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A Rare Case Of Spontaneous Remission And Relapse Of A Primary Central Nervous System Lymphoma

Ramos, R. 2018Lymphoma

Ramos, R., Fernandes, J. S., Almeida, M., & Almeida, R. (2018). A Rare Case of Spontaneous Remission and Relapse of a Primary Central Nervous System Lymphoma. Acta medica portuguesa, 31(12), 777–783. https://doi.org/10.20344/amp.10198

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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.

Case Details

Disease Location

Brain

Personal Characteristics

50-year-old female, medicated with a serotonin reuptake inhibitor (escitalopram), bromazepam and oral contraceptive

Clinical Characteristics

January 2014: - she began to complain of blurred vision. - CT scan revealed a 3 cm suprasellar hyperdense lesion - prolactin values (prl) of 183 ng/ml - macroprolactinoma was suspected. May 2014: - symptoms resolved without treatment - MRI showed remission of the suprasellar lesion but also showed two new nodular lesions, with the largest in the right caudate nucleus with subependymal expression, and the other in the anterior part of the third ventricle. - new prl of 76 ng/ml. - the diagnosis of macroprolactinoma was dropped and an inflammatory condition was suspected. August 2014: - symptoms reappeared - brain MRI revealed regression of the lesion in the anterior portion of the third ventricle, and marked enlargement of the right caudate nucleus periventricular lesion. - cns lymphoma was hypothesized october 2014: - right caudate nucleus lesion biopsy - the pathology revealed a diffuse large b-cell lymphoma. - chemotherapy was initiated with high-dose cytarabine + high-dose methotrexate + rituximab and radiotherapy

Remission Characteristics

May 2014: without any type of medication and after clinical improvement of the visual complaints. The MRI showed remission of the suprasellar lesion.

Treatment & Mechanisms

Proposed Remission Mechanisms

Concomitant viral infection leading to an increased number of body natural killer cells, which attack the tumor cells.

Clinical Treatment

- right caudate nucleus lesion biopsy (after remission) - chemotherapy was initiated with high-dose cytarabine + high-dose methotrexate + rituximab and radiotherapy (45 gray fractionated in 4.5 gray/day, 5 times/week)