|Year : 2016 | Volume
| Issue : 3 | Page : 188-191
Pediatric intraventricular B-cell lymphoma and malignant meningioma: A report of two rare cases and review of literature
Pravin Survashe, Hrushikesh U Kharosekar, Harish Naik, Vernon Velho
Department of Neurosurgery, Grant Medical College and Sir J. J. Group of Hospitals, Mumbai, Maharashtra, India
|Date of Web Publication||5-Aug-2016|
Dr. Hrushikesh U Kharosekar
Department of Neurosurgery, Grant Medical College and Sir J. J. Group of Hospitals, Mumbai, Maharashtra
Source of Support: None, Conflict of Interest: None
Intraventricular tumors are rare masses that are found less commonly in the pediatric population. These tumors occupy a nonfunctional space and are often able to grow into a considerable size before clinical manifestations; these masses are treated surgically but pose a significant challenge to neurosurgeons. Primary central nervous system lymphoma (PCNSL) is a rare tumor accounting for 4% of all brain neoplasms, <10 cases have been described in the literature. Intraventricular meningiomas (IVMs) are rare tumors, accounting for 0.5-5% of all intracranial meningiomas. Malignant IVM (MIVM) is even rarer.
Keywords: B-cell, intraventricular, lymphoma, malignant meningioma, pediatric
|How to cite this article:|
Survashe P, Kharosekar HU, Naik H, Velho V. Pediatric intraventricular B-cell lymphoma and malignant meningioma: A report of two rare cases and review of literature. Int J Health Allied Sci 2016;5:188-91
|How to cite this URL:|
Survashe P, Kharosekar HU, Naik H, Velho V. Pediatric intraventricular B-cell lymphoma and malignant meningioma: A report of two rare cases and review of literature. Int J Health Allied Sci [serial online] 2016 [cited 2019 Sep 20];5:188-91. Available from: http://www.ijhas.in/text.asp?2016/5/3/188/187835
| Introduction|| |
Intraventricular tumors are rare masses that are found less commonly in the pediatric population (<1%). The most common pediatric intraventricular tumors include ependymomas, choroid plexus papillomas (CPPs), and astrocytomas that frequently occur in the lateral and frontal horn. Less common intraventricular tumors include subependymal giant cell astrocytomas, oligodendrogliomas, subependymomas, pilocytic astrocytomas, neurocytoma, and choroid plexus carcinomas. These tumors occupy a nonfunctional space and are often able to grow into a considerable size before clinical manifestations. Given their benign nature, these masses are treated surgically but pose a significant challenge to neurosurgeons. 
Primary central nervous system lymphoma (PCNSL) is a rare tumor accounting for 4% of all brain neoplasms. In immunocompetent patients, it tends to present as a large solitary hemispheric mass with periventricular lesions. PCNSL is a rare malignant tumor, and PCNSLs located in the cerebral ventricles in immunocompetent patients are extremely rare, <10 cases have been described in the literature. Intraventricular meningiomas (IVMs) are rare tumors, accounting for 0.5-5% of all intracranial meningiomas. Malignant IVM (MIVM) is even rarer. To the best of our knowledge, only 10 cases of MIVM are reported till date, and this is only the second case of a child with MIVM reported. ,
| Case Reports|| |
Case report 1
A 12-year-old male child was brought to us by parents with complaints of a headache and vomiting since 1 month. On neurological examination, the child was conscious but drowsy. Fundus examination showed papilledema. Magnetic resonance imaging (MRI) of the brain with contrast was suggestive of well-defined isointense lesion at foramen of Monro on T1-weighted images which were showing bright, homogeneous enhancement on contrast. It was associated with gross hydrocephalus. The patient was operated for right frontal craniotomy, transcallosal approach with excision of lesion with ventriculo-peritoneal shunt. Intraoperatively, it was grayish white, suckable lesion which was moderately vascular. Postoperative course was uneventful; the patient was discharged without any deficit. Histopathological examination revealed tumor cells arranged in lobules and nests, showing abnormal mitosis and extensive apoptosis. Tumor cells were positive for CD 45/Pax5/CD 10, myeloperoxidase negative. Ki-67 index was more than 95%, suggestive of lymphoblastic lymphoma - B-cell. The patient was planned for postoperative chemotherapy [Figure 1].
|Figure 1: Case 1 - (a) Magnetic resonance imaging brain T1-weighted images, axial cuts showing the lesion near foramen of Monro. (b) Computed tomography brain with contrast showing the lesion. (c) Magnetic resonance imaging brain T1-weighted images with contrast showing the lesion. (d) Sagittal images of magnetic resonance imaging the brain. (e) Postoperative magnetic resonance imaging axial cuts. (f) Postoperative magnetic resonance imaging, sagittal cuts|
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Case report 2
A 2-year-old female child presented to us with a history of two episodes of generalized seizures and left side weakness. On neurological examination, she was conscious and oriented. Left-sided hemiparesis was present (MRC 3/5). MRI brain with contrast was suggestive of heterogeneously mass lesion of 10 cm × 8 cm × 8 cm in size in the right frontal region with dilatation of right lateral ventricle. The patient was operated for right frontoparietal craniotomy with gross total excision of lesion. Intraoperatively lesion was intraventricular, arising from wall of the right lateral ventricle and extending into the parenchyma; it was reddish black, firm, suckable, and highly vascular. Complete excision was achieved. Postoperative course was uneventful, power on the left side improved to MRC 4/5. Histopathology examination was suggestive of malignant papillary meningioma. The patient was referred for radiotherapy [Figure 2].
|Figure 2: Case 2 - (a) Computed tomography brain with contrast showing the large lesion, heterogeneous enhancement with dilatation of the right lateral ventricle. (b) Magnetic resonance imaging brain T1-weighted images. Axial cuts. (c) Magnetic resonance imaging brain T2-weighted images, coronal cuts showing the intraventricular lesion extending into parenchyma. (d) T1-weighted contrast images. (e) Postoperative computed tomography brain showing complete excision|
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| Discussion|| |
Intraventricular PCNSLs are rare tumors. PCNSLs are located in the brain hemisphere (38%), thalamus/basal ganglia (16%), corpus callosum (14%), and periventricular regions (12%), with a single tumor in 60-70% of patients and multiple in the remainder. In immunocompetent patients, primary CNS lymphoma tends to present as a solitary intraparenchymal mass. The imaging features of PCNSL are not characteristic, usually showing hypo-or iso-intense lesions on T1-weighted MRI, iso- or hyper-intense lesions on T2-weighted MRI and most lesions show moderate-to-marked contrast enhancement on MR imaging with variable surrounding edema. The imaging characteristics of PCNSL are created by the hypercellularity of the tumor and its high nuclear to cytoplasmic ratio. The lesions appear as hyperdense or isodense on noncontrasted CT. However, the hyperdense appearance on CT can also help distinguish the tumor from metastasis and gliomas which tend to be more hypodense. With contrast enhanced CT, these lesions almost always demonstrate enhancement due to disruption of the blood-brain barrier. 
Lymphoma of the CNS can present with a range of symptoms and imaging findings which can challenge both the radiologist and clinician to come to a diagnosis. Despite characteristic radiologic features of CNS lymphoma, it remains a histological diagnosis and requires either direct brain biopsy or histological confirmation from the cerebrospinal fluid (CSF). It is important to distinguish between primary and secondary forms. Secondary CNS lymphoma is involvement of the CNS from a systemic lymphoma and is more common than PCNSL. PCNSL comprises only 1% of all lymphomas. Histologically, 95% of PCNSL are diffuse large B-cell lymphomas. Most cases of PCNSL in the immunocompetent are diagnosed between 45 and 70 years of age and men and women are equally affected. Differential for intraventricular CNS lesions includes central neurocytoma, meningioma, ependymomas, CPPs, and metastasis. PCNSL is a very chemosensitive and radiosensitive tumor. Most oncologists agree upon a methotrexate chemotherapy regimen in addition to whole brain radiation. ,
IVMs are rare tumors, accounting for <3% of all intracranial meningiomas. They originate from the choroid plexus stroma and the tela choroidea. The vast majority of IVMs are benign, and MIVMs are rare; to the best of our knowledge, only 10 cases have been described so far with single case in pediatric age group (8 years). In MIVM, owing to its susceptibility to spreading through the CSF and the possibility of extraneural metastasis, both neuraxis investigation, and assessment of other systemic organs, particularly the lung and liver, should be carefully performed to detect and treat any metastasis as early as possible. Surgical management of IVMs requires careful planning. Meningiomas are solid, discrete lesions that can be totally excised if plane of cleavage is present. 
| Conclusion|| |
Intraventricular lymphoma and malignant meningioma even though rare should always be kept as differential diagnoses when treating intraventricular tumors. This helps in planning postoperative treatment which is important in both the lesions.
We would like to thank Dean for giving the permission to publish the article.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Bertalanffy H, Krayenbühl N, Wess C, Bozinov O. Ventricular tumors. In: Winn HR, editor. Youman′s Neurological Surgery. 6 th
ed. Philadelphia: Elsevier Saunders; 2011. p. 1534-69.
Funaro K, Bailey KC, Aguila S, Agosti SJ, Vaillancourt C. A case of intraventricular primary central nervous system lymphoma. Radiol Case 2014;8:1-8.
Tao CY, Wang JJ, Li H, You C. Malignant intraventricular meningioma with craniospinal dissemination and concurrent pulmonary metastasis. World J Surg Oncol 2014;12:238.
[Figure 1], [Figure 2]