ECE2023 Symposia Aberrant/illicit expression of receptors in adrenal lesions (3 abstracts)
LHCG Receptor in pheochromocytoma
Antoine-Guy Lopez 1 , Céline Duparc 2 , Sylvie Renouf 2 , Jean-Christophe Sabourin 3 , Alexandre Buffet 4 , Anne-Paule Gimenez-Roqueplo 4 , Christophe Dubessy 2 , Estelle Louiset 2 & Hervé Lefebvre 1
1Univ Rouen Normandie, INSERM, NORDIC UMR 1239, CHU Rouen, Department of Endocrinology, F-76000 Rouen, France; 2Univ Rouen Normandie, INSERM, NORDIC UMR 1239, F-76000 Rouen, France; 3Univ Rouen Normandie, INSERM, UMR 1245, CHU Rouen, Department of Pathology, Tumor BioBank-Centre for Biological Resources, F-76000 Rouen, France; 4Univ Paris, PARCC, INSERM, Department of Genetics, AP-HP, European Hospital Georges Pompidou, Paris, France
Pheochromocytomas/paragangliomas (PPGL) are catecholamine-producing neuroendocrine tumors that account for less than 1% of all hypertension cases. Despite their rarity, these tumors must be early detected and treated to prevent potentially life-threatening adrenergic crises. Unfortunately, PPGL are frequently unrecognized during pregnancy, leading to a high risk of either maternal or fetal complications. In return, pregnancy can trigger catecholamine secretory discharges in patients with PPGL through diverse mechanisms which are not totally understood. The observation that symptoms of catecholamine excess may occur during the first trimester of gestation suggests that pregnancy may activate the secretory activity of PPGL through the involvement of gestational hormones. We have recently reported a case of silent pheochromocytoma revealed in a pregnant woman by adrenergic myocarditis. In vitro studies revealed that hCG stimulated epinephrine secretion by cultured cells derived from the patient’s pheochromocytoma. The tumor expressed the LHCG receptor (LHCGR), which was colocalized with catecholamine-producing enzymes. Expression of the LHCGR was also detected in an independent series of pheochromocytomas. In silico studies have been conducted in The Cancer Genome Atlas (TCGA) cohort and French COMETE databases which contain genomic and transcriptomic analyses of solid tumor types including PPGL. They revealed that PPGL display the highest expression levels of LHCGR mRNA among the 32 tumor types of TCGA. Interestingly, LHCGR expression level was higher in epinephrine-secreting PPGL, i.e. related to RET, NF1, TMEM127, MAX mutations, vs other subgroups of PPGL, and in pheochromocytomas vs paragangliomas. These data show that pregnancy can favor adrenergic crises in patients with PPGL through hCG-induced stimulation of epinephrine production by the tumors. They indicate that preconceptional diagnosis is particularly important in patients with gene mutations that predispose to epinephrine-secreting pheochromocytomas, who may experience signs of catecholamine excess as soon as the first trimester of gestation.
Volume 90
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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