) and hypercortisolism(1/11). ACCs were predominantly right-located and heterogeneous with a mean size of 14,8 ±4 cm. Six patients had metastatic localization at presentation. Mean ENSAT stage at diagnosis was 3±1 and 7 patients had Weiss score >6. Heterozygosity loss of 17p13 was identified in one case. Complete resection was achieved in 6 cases out of 11. Only 7 and 3 patients had adjuvant treatment with mitotane and chemotherapy (EDP protocol) respectively. At 7±5 years of follow up, relapse rate was 50% and mortality rate 28%.
Conclusion: We meet the epidemiological and clinical profile of ACCs as observed in large Western cohorts. However, we highlight alarming traits specific to our context: diagnostic delay leading to severe presentations with tumor size, cancer stage, and histopathological score significantly more advanced than those reported in the literature, the unavailability of biomolecular study platforms limiting the possibility of personalized management, and limited access to adjuvant therapies due to economic constraints in developing countries. These factors may explain the disappointing therapeutic outcomes and the poorer prognosis of ACCs in our context.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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