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  Table of Contents  
Year : 2014  |  Volume : 51  |  Issue : 4  |  Page : 468-469

Isolated central nervous system relapse in a patient with systemic diffuse large B-cell lymphoma: Diagnostic and treatment challenges

1 Department of Radiation Oncology, Regional Cancer Center, JIPMER, Pondicherry, India
2 Department of Medical Oncology, Regional Cancer Center, JIPMER, Pondicherry, India

Date of Web Publication1-Feb-2016

Correspondence Address:
Dr. P Das
Department of Radiation Oncology, Regional Cancer Center, JIPMER, Pondicherry
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-509X.175319

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How to cite this article:
Das P, Dubashi B, Joseph D, Jain A. Isolated central nervous system relapse in a patient with systemic diffuse large B-cell lymphoma: Diagnostic and treatment challenges. Indian J Cancer 2014;51:468-9

How to cite this URL:
Das P, Dubashi B, Joseph D, Jain A. Isolated central nervous system relapse in a patient with systemic diffuse large B-cell lymphoma: Diagnostic and treatment challenges. Indian J Cancer [serial online] 2014 [cited 2021 Aug 5];51:468-9. Available from: https://www.indianjcancer.com/text.asp?2014/51/4/468/175319


A 59-year-old female with no associated co-morbidities was diagnosed as non-Hodgkin's lymphoma; Diffuse large B cell type, Ann Arbor Stage IIIA. She was treated with four cycles of chemotherapy (R-CHOP) and developed a fall in ejection fraction. Further doses of anthracycline were withheld (R-COP) and she completed six cycles of chemotherapy. Post treatment evaluation with PET scan revealed complete remission. After a disease free interval of six months she developed multiple episodes of seizures. MRI revealed hyper-intense mass lesion of 4 × 3 cm in the left frontal lobe [Figure 1]. She underwent craniotomy and resection of the lesion. Histopathology was consistent with small round cell tumour. Immunohistochemistry revealed LCA, CD20 and CD10 positive and was negative for GFAP, Pan CK and CD3 with Ki-67 80% positive suggestive of diffuse large B-cell lymphoma. Staging work up with PET scan including CSF study was negative. Viral serology for HIV, Hepatitis B and Hepatitis C were negative. A final diagnosis of isolated CNS relapse in a patient with systemic NHL was made. She received four cycles of systemic hyper CVAD protocol of chemotherapy with omission of anthracycline followed by whole brain radiation therapy (WBRT). Tolerance to chemotherapy was good. She developed grade-IV febrile neutropenia requiring antibiotics and growth factors. She is currently in complete remission [Figure 1].
Figure 1: Gadolinium contrast enhanced T1W MRI on coronal view showing enhancing mass lesion in left frontal region with mass effect, compression of left lateral ventricle and minimal midline shift to right side is visualised

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Central nervous system (CNS) relapse usually occur during progression or after recurrence of systemic lymphoma. They often present as an expression of end-stage disease refractory to most therapeutic strategies. In contrast, isolated CNS relapse with no evidence of lymphoma outside the CNS at the time of relapse occurs in approximately 1% of patients with systemic non-Hodgkin's lymphoma. Isolated CNS disease as the initial relapse site after a complete response (CR) to systemic treatment may be potentially treatable.[1],[2] The simultaneous development of systemic and CNS disease as seen in most patients has important therapeutic implications. Symptoms and signs of CNS involvement are caused by either leptomeningeal and/or parenchymal involvement and in recent years, since the routine use of MRI, parenchymal involvement is recognized more frequently.[3],[4]

Conventional therapy for overt meningeal lymphoma consists of intrathecal or intraventricular chemotherapy often combined with WBRT. The survival data of patients with leptomeningeal lymphoma is dismal despite the high rate of initial response and ranges between two and six months with a one-year survival of 12-23%.[4] The objective of this report is to demonstrate the rarity of presentation and the role of hyper CVAD protocol chemotherapy with whole brain radiotherapy in the treatment of isolated CNS relapse.

  References Top

Doolittle ND, Abrey LE, Shenkier TN, Tali S, Bromberg JE, Edward A, et al. Brain parenchyma involvement as isolated central nervous system relapse of systemic non-Hodgkin lymphoma: An International Primary CNS Lymphoma Collaborative Group report. Blood 2008;111:1085-93.  Back to cited text no. 1
Feugier P, Virion JM, Tilly H. Incidence and risk factors for central nervous system occurrence in elderly patients with diffuse large B-cell lymphoma: Influence of rituximab. Ann Oncol 2004;15:129-33.  Back to cited text no. 2
Bokstein F, Lossos A, Izidore S, Lossos IS, Siegal T. Central nervous system relapse of systemic non-Hodgkin's lymphoma: Results of treatment based on high-dose methotrexate combination chemotherapy. Leuk Lymphoma 2002;43:587-93.  Back to cited text no. 3
van Besien K, Ha CS, Murphy S, McLaughlin P, Rodriguez A, Amin K, et al. Risk factors, treatment, and outcome of central nervous system recurrence in adults with intermediate-grade and immunoblastic lymphoma. Blood 1998;91:1178-84.  Back to cited text no. 4


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