|Year : 2021 | Volume
| Issue : 1 | Page : 25-27
Primary intraventricular central nervous system lymphoma of lateral ventricle in an immunocompetent patient
Kavita Mardi, Lalita Negi, Tenzin Lanzhe, Pooja Murgai
Department of Pathology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
|Date of Submission||10-Apr-2021|
|Date of Acceptance||16-Feb-2022|
|Date of Web Publication||12-Apr-2022|
Dr. Kavita Mardi
Department of Pathology, Indira Gandhi Medical College, Set No 14, Type VI Quarters, IAS Colony, Meheli, Shimla, Himachal Pradesh.
Source of Support: None, Conflict of Interest: None
Primary CNS lymphoma (PCNSL) is a rare variant of extranodal non-Hodgkin’s lymphoma and accounts for 3%–5% of all primary brain tumors. Their intraventricular location is extremely rare with only a few cases on record so far. All the reported cases showed bilateral ventricular involvement. We report the first case of unilateral lateral ventricle PCNL in a 22-year-old female who presented with intractable vomiting and headaches. Computed tomography (CT) followed by magnetic resonance imaging (MRI) revealed lobulated altered signal intensity lesion in the left temporal horn of lateral ventricle with surrounding peritumoral edema causing midline shift. With these imaging features, the diagnosis of intraventricular meningioma was suggested. Microscopic examination of the excised mass revealed round-to-ovoid tumor cells with scant eosinophilic cytoplasm with large and hyperchromatic nuclei, prominent nucleoli, irregular nuclear membrane, and frequent mitotic figures. The tumor cells are positive for leukocyte common antigen, CD20, CD10, CD43; and were negative for CD3, synaptophysin, chromagranin, pan-cytokeratin (pan-CK), Epstein-Barr virus (EBV), and glial fibrillary acidic protein. The Ki-67 was very high (80%). With these histopathological and immunohistochemistry (IHC) findings, diagnosis of NHL of diffuse large B cell type was rendered.
Keywords: CNS, intraventricular, lateral ventricle, lymphoma, NHL
|How to cite this article:|
Mardi K, Negi L, Lanzhe T, Murgai P. Primary intraventricular central nervous system lymphoma of lateral ventricle in an immunocompetent patient. Int J Neurooncol 2021;4:25-7
| Introduction|| |
Primary CNS lymphoma (PCNSL) is a rare variant of extranodal non-Hodgkin’s lymphoma and accounts for 3%–5% of all primary brain tumors. These tumors are almost always found within the brain parenchyma and may be superficial (subpial) or deep-seated (subependymal). Intraventricular PCNSLs are extremely rare with less than 20 cases reported in literature to date. All the four reported cases in the lateral ventricle show bilateral involvement.,, We describe one such rare occurrence of PCNSL in the lateral ventricle of a 22-year-old woman. This is the first reported case involving a unilateral lateral ventricle.
| Case report|| |
A 22-year-old woman presented with intractable vomiting and headaches. She had no previous medical history. The findings of the neurological examination were normal. There was no motor or sensory deficit and cranial nerves were normal. The rest of the clinical examination revealed normal findings. Her initial blood work was negative. Computed tomography (CT) followed by magnetic resonance imaging (MRI) revealed lobulated altered signal intensity lesion in the left temporal horn of lateral ventricle with surrounding peritumoral edema causing midline shift [Figure 1]. With these imaging features, the diagnosis of intraventricular meningioma was suggested. Left pterional craniotomy with excision of large fleshy extra-axial of size 5 cm × 4 cm from left lateral ventricle was performed. Microscopic examination revealed round-to-ovoid tumor cells with scant eosinophilic cytoplasm with large and hyperchromatic nuclei with prominent nucleoli and irregular nuclear membrane [Figure 2]. Frequent mitotic figures were present, and tumor cells are positive for leukocyte common antigen, CD20 [Figure 3], CD10, CD43; and were negative for CD3, synaptophysin, chromagranin, pan-cytokeratin (pan-CK), Epstein-Barr virus (EBV), and glial fibrillary acidic protein. The Ki-67 was very high (80%). These histological and immunohistochemistry (IHC) findings were suggestive of non-Hodgkin’s lymphoma (diffuse large B-cell type). Postoperatively, the patient recovered without any deficit. She was commenced on chemotherapy with intrathecal/intravenous methotrexate and remained well 2 months after the diagnosis.
|Figure 1: MRI revealing lobulated lesion in the left temporal horn of lateral ventricle with surrounding peritumoral edema|
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|Figure 2: Round to ovoid tumor cells with scant eosinophilic cytoplasm, large, and hyperchromatic nuclei with prominent nucleoli and irregular nuclear membrane (H&E, 40×)|
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| Discussion|| |
Primary central nervous system lymphoma (PCNSL) is a rare type of extra-nodal non-Hodgkin lymphoma (NHL) that can be found in the brain, leptomeninges, and spinal cord without evidence of systemic lymphoma. PCNSL constitutes 1% of all NHLs and 3%–5% of all primary brain tumors. Intraventricular PCNSLs are extremely rare with only few case reports on record.,,,,,, Most of these case reports have described PCNSL located in a single cerebral ventricle except the involvement of the right lateral ventricle and fourth ventricle in one of the reports.
As opposed to secondary CNS lymphoma, the lesions of PCNSL are nearly always found within the brain parenchyma. Pure intraventricular involvement by PCNSL is very rare. In the majority of immunocompetent patients, the lesions are solitary and most are supratentorial, with the posterior fossa being a somewhat rare location. Multiple lesions have been reported in 20%–40% of nonautoimmune deficiency syndrome (AIDS) PCNSL. The association between AIDS-related PCNSL and EBV expression in the tumor is approximately 100%. By contrast, EBV is rarely detected in PCNSL of immunocompetent patients, suggesting a different pathogenesis in each group. Our patient is relatively younger, immunocompetent, had a large mass in left lateral ventricle, and did not harbor EBV in the tumor. Approximately 90% of cases of intraventricular lymphomas are aggressive, diffuse large B-cell lymphoma (DLBCL) with a poor outcome. This is followed by T-cell lymphomas and mucosa-associated lymphoid tissue (MALT) lymphoma. Other types of lymphoma, such as low-grade subtypes, are extremely rare.
The presence of homogeneously enhanced intraventricular MRI lesions should raise the suspicion of PCNSLs. Although advanced imaging techniques can increase the diagnostic accuracy and help in differentiating PCNSL from other tumorous or nontumorous lesions, brain biopsy is required to confirm the diagnosis. Differential diagnosis for intraventricular CNS lesions includes central neurocytomas, meningiomas, ependymomas, choroid plexus papilloma, metastasis, neurocysticercosis, and rarely PCNSL.
The most important role of imaging in PCNSL is directing clinicians to perform a stereotactic biopsy or obtain cerebrospinal fluid (CSF) in order to obtain a histologic diagnosis and avoid futile attempts at resection.
Resection does not play a therapeutic role in PCNSL and can worsen neurologic deficits. Overall, prognosis has improved over the past several decades and PCNSL is a very chemosensitive and radiosensitive tumor. Most oncologists agree on a methotrexate chemotherapy regimen in addition to whole-brain radiation, although there is an increased risk of neurotoxicity with radiation.
In conclusion, the role of imaging in aiding to distinguish from other tumors seen in intraventricular location and the diagnosis of PCNSL is essentially based on biopsy and histopathological examination. It is important that primary B-cell lymphoma should be included in the list of differential diagnosis of intraventricular tumors.
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Conflicts of interest
There are no conflicts of interest.
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