Journal of neurology · 2026
Radiological and FDG-PET imaging features of Epstein-Barr virus-positive primary central nervous system lymphomas.
СтатьяМногоцентровое исследованиеНазвание и аннотация: Перевод в очереди
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Schulz Nina, Herrán de la Gala Dario, Rozenblum Laura, Lazzari Patrizia, Morel Véronique, Boussen Ines, Abraham Julie, Le Cann Marie, Soussain Carole, Dorel Marie, Ursu Renata, Leclercq Delphine, Blonksi Marie, Mokhtari Karima, Mathon Bertrand, Hoang-Xuan Khe, Choquet Sylvain, Houillier Caroline, Nichelli Lucia
Аннотация
BACKGROUND: Epstein-Barr virus (EBV)-associated primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin's lymphoma closely linked to immunodeficiency. Imaging characteristics of EBV-associated are reported to differ from those of typical EBV-negative PCNSL. This study aims to describe the radiological and nuclear medicine imaging features in a large cohort of patients with EBV-associated PCNSL.
METHODS: We conducted a multicenter retrospective descriptive study between 2008 and 2025 on patients with a diagnosis of EBV-associated PCNSL. MRI variables and FDG-PET/CT uptake were assessed.
RESULTS: Fifty-eight cases of EBV-associated PCNSL were included. All but 1 patient were immunosuppressed. Multiple lesions were present in 71% of cases (41/58). Supratentorial involvement was observed in 90% of cases (52/58). Heterogeneous contrast enhancement was noted in 90% (52/58), with ring-like enhancement in 41% (24/58). Leptomeningeal enhancement occurred in 31% of cases (18/58), and within this group, 50% showed perivascular space enhancement. Lesions showed hypercellularity in 83% (48/58) and intralesional hemorrhage in 81% (47/58). An "eccentric target" sign was present in 26% of cases (15/58), while a "concentric target" sign in 14% (5/35). On FDG-PET, 25/30 patients had hypermetabolic lesions (25/30, 83%).
CONCLUSION: Diagnosing EBV-associated PCNSL is challenging due to its rarity and the broad differential diagnosis. Multiple necrotic and hemorrhagic lesions are the most suggestive MRI feature of EBV-associated PCNSL. "Eccentric" and "concentric" target signs, typically associated with CNS toxoplasmosis, can be observed. FDG-PET often reveals hypermetabolic lesions that support a neoplastic diagnosis. Histological confirmation remains essential for confidently treating this tumor entity.
METHODS: We conducted a multicenter retrospective descriptive study between 2008 and 2025 on patients with a diagnosis of EBV-associated PCNSL. MRI variables and FDG-PET/CT uptake were assessed.
RESULTS: Fifty-eight cases of EBV-associated PCNSL were included. All but 1 patient were immunosuppressed. Multiple lesions were present in 71% of cases (41/58). Supratentorial involvement was observed in 90% of cases (52/58). Heterogeneous contrast enhancement was noted in 90% (52/58), with ring-like enhancement in 41% (24/58). Leptomeningeal enhancement occurred in 31% of cases (18/58), and within this group, 50% showed perivascular space enhancement. Lesions showed hypercellularity in 83% (48/58) and intralesional hemorrhage in 81% (47/58). An "eccentric target" sign was present in 26% of cases (15/58), while a "concentric target" sign in 14% (5/35). On FDG-PET, 25/30 patients had hypermetabolic lesions (25/30, 83%).
CONCLUSION: Diagnosing EBV-associated PCNSL is challenging due to its rarity and the broad differential diagnosis. Multiple necrotic and hemorrhagic lesions are the most suggestive MRI feature of EBV-associated PCNSL. "Eccentric" and "concentric" target signs, typically associated with CNS toxoplasmosis, can be observed. FDG-PET often reveals hypermetabolic lesions that support a neoplastic diagnosis. Histological confirmation remains essential for confidently treating this tumor entity.