Universität zu Köln

von Baumgarten, Louisa, Illerhaus, Gerald, Korfel, Agnieszka, Schlegel, Uwe, Deckert, Martina and Dreyling, Martin (2018). The Diagnosis and Treatment of Primary CNS Lymphoma. Dtsch. Arztebl. Int., 115 (25). S. 419 - 433. COLOGNE: DEUTSCHER AERZTE-VERLAG GMBH. ISSN 1866-0452

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Abstract

Background: Primary central nervous system lymphoma is a diffuse large B-cell lymphoma with exclusive manifestation in the central nervous system (CNS), leptomeninges, and eyes. Its incidence is 0.5 per 100 000 persons per year. Currently, no evidence-based standard of care exists. Methods: This review is based on pertinent publications (2000-2017) retrieved by a selective search in PubMed. Results: The clinical and neuroradiological presentation of primary CNS lymphoma is often nonspecific, and histopathological confirmation is obligatory. The disease, if left untreated, leads to death within weeks or months. If the patient's general condition permits, treatment should consist of a high-dose chemotherapy based on methotrexate (HD-MTX) combined with rituximab and other cytostatic drugs that penetrate the blood-brain barrier. Long-term survival can be achieved in patients under age 70 by adding non-myeloablative consolidation chemotherapy or high-dose chemotherapy with autologous stem cell transplantation (HD-AST) to the induction therapy. Clinical trials comparing the efficacy and toxicity of these two treatment strategies are currently underway. Consolidation whole-brain radiotherapy is associated with the risk of severe neurotoxicity and should be reserved for patients who do not qualify for systemic treatment. Some 30% of patients are refractory to primary treatment, and at least 50% relapse. In patients who are still in good general condition, relapse can be managed with HD-AST. Re-exposure to conventional HD-MTX-based polychemotherapy is another option, if the initial response was durable. The 5-year survival rate of all treated patients is 31%, according to registry data. Conclusion: Current recommendations for the treatment of primary CNS lymphoma are based on only a small number of prospective clinical trials. Patients with this disease should be treated by interdisciplinary teams in experienced centers, and preferably as part of a controlled trial.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code von Baumgarten, Louisa UNSPECIFIED UNSPECIFIED UNSPECIFIED Illerhaus, Gerald UNSPECIFIED UNSPECIFIED UNSPECIFIED Korfel, Agnieszka UNSPECIFIED UNSPECIFIED UNSPECIFIED Schlegel, Uwe UNSPECIFIED UNSPECIFIED UNSPECIFIED Deckert, Martina UNSPECIFIED UNSPECIFIED UNSPECIFIED Dreyling, Martin UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-182682
DOI:	10.3238/arztebl.2018.0419
Journal or Publication Title:	Dtsch. Arztebl. Int.
Volume:	115
Number:	25
Page Range:	S. 419 - 433
Date:	2018
Publisher:	DEUTSCHER AERZTE-VERLAG GMBH
Place of Publication:	COLOGNE
ISSN:	1866-0452
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language CENTRAL-NERVOUS-SYSTEM; WHOLE-BRAIN RADIOTHERAPY; HIGH-DOSE CHEMOTHERAPY; INTERNATIONAL EXTRANODAL LYMPHOMA; STEM-CELL TRANSPLANTATION; MULTICENTER PHASE-II; QUALITY-OF-LIFE; IMMUNOCOMPETENT PATIENTS; DIFFERENTIAL-DIAGNOSIS; INTENSIVE CHEMOTHERAPY Multiple languages Medicine, General & Internal Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/18268