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Primary Isolated Lymphoma of the Fourth Ventricle in an Immunocompetent Patient

Primary Isolated Lymphoma of the Fourth Ventricle in an Immunocompetent Patient Hindawi Publishing Corporation Case Reports in Oncological Medicine Volume 2013, Article ID 614658, 4 pages http://dx.doi.org/10.1155/2013/614658 Case Report Primary Isolated Lymphoma of the Fourth Ventricle in an Immunocompetent Patient 1 2 3 1 Rakan Bokhari, Ahmad Ghanem, Mahmoud Alahwal, and Saleh Baeesa Division of Neurological Surgery, Faculty of Medicine, King Abdulaziz University, P.O. Box 80215, Jeddah 21589, Saudi Arabia Department of Pathology, Faculty of Medicine, King Abdulaziz University, P.O. Box 80215, Jeddah 21589, Saudi Arabia Department of Medicine, Faculty of Medicine, King Abdulaziz University, P.O. Box 80215, Jeddah 21589, Saudi Arabia Correspondence should be addressed to Saleh Baeesa; sbaeesa@kau.edu.sa Received 16 February 2013; Accepted 7 March 2013 Academic Editors: A. Goodman, R. Martinez, and K. Tanaka Copyright © 2013 Rakan Bokhari et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Primary central nervous lymphoma (PCNSL) is a rare variant of extranodal non-Hodgkin’s lymphoma with a especially poor prognosis. The diagnosis is usually encountered in immunodefici ent patients but is also encountered, albeit uncommonly, in the immunocompetent. We present a 50-year-old male who developed signs and symptoms of increased intracranial pressure. Imaging revealed the presence of a fourth ventricle mass with obstructive hydrocephalus. First, the patient underwent emergency endoscopic third ventriculostomy followed, few days later, by complete tumor resection via a posterior fossa craniotomy. Postoperative histopathology revealed the lesion to be a PCNSL. He received adjuvant chemotherapy and radiation and remained with no recurrence on regular imaging studies for 18-month followup. We report herein the fourth case of isolated PCNSL lesion to the fourth ventricle in the literature and provide the rationale for our belief that craniotomy and tumor resection, if feasible, should be the initial line of management in similar cases to relieve hydrocephalus and achieve the diagnosis. 1. Introduction unrelenting vomiting associated with moderate nausea that had progressed over the past 2 weeks. Initially attributed to Primary central nervous system lymphoma (PCNSL) is a rare gastritis, his symptoms did not improve with proton pump variant of extranodal non-Hodgkin’s lymphoma (NHL) with inhibitor and antiemetic medications. an uncertain origin and pathogenesis [1, 2]. It accounts for His level of consciousness had started to drop over the about 1% of all primary brain tumors and is most commonly preceding 24 hours with significant increase in the severity adiffuselarge B-cell lymphoma [ 1, 2]. PCNSL is usually of his headache. Physical exam showed a depressed Glasgow associated with immunodeficiency (most commonly HIV) coma score (GCS) of 12 equally reactive pupils with no focal butisincreasinglyobservedinimmunocompetent patients neurological deficit. [2]. We present what is, to the authors’ best knowledge, the An urgent brain computed tomography (CT) scan fourth case of PCNSLisolatedtothe fourth ventricleand revealed obstructive hydrocephalus with an infratentorial the eleventh case of solely intraventricular PCNSL [3–5]. We somewhat hyperdense lesion in the fourth ventricular that attempt to raise awareness of this rare entity and describe enhanced homogeneously (Figure 1). our experience with this rare entity. Unlike the consensus The patient was admitted and urgent endoscopic third towards PCNSL in general, we believe that surgical resection, ventriculostomy was performed, with rapid recovery of his if feasible, in similar cases presenting with hydrocephalus is level of consciousness postoperatively. Cerebrospinal u fl id the optimal line of management [6, 7]. (CSF) analysis and cytology results were within normal and negative for malignant cells. 2. Case Report Further brain and spine imaging with magnetic reso- nance (MR) scan demonstrated a 20× 25× 30 mm strongly A 50-year-old male physician, with no medical comorbidities, presented to the emergency room with history of forceful enhancing mass occupying the inferior half of the fourth 2 Case Reports in Oncological Medicine Figure 1: BrainCTscandemonstrating hyperdense tumor(arrow) filling the 4th ventricle. Figure 3: Axial T1-WI demonstrating 20×25×30 mm intensely enhancing fourth ventricular tumor with obstructive hydro- cephalus. Figure 4: Hematoxylin and Eosin stain showing neoplastic growth formed of highly pleomorphic cells with hyperchromasia, irregular nuclear membranes, vesicular nucleus, and prominent nucleoli. Figure 2: Sagittal T1-WI demonstrating 20×25×30 mm intensely Aggregations of mature lymphocytes also seen, with areas of enhancing fourth ventricular tumor with obstructive hydro- hemorrhagic necrosis apoptosis and calcification seen. cephalus. ventricle (Figures 2 and 3). The mass acquired the shape of was proceeded using ultrasonic aspiration and was attainable the ventricular cavity, extended towards the lateral foramina utilizing an easily identifiable tumor-brainstem interface. of Luschka and inferiorly towards the obex without evidence Histopathology would show the cells positive for CD45 of fourth ventricular dilation. No evidence of parenchymal (Leukocyte Common Antigen) as well as for CD79a, CD20 invasion or drop metastases was seen on imaging of the (B-cell antigens), and HLA-DR and focally positive for CD10 wholespine.Theradiologist’s impression wasthatofan and vimentin. The morphology and immunohistochemical ependymoma or possibly a subependymoma. profile was consistent with a high-grade B-cell lymphoma The patient was then shifted, 2 days later, to the operating (Figures 4 and 5). theatrefor resectionofatumorofthe fourth ventricle. Consequent to receiving the histopathology results, stag- Midline posterior fossa craniotomy was performed and the ingworkupconsistingofneck,chest,abdomen,andpelvisCT tumor was accessed through a transvermian approach. eTh studies would show no disease elsewhere and HIV tests were tumorwas exophytic, grayishincolor,firm,and fairly negative. vascular, being fed by leptomeningeal vessels arising from The patient has a smooth postoperative period and his top of the brainstem. Invasion of the lower pons and upper vomiting and gait imbalance had markedly improved. medulla oblongata in addition to the superior vermis was He received a 6 cycles of intravenous and intrathecal noted intraoperatively. methotrexate with subsequent whole brain radiotherapy and Initially, biopsy was taken and frozen section was sugges- boost to the surgical bed of total of 30 Gys. Both immediate tive of malignant neoplasm with differential diagnosis includ- and 18-month postoperative brain and whole spine MR ing medulloblastoma and ependymoma. Complete resection imaging scans showed no recurrence (Figures 6 and 7). Case Reports in Oncological Medicine 3 Figure 5: Immunohistochemical stains were positive for CD45 (Leukocyte Common Antigen). Figure 7: Enhanced axial T1-WI demonstrating no residual tumor. of spectroscopy and diffusion-weighted imaging, in the differentiation of these lesions from other tumors is limited owing to the similarity in profile with the highly cellular and actively proliferating glioblastomas and metastases [8, 9]. PCNSL shows restricted diffusion and MR spectroscopy shows elevated lipids with high choline/creatinine ratios [9]. Figure 6: Enhanced sagittal T1-WI demonstrating no residual These n fi dings are consistent with other neoplastic lesions tumor. butmay be of valueindistinguishingitfrominfective or demyelinating processes [9]. Our case is unusual in that the lesion is isolated to the fourth ventricle, an extremely rare location that has only been 3. Discussion documented in three other cases in literature [3–5]withan Central nervous system (CNS) lymphomas are considered additional case having an associated lateral ventricle focus by neurosurgeons as nonsurgical tumors due to their diffuse [10]. A solitary enhancing mass in an immunocompetent infiltration and exquisite sensitivity to chemoradiation [ 2]. patientthatforms acastofthe fourth ventricleshouldraise In fact, attempted resection or decompression has been the suspicion of an ependymoma or, less likely, a subependy- showntobeofnobenetfi , andpossiblyharmful [ 6, 7]. The moma or medulloblastoma. The diagnosis of lymphoma in preoperative identification of these tumors is therefore of our case was indeed unforeseen and serves to reinforce the great relevance, as their suspicion changes the goal of surgery recommendation of previous authors to include it in the from a potentially morbid resection to a minimally invasive differential diagnosis of an intraventricular lesion in the biopsy fortissuediagnosis andinitiationofappropriate appropriate setting, even it is if solitary [4, 5]. therapy. Survival outcomes mentioned for PCNSL have been The literature mentions a constellation of radiologic uniformly disappointing despite recent advances and the signsthatshouldalert thetreatingsurgeon preoperatively to oen ft initial dramatic response to therapy [ 2, 11]. Current suspect CNS lymphoma [8]. These signs are dependent on guidelines state that combination chemoradiation offers the the patient’s immune status, previous treatment administra- best outcome, with median overall survival extended to 2–4 tion, and whether the lymphoma arose primarily from the years and 5-year survival rate of 20–40% [2]. These compare CNS or had disseminated there secondarily [8]. The classic favorably to those of radiation alone, which oer ff ed median appearance of PCNSL in an untreated immunocompetent survival of less than 18 months and a 5-year survival rate patientisthatofmultiplesupratentoriallesions that areclose of less than 5%. Chemotherapy usually consists of high- to the brain CSF interface; which may be either superficial dose methotrexate-based regimen while radiation consists of (subpial) or deepseated (subependymal). They appear iso- whole brain radiation therapy with or without local boost to to hypointense on T1-weighted images and hyperintense on the tumor bed [2]. T2-weighted images. Contrast uptake is usually avid and eTh previously reported similar 3 cases are summa- homogenous. eTh value of advanced MR imaging, consisting rized in Table 1. eTh unusual location of the tumor in the 4 Case Reports in Oncological Medicine Table 1: Summary of the reported cases of 4th ventricular PCNSL in the literature. Author Age Surgical CSF Presentation Radiology Adjuvant therapy Survival (reference) (yrs)/sex treatment cytology Negative radiology but Meningitis, Werneck et al. aer ft mortem small tumor Diagnosis aeft r 17/F diagnosis aeft r N/A Positive N/A [3] was found with mortem mortem carcinomatous meningitis Cytarabine and Headache, Haegelen et al. Tumor filling the 4th steroids with stem 33/F vomiting with GTR Negative 7months [4] ventricle with no HCP cell transplantation cerebellar signs with WBRT Homogenously enhancing Headache, tumor in the caudal fourth Hill et al. [5]69/F Biopsy N/A IV and IT MTX 3 months vomiting ventricle without hydrocephalus Homogenously enhancing Regimen Headache, tumor in the caudal fourth containing IV and Current case 50/M vomiting with GTR Negative 18 months ventricle with IT MTX and drowsiness hydrocephalus radiotherapy GTR: gross total resection; IV: intravenous; IT: intrathecal; MTX: methotrexate; N/A: not available. surgically accessible posterior fossa, in addition to its iso- [3] L. C. Werneck, Z. Hatschbach, A. H. Mora, and E. M. Novarak, “Meningite por linfoma primitivo do sistema nervoso central. lation and subsequent hydrocephalus, may present a com- Relato de um case,” Arq Neuropsiquiatr,vol.35, no.4,pp. 366– pelling indication for craniotomy and lymphoma resection 372, 1977 (French). even with a known diagnosis. This procedure is of limited [4] C. Haegelen, L. Riffaud, M. Bernard, and X. Morandi, “Primary penaltywhencomparedtoasupratentorial craniotomy for isolated lymphoma of the fourth ventricle: case report,” Journal access of typical deep peri- or intraventricular lesions that of Neuro-Oncology,vol.51, no.2,pp. 129–131, 2001. account for the current consensus. Given the responsiveness [5] C.S.Hill,A.F.Khan, S. Bloom, S. McCartney, andD.Choi, of these tumors to chemoradiation, such surgery oeff rs “A rare case of vomiting: fourth ventricular B-cell lymphoma,” the possibility of rendering the patient shunt independent Journal of Neuro-Oncology, vol. 93, no. 2, pp. 261–262, 2009. even with subtotal resection. eTh outcome is made more [6] B. Bataille, V. Delwail, E. Menet et al., “Primary intracerebral desirable with the recent report of systemic (cutaneous) malignant lymphoma: report of 248 cases,” Journal of Neuro- PCNSL dissemination along the subcutaneous tract of a surgery, vol. 92, no. 2, pp. 261–266, 2000. ventriculoperitoneal shunt and the dicul ffi ty in accessing the [7] T.Batchelor andJ.S.Loeffler, “Primary CNSlymphoma,” posterior fossa during an endoscopic third ventriculostomy Journal of Clinical Oncology, vol. 24, no. 8, pp. 1281–1288, 2006. [12]. [8] K.K.Yap,T.Sutherland, E. Liew,C.J.Tartaglia,M.Pang, andN. Trost, “Magnetic resonance features of primary central nervous system lymphoma in the immunocompetent patient: a pictorial 4. Conclusion essay,” Journal of Medical Imaging and Radiation Oncology,vol. We emphasize that PCNSL should be included in the dif- 6, no. 2, pp. 179–186, 2012. ferential diagnosis of intraventricular tumors, considering its [9] T. T. Zacharia, M. Law, T. P. Naidich, and N. E. Leeds, “Cen- implications on management planning. We also believe that tral nervous system lymphoma characterization by diffusion- weighted imaging and MR spectroscopy,” Journal of Neuroimag- resection is worthwhile, not contraindicated, in the context ing,vol.18, no.4,pp. 411–417, 2008. of a fourth ventricle lesion causing hydrocephalus. This will [10] R. Brar, A. Prasad, T. Sharma, and N. Vermani, “Multifocal serve to spare the patient the morbidity of shunt dependency. lateral and fourth ventricular B-cell primary CNS lymphoma,” Clinical Neurology and Neurosurgery,vol.114,no. 3, pp.281–283, Conflict of Interests [11] M. Reni and A. J. M. Ferreri, “Therapeutic management of eTh authors declare that they have no competing interests. refractory or relapsed primary central nervous system lym- phomas,” Annals of Hematology,vol.80,supplement3,pp.B113– B117, 2001. References [12] J. H. Ko,P.H.Lu, T. C. Tang,and Y. H. Hsu, “Metastasisof [1] T. Batchelor and J. S. Loeffler, “Primary CNS lymphoma,” primary CNS lymphoma along a ventriculoperitoneal shunt,” Journal of Clinical Oncology,vol.24, no.8,pp. 1281–1288, 2006. Journal of Clinical Oncology,vol.29, no.34, pp.823–824,2011. [2] M.S.del Rio, A. Rousseau,C.Soussain, D. Ricard,and K. Hoang-Xuan, “Primary CNS lymphoma in immunocompetent patients,” Oncologist,vol.14, no.5,pp. 526–539, 2009. 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Primary Isolated Lymphoma of the Fourth Ventricle in an Immunocompetent Patient

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Copyright © 2013 Rakan Bokhari et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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Hindawi Publishing Corporation Case Reports in Oncological Medicine Volume 2013, Article ID 614658, 4 pages http://dx.doi.org/10.1155/2013/614658 Case Report Primary Isolated Lymphoma of the Fourth Ventricle in an Immunocompetent Patient 1 2 3 1 Rakan Bokhari, Ahmad Ghanem, Mahmoud Alahwal, and Saleh Baeesa Division of Neurological Surgery, Faculty of Medicine, King Abdulaziz University, P.O. Box 80215, Jeddah 21589, Saudi Arabia Department of Pathology, Faculty of Medicine, King Abdulaziz University, P.O. Box 80215, Jeddah 21589, Saudi Arabia Department of Medicine, Faculty of Medicine, King Abdulaziz University, P.O. Box 80215, Jeddah 21589, Saudi Arabia Correspondence should be addressed to Saleh Baeesa; sbaeesa@kau.edu.sa Received 16 February 2013; Accepted 7 March 2013 Academic Editors: A. Goodman, R. Martinez, and K. Tanaka Copyright © 2013 Rakan Bokhari et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Primary central nervous lymphoma (PCNSL) is a rare variant of extranodal non-Hodgkin’s lymphoma with a especially poor prognosis. The diagnosis is usually encountered in immunodefici ent patients but is also encountered, albeit uncommonly, in the immunocompetent. We present a 50-year-old male who developed signs and symptoms of increased intracranial pressure. Imaging revealed the presence of a fourth ventricle mass with obstructive hydrocephalus. First, the patient underwent emergency endoscopic third ventriculostomy followed, few days later, by complete tumor resection via a posterior fossa craniotomy. Postoperative histopathology revealed the lesion to be a PCNSL. He received adjuvant chemotherapy and radiation and remained with no recurrence on regular imaging studies for 18-month followup. We report herein the fourth case of isolated PCNSL lesion to the fourth ventricle in the literature and provide the rationale for our belief that craniotomy and tumor resection, if feasible, should be the initial line of management in similar cases to relieve hydrocephalus and achieve the diagnosis. 1. Introduction unrelenting vomiting associated with moderate nausea that had progressed over the past 2 weeks. Initially attributed to Primary central nervous system lymphoma (PCNSL) is a rare gastritis, his symptoms did not improve with proton pump variant of extranodal non-Hodgkin’s lymphoma (NHL) with inhibitor and antiemetic medications. an uncertain origin and pathogenesis [1, 2]. It accounts for His level of consciousness had started to drop over the about 1% of all primary brain tumors and is most commonly preceding 24 hours with significant increase in the severity adiffuselarge B-cell lymphoma [ 1, 2]. PCNSL is usually of his headache. Physical exam showed a depressed Glasgow associated with immunodeficiency (most commonly HIV) coma score (GCS) of 12 equally reactive pupils with no focal butisincreasinglyobservedinimmunocompetent patients neurological deficit. [2]. We present what is, to the authors’ best knowledge, the An urgent brain computed tomography (CT) scan fourth case of PCNSLisolatedtothe fourth ventricleand revealed obstructive hydrocephalus with an infratentorial the eleventh case of solely intraventricular PCNSL [3–5]. We somewhat hyperdense lesion in the fourth ventricular that attempt to raise awareness of this rare entity and describe enhanced homogeneously (Figure 1). our experience with this rare entity. Unlike the consensus The patient was admitted and urgent endoscopic third towards PCNSL in general, we believe that surgical resection, ventriculostomy was performed, with rapid recovery of his if feasible, in similar cases presenting with hydrocephalus is level of consciousness postoperatively. Cerebrospinal u fl id the optimal line of management [6, 7]. (CSF) analysis and cytology results were within normal and negative for malignant cells. 2. Case Report Further brain and spine imaging with magnetic reso- nance (MR) scan demonstrated a 20× 25× 30 mm strongly A 50-year-old male physician, with no medical comorbidities, presented to the emergency room with history of forceful enhancing mass occupying the inferior half of the fourth 2 Case Reports in Oncological Medicine Figure 1: BrainCTscandemonstrating hyperdense tumor(arrow) filling the 4th ventricle. Figure 3: Axial T1-WI demonstrating 20×25×30 mm intensely enhancing fourth ventricular tumor with obstructive hydro- cephalus. Figure 4: Hematoxylin and Eosin stain showing neoplastic growth formed of highly pleomorphic cells with hyperchromasia, irregular nuclear membranes, vesicular nucleus, and prominent nucleoli. Figure 2: Sagittal T1-WI demonstrating 20×25×30 mm intensely Aggregations of mature lymphocytes also seen, with areas of enhancing fourth ventricular tumor with obstructive hydro- hemorrhagic necrosis apoptosis and calcification seen. cephalus. ventricle (Figures 2 and 3). The mass acquired the shape of was proceeded using ultrasonic aspiration and was attainable the ventricular cavity, extended towards the lateral foramina utilizing an easily identifiable tumor-brainstem interface. of Luschka and inferiorly towards the obex without evidence Histopathology would show the cells positive for CD45 of fourth ventricular dilation. No evidence of parenchymal (Leukocyte Common Antigen) as well as for CD79a, CD20 invasion or drop metastases was seen on imaging of the (B-cell antigens), and HLA-DR and focally positive for CD10 wholespine.Theradiologist’s impression wasthatofan and vimentin. The morphology and immunohistochemical ependymoma or possibly a subependymoma. profile was consistent with a high-grade B-cell lymphoma The patient was then shifted, 2 days later, to the operating (Figures 4 and 5). theatrefor resectionofatumorofthe fourth ventricle. Consequent to receiving the histopathology results, stag- Midline posterior fossa craniotomy was performed and the ingworkupconsistingofneck,chest,abdomen,andpelvisCT tumor was accessed through a transvermian approach. eTh studies would show no disease elsewhere and HIV tests were tumorwas exophytic, grayishincolor,firm,and fairly negative. vascular, being fed by leptomeningeal vessels arising from The patient has a smooth postoperative period and his top of the brainstem. Invasion of the lower pons and upper vomiting and gait imbalance had markedly improved. medulla oblongata in addition to the superior vermis was He received a 6 cycles of intravenous and intrathecal noted intraoperatively. methotrexate with subsequent whole brain radiotherapy and Initially, biopsy was taken and frozen section was sugges- boost to the surgical bed of total of 30 Gys. Both immediate tive of malignant neoplasm with differential diagnosis includ- and 18-month postoperative brain and whole spine MR ing medulloblastoma and ependymoma. Complete resection imaging scans showed no recurrence (Figures 6 and 7). Case Reports in Oncological Medicine 3 Figure 5: Immunohistochemical stains were positive for CD45 (Leukocyte Common Antigen). Figure 7: Enhanced axial T1-WI demonstrating no residual tumor. of spectroscopy and diffusion-weighted imaging, in the differentiation of these lesions from other tumors is limited owing to the similarity in profile with the highly cellular and actively proliferating glioblastomas and metastases [8, 9]. PCNSL shows restricted diffusion and MR spectroscopy shows elevated lipids with high choline/creatinine ratios [9]. Figure 6: Enhanced sagittal T1-WI demonstrating no residual These n fi dings are consistent with other neoplastic lesions tumor. butmay be of valueindistinguishingitfrominfective or demyelinating processes [9]. Our case is unusual in that the lesion is isolated to the fourth ventricle, an extremely rare location that has only been 3. Discussion documented in three other cases in literature [3–5]withan Central nervous system (CNS) lymphomas are considered additional case having an associated lateral ventricle focus by neurosurgeons as nonsurgical tumors due to their diffuse [10]. A solitary enhancing mass in an immunocompetent infiltration and exquisite sensitivity to chemoradiation [ 2]. patientthatforms acastofthe fourth ventricleshouldraise In fact, attempted resection or decompression has been the suspicion of an ependymoma or, less likely, a subependy- showntobeofnobenetfi , andpossiblyharmful [ 6, 7]. The moma or medulloblastoma. The diagnosis of lymphoma in preoperative identification of these tumors is therefore of our case was indeed unforeseen and serves to reinforce the great relevance, as their suspicion changes the goal of surgery recommendation of previous authors to include it in the from a potentially morbid resection to a minimally invasive differential diagnosis of an intraventricular lesion in the biopsy fortissuediagnosis andinitiationofappropriate appropriate setting, even it is if solitary [4, 5]. therapy. Survival outcomes mentioned for PCNSL have been The literature mentions a constellation of radiologic uniformly disappointing despite recent advances and the signsthatshouldalert thetreatingsurgeon preoperatively to oen ft initial dramatic response to therapy [ 2, 11]. Current suspect CNS lymphoma [8]. These signs are dependent on guidelines state that combination chemoradiation offers the the patient’s immune status, previous treatment administra- best outcome, with median overall survival extended to 2–4 tion, and whether the lymphoma arose primarily from the years and 5-year survival rate of 20–40% [2]. These compare CNS or had disseminated there secondarily [8]. The classic favorably to those of radiation alone, which oer ff ed median appearance of PCNSL in an untreated immunocompetent survival of less than 18 months and a 5-year survival rate patientisthatofmultiplesupratentoriallesions that areclose of less than 5%. Chemotherapy usually consists of high- to the brain CSF interface; which may be either superficial dose methotrexate-based regimen while radiation consists of (subpial) or deepseated (subependymal). They appear iso- whole brain radiation therapy with or without local boost to to hypointense on T1-weighted images and hyperintense on the tumor bed [2]. T2-weighted images. Contrast uptake is usually avid and eTh previously reported similar 3 cases are summa- homogenous. eTh value of advanced MR imaging, consisting rized in Table 1. eTh unusual location of the tumor in the 4 Case Reports in Oncological Medicine Table 1: Summary of the reported cases of 4th ventricular PCNSL in the literature. Author Age Surgical CSF Presentation Radiology Adjuvant therapy Survival (reference) (yrs)/sex treatment cytology Negative radiology but Meningitis, Werneck et al. aer ft mortem small tumor Diagnosis aeft r 17/F diagnosis aeft r N/A Positive N/A [3] was found with mortem mortem carcinomatous meningitis Cytarabine and Headache, Haegelen et al. Tumor filling the 4th steroids with stem 33/F vomiting with GTR Negative 7months [4] ventricle with no HCP cell transplantation cerebellar signs with WBRT Homogenously enhancing Headache, tumor in the caudal fourth Hill et al. [5]69/F Biopsy N/A IV and IT MTX 3 months vomiting ventricle without hydrocephalus Homogenously enhancing Regimen Headache, tumor in the caudal fourth containing IV and Current case 50/M vomiting with GTR Negative 18 months ventricle with IT MTX and drowsiness hydrocephalus radiotherapy GTR: gross total resection; IV: intravenous; IT: intrathecal; MTX: methotrexate; N/A: not available. surgically accessible posterior fossa, in addition to its iso- [3] L. C. Werneck, Z. Hatschbach, A. H. Mora, and E. M. Novarak, “Meningite por linfoma primitivo do sistema nervoso central. lation and subsequent hydrocephalus, may present a com- Relato de um case,” Arq Neuropsiquiatr,vol.35, no.4,pp. 366– pelling indication for craniotomy and lymphoma resection 372, 1977 (French). even with a known diagnosis. This procedure is of limited [4] C. Haegelen, L. Riffaud, M. Bernard, and X. Morandi, “Primary penaltywhencomparedtoasupratentorial craniotomy for isolated lymphoma of the fourth ventricle: case report,” Journal access of typical deep peri- or intraventricular lesions that of Neuro-Oncology,vol.51, no.2,pp. 129–131, 2001. account for the current consensus. Given the responsiveness [5] C.S.Hill,A.F.Khan, S. Bloom, S. McCartney, andD.Choi, of these tumors to chemoradiation, such surgery oeff rs “A rare case of vomiting: fourth ventricular B-cell lymphoma,” the possibility of rendering the patient shunt independent Journal of Neuro-Oncology, vol. 93, no. 2, pp. 261–262, 2009. even with subtotal resection. eTh outcome is made more [6] B. Bataille, V. Delwail, E. Menet et al., “Primary intracerebral desirable with the recent report of systemic (cutaneous) malignant lymphoma: report of 248 cases,” Journal of Neuro- PCNSL dissemination along the subcutaneous tract of a surgery, vol. 92, no. 2, pp. 261–266, 2000. ventriculoperitoneal shunt and the dicul ffi ty in accessing the [7] T.Batchelor andJ.S.Loeffler, “Primary CNSlymphoma,” posterior fossa during an endoscopic third ventriculostomy Journal of Clinical Oncology, vol. 24, no. 8, pp. 1281–1288, 2006. [12]. [8] K.K.Yap,T.Sutherland, E. Liew,C.J.Tartaglia,M.Pang, andN. Trost, “Magnetic resonance features of primary central nervous system lymphoma in the immunocompetent patient: a pictorial 4. Conclusion essay,” Journal of Medical Imaging and Radiation Oncology,vol. We emphasize that PCNSL should be included in the dif- 6, no. 2, pp. 179–186, 2012. ferential diagnosis of intraventricular tumors, considering its [9] T. T. Zacharia, M. Law, T. P. Naidich, and N. E. Leeds, “Cen- implications on management planning. We also believe that tral nervous system lymphoma characterization by diffusion- weighted imaging and MR spectroscopy,” Journal of Neuroimag- resection is worthwhile, not contraindicated, in the context ing,vol.18, no.4,pp. 411–417, 2008. of a fourth ventricle lesion causing hydrocephalus. This will [10] R. Brar, A. Prasad, T. Sharma, and N. Vermani, “Multifocal serve to spare the patient the morbidity of shunt dependency. lateral and fourth ventricular B-cell primary CNS lymphoma,” Clinical Neurology and Neurosurgery,vol.114,no. 3, pp.281–283, Conflict of Interests [11] M. Reni and A. J. M. Ferreri, “Therapeutic management of eTh authors declare that they have no competing interests. refractory or relapsed primary central nervous system lym- phomas,” Annals of Hematology,vol.80,supplement3,pp.B113– B117, 2001. References [12] J. H. Ko,P.H.Lu, T. C. Tang,and Y. H. Hsu, “Metastasisof [1] T. Batchelor and J. S. Loeffler, “Primary CNS lymphoma,” primary CNS lymphoma along a ventriculoperitoneal shunt,” Journal of Clinical Oncology,vol.24, no.8,pp. 1281–1288, 2006. Journal of Clinical Oncology,vol.29, no.34, pp.823–824,2011. [2] M.S.del Rio, A. Rousseau,C.Soussain, D. Ricard,and K. Hoang-Xuan, “Primary CNS lymphoma in immunocompetent patients,” Oncologist,vol.14, no.5,pp. 526–539, 2009. 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