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Endocrine Abstracts (2025) 110 EP1266 | DOI: 10.1530/endoabs.110.EP1266

1Department of Endocrinology, Diabetology, and Metabolism, Lille University Hospital, Lille, France; 2University of Lille, CHU-Lille, Inserm U1172, Lille Neuroscience & Cognition, Lille, France; 3Department of Hematology, Lille University Hospital, Lille, France; 4Department of Endocrine and Metabolic Surgery, Lille University Hospital, Lille, France; 5CANTHER-Cancer-Heterogeneity Plasticity and Resistance to Therapies, University of Lille, UMR9020-U1277-CNRS, INSERM, CHU Lille, Lille, France; 6Department of Neurosurgery, Lille University Hospital, Lille, France., Lille, France; 7Department of Radiology, Lille University Hospital, Lille, France; 8INSERM U1190 Translational Research in Diabetes, Firendo Rare Diseases Competence Center PRISIS, (Pathologies of insulin Resistance and Insulin Sensitivity), Lille University Hospital, Lille, France


JOINT2020

A rare and aggressive form of B-cell lymphoma known as intravascular large B-cell lymphoma (IVLBCL) is characterized by the growth of large cells within the lumen of blood vessels. While previous studies have documented a wide range of symptoms caused by neoplastic cells obstructing small vessels in various organs, there is limited literature on IVLBCL cases with hypothalamic-pituitary functional impairment. Here, we present a rare case of IVLBCL that provoked hypopituitarism. A 58-year-old male patient presented with symptoms that progressively developed over several weeks, including deteriorated general condition, intermittent episodes of confusion, daily high fever, and polyuropolydipsia syndrome (5 liters per day). Laboratory investigations revealed mild hypernatremia (sodium, 149 mEq/l)as well as elevated serum levels of lactate dehydrogenase (LDH; 566 IU/l)and β2 microglobulin 8.31 mg/l(0.8-2.34). Basal hormone analysis demonstrated hypopituitarism. Brain magnetic resonance imaging (MRI), however, showed a lesion infiltrating the floor of the third ventricle (21 × 18 × 15 mm), extending to encompass the entire pituitary stalk. The lesion appeared homogeneous and exhibited strong contrast enhancement after gadolinium administration. 18F-fluorodeoxyglucose (FDG)-positron emission tomography revealed enhanced FDG uptake of the lesion infiltrating the floor of the third ventricle, extending to encompass the entire pituitary stalk. In the biopsy of the clinically unaffected skin, immunohistochemistry revealed the presence of intravascular neoplastic cells positive for B-cell markers CD20, CD5, and MUM1, confirming the diagnosis of intravascular large B-cell lymphoma. Corticotropic and thyrotropic deficiencies were supplemented, and diabetes insipidus was treated with desmopressin. Additionally, the patient underwent R-CHOP chemotherapy, which resulted in a complete response. The patient remained in remission for six months following the cessation of chemotherapy. Hypopituitarism associated with IVLBCL was first described in 1986. A limited number of cases of IVLBCL involving functional abnormalities of the hypothalamic-pituitary axis have been reported. IVLBCL with hypothalamic or pituitary involvement can be effectively managed with hormone replacement therapy followed by chemotherapy. Consequently, timely identification of clinicopathological features is crucial for reducing mortality rates.

Volume 110

Joint Congress of the European Society for Paediatric Endocrinology (ESPE) and the European Society of Endocrinology (ESE) 2025: Connecting Endocrinology Across the Life Course

European Society of Endocrinology 
European Society for Paediatric Endocrinology 

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