Multiple central nervous system lesions on chronic corticosteroid therapy – a diagnostic challenge

• Main Authors: Rute Caçola, Luís Augusto, Miguel Bernardes, Raquel Real
• Format: Article
• Language: English
• Published: Sociedade Galega de Medicina Interna 2015-03-01
• Series: Galicia Clínica
• Subjects: Neoplasias primarias del sistema nervioso central; Linfoma difuso de células B grandes; Infecciones causadas por el virus de Epstein-Barr; Corticoides
• Online Access: http://www.galiciaclinica.info/publicacion.asp?f=646
• ISSN: 0304-4866; 1989-3922

Primary central nervous system lymphoma (PSNCL) is a rare extranodal non-Hodgkin´s lymphoma (NHL), representing less than 1% of NHLs and approximately 2% of all primary brain tumors. PCNSL can be a diagnostic challenge, presenting with variable neurological symptoms that reflect involvement of the brain parenchyma and, more rarely, other nervous structures, such as the eye, spinal cord or peripheral nerves. There is no formal consensus regarding how diagnostic evaluation for suspected CNS lymphoma should be performed, which may result in a delayed diagnosis. By writing up this clinical case and review of the literature, further evidence is provided for loss of distinctive histological and radiographic findings of PCNSLs on corticosteroid therapy, which may difficult the diagnosis workup . A 75-year-old woman with systemic lupus erythematosus chronically medicated with Hydroxychloroquine 400 mg, Deflazacort 9 mg and Azathioprine 25mg daily, presented to the hospital with a 5-month history of progressive neurological deficits. As in most CNS lymphomas developing in immunocompromised individuals, she was diagnosed with diffuse large B-cell lymphoma, Epstein-Barr virus positive. This clinical case was particularly challenging, because lesions were not accessible to biopsy and therapy with corticosteroids masqueraded and delayed the diagnosis of PCNSL, via the profound apoptotic effect of corticosteroids on lymphoma cells, causing them to disappear in a very short time interval (“vanishing lymphoma” phenomenon), and reducing lesion contrast enhancement on brain MRI. As reported in the literature, the clinical course was aggressive, and the patient died 7 months after the first symptoms.