Case report and review of the literature of primary central nervous system lymphoma of the fourth ventricle
- Department of Neurosurgery, Osaka Metropolitan University, Osaka, Japan.
- Department of Hematology, Sumitomo Hospital, Osaka, Japan.
- Department of Pathology, Osaka Metropolitan University, Osaka, Japan.
Kosuke Nakajo, Department of Neurosurgery, Osaka Metropolitan University, Osaka, Japan.
DOI:10.25259/SNI_654_2022Copyright: © 2022 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Yuichiro Kojima1, Kosuke Nakajo1, Tsutomu Ichinose1, Yoichiro Morikawa2, Masahiko Osawa3, Takeo Goto1. Case report and review of the literature of primary central nervous system lymphoma of the fourth ventricle. 11-Nov-2022;13:529
How to cite this URL: Yuichiro Kojima1, Kosuke Nakajo1, Tsutomu Ichinose1, Yoichiro Morikawa2, Masahiko Osawa3, Takeo Goto1. Case report and review of the literature of primary central nervous system lymphoma of the fourth ventricle. 11-Nov-2022;13:529. Available from: https://surgicalneurologyint.com/?post_type=surgicalint_articles&p=11987
Background: Primary central nervous system lymphoma of the fourth ventricle is very rare. We present a case of primary central nervous system lymphoma originating from the fourth ventricle and review cases reported in the literature.
Case Description: A 54-year-old man with no previous medical history presented with headache and nausea. Magnetic resonance imaging showed a homogeneously enhancing tumor in the fourth ventricle and obstructive hydrocephalus. We performed biopsy of the tumor, which was diagnosed pathologically as diffuse large B-cell lymphoma. Although the tumor disappeared after 5 cycles of R-MPV regimen, the patient required repeated ventricular drainage and finally received a ventriculoperitoneal shunt. Complete response was achieved after 2 cycles of high-dose cytarabine chemotherapy with an autologous peripheral blood stem cell transplant. There was no sign of recurrence at 20 months after biopsy.
Conclusion: Morbidity arising due to radical resection/radiotherapy of resistant primary central nervous system lymphoma originating from the fourth ventricle could be prevented by ventriculoperitoneal shunting with chemotherapy and autologous blood stem cell transplantation.
Keywords: Fourth ventricle, Lymphoma, Hydrocephalus
Primary central nervous system lymphoma (PCNSL) is a rare variant of extranodal nonHodgkin’s lymphoma. The incidence rate has recently increased to 0.43 cases/100,000 people/ year.[
A 54-year-old man with no relevant previous medical or family history presented to our hospital complaining of headache, nausea, diplopia, and dizziness. Neurological examination revealed bilateral abducens nerve palsy and left facial nerve palsy and ataxia. Computed tomography (CT) showed a slightly high-density tumor [
The tumor showed slightly high density on CT (a), slightly hypointensity on T1-weighted image (b), iso- to slightly high intensity on a T2-weighted image (c), uniformly high intensity on diffusion-weighted image (d), and heterogeneous enhancement with gadolinium (e: axial, f: sagittal, and g: coronal).
Biopsy was performed by the transmedullary fissure approach. Histopathological examination revealed diffusely growing tumor cells with a high nucleocytoplasmic ratio and differently sized nuclei, some of which had mucus and multiple mitotic figures [
Histopathological examination revealed diffusely growing tumor cells with a high nucleocytoplasmic ratio and differently sized nuclei, some of which had mucus, and multiple mitotic figures (a). The tumor was negative for CD10 (b) and was positive for CD20 (c), CD79a (d), Bcl-6 (e), and MUM-1 (f). a: Hematoxylin-eosin staining, ×200, b: CD10, ×400, c: CD20, ×400, d: CD79a, ×400, e: Bcl-6, ×400, and f: MUM-1, ×400.
The tumor decreased in size but the obstructive hydrocephalus remained after 5 cycles of R-MPV regimen (a: axial and b: sagittal). The patient achieved complete response after chemotherapy with autologous peripheral blood stem cell transplantation. There was no sign of recurrence at 20 months after biopsy (c: axial and d: sagittal).
The incidence rate of PCNSLs has increased recently and is currently 0.43/100,000 people/year.[
Our literature review of PCNSL originating from fourth ventricle [
The treatment for PCNSL has traditionally been whole-brain radiation therapy alone; however, median survival has been reported as 8–12 months and the 5-year survival rate as 6%.[
PCNSLs are often highly responsive to steroids, with an efficacy rate of 40%, because steroid glucocorticoid is a strong inducer of apoptosis of lymphoid cells.[
We report an extremely rare case of PCNSL originating from the fourth ventricle. Morbidity arising due to radical resection/radiotherapy of resistant PCNSL originating from the fourth ventricle could be prevented by ventriculoperitoneal shunting with chemotherapy and autologous blood stem cell transplantation.
The authors certify that they have obtained all appropriate patient consent.
There are no conflicts of interest.
The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.
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