A Rare Case of Spontaneous Remission and Relapse of a Primary Central Nervous System Lymphoma

Acta Med Port. 2018 Dec 28;31(12):777-783. doi: 10.20344/amp.10198. Epub 2018 Dec 28.

Abstract

Primary central nervous system lymphoma remission after steroid treatment is a well-known phenomenon, but remission without any type of treatment is extremely rare. We present a rare case of spontaneous remission of a diffuse large B-cell lymphoma of the central nervous system as well as its subsequent reappearance in another location. The atypical presentation misled the neurosurgeons and neurologists, delaying diagnosis and treatment. The patient underwent brain biopsy after the relapse and started radiotherapy and chemotherapy with cytarabine + methotrexate + rituximab. As of 32 months after the diagnosis, the patient remained asymptomatic, with no focal neurological deficits and the disease in complete remission. A PubMed search of the literature up to June 2017 regarding spontaneous remission central nervous system lymphoma was also carried out.

É bem conhecida a remissão com corticoterapia dos linfomas primários do sistema nervoso central, mas a sua remissão sem qualquer tipo de tratamento é extremamente rara. Apresentamos um caso raro de uma remissão espontânea e posterior recidiva noutra localização de um linfoma difuso de grandes células B do sistema nervoso central. A apresentação atípica deste caso confundiu os neurocirurgiões e neurologistas, atrasando o diagnóstico e tratamento. A doente foi submetida a biópsia de uma das lesões cerebrais e, posteriormente, iniciou radioterapia e quimioterapia com citarabina + metotrexato + rituximab. Neste momento, 32 meses após o diagnóstico histológico, a doente encontra-se assintomática, sem défices neurológicos focais e com remissão completa da doença. Foi também efetuada uma pesquisa na PubMed até junho de 2017 sobre a remissão espontânea dos linfomas do sistema nervoso central.

Keywords: Central Nervous System Neoplasms/drug therapy; Central Nervous System Neoplasms/radiotherapy; Lymphoma/drug therapy; Lymphoma/radiotherapy.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Brain / diagnostic imaging
  • Brain / pathology
  • Brain Neoplasms / diagnostic imaging
  • Brain Neoplasms / pathology
  • Central Nervous System Neoplasms / diagnostic imaging*
  • Central Nervous System Neoplasms / drug therapy
  • Central Nervous System Neoplasms / pathology
  • Female
  • Humans
  • Lymphoma, Large B-Cell, Diffuse / diagnostic imaging*
  • Lymphoma, Large B-Cell, Diffuse / drug therapy
  • Lymphoma, Large B-Cell, Diffuse / pathology
  • Magnetic Resonance Imaging
  • Middle Aged
  • Neoplasm Recurrence, Local / diagnostic imaging*
  • Neoplasm Recurrence, Local / drug therapy
  • Neoplasm Recurrence, Local / pathology
  • Neoplasm Regression, Spontaneous*
  • Remission Induction