Lakshmaiah, Kuntegowdanahalli, Viveka, Belathur, Anil Kumar, N., Saini, Monika Lamba, Sinha, Sanjib, & Saini, Kamal
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Lakshmaiah, Kuntegowdanahalli, Viveka, Belathur, Anil Kumar, N., Saini, Monika Lamba, Sinha, Sanjib, & Saini, Kamal Gastric diffuse large B cell lymphoma presenting as paraneoplastic cerebellar degeneration: Case report and review of literature Article. Version publiée - Published version. © ELSEVIER Citation APA: Lakshmaiah, K., Viveka, B., Anil Kumar, N., Saini, M. L., Sinha, S., & Saini, K. (2013, December). Gastric diffuse large B cell lymphoma presenting as paraneoplastic cerebellar degeneration: Case report and review of literature. Journal of Egyptian National Cancer Institute, 25(4), 231-235. doi:10.1016/j.jnci.2013.07.001 DOI: 10.1016/j.jnci.2013.07.001 Also available at: http://hdl.handle.net/2013/ULB-DIPOT:oai:dipot.ulb.ac.be:2013/168178 Cet article publié par ELSEVIER provient du Dépôt institutionnel de l’Université Libre de Bruxelles, DI- fusion http://difusion.ulb.ac.be. 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Systematic downloading of articles published by ELSEVIER that are available in DI-fusion is not permitted. Journal of the Egyptian National Cancer Institute (2013) 25, 231–235 Cairo University Journal of the Egyptian National Cancer Institute www.nci.cu.adu.eg www.sciencedirect.com Case Report Gastric diffuse large B cell lymphoma presenting as paraneoplastic cerebellar degeneration: Case report and review of literatureq K.C. Lakshmaiah a, B.K. Viveka a,*, N. Anil Kumar a, Monika L. Saini b, Sanjib Sinha c, Kamal S. Saini d a Department of Medical Oncology, Kidwai Memorial Institute of Oncology, India b Department of Anatomie Pathologique, Universite´ Catholique de Louvain, Brussels, Belgium c Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), India d Breast International Group, Department of Medical Oncology, Jules Bordet Institute, Universite´ Libre de Bruxelles, Brussels, Belgium Received 22 April 2013; accepted 1 July 2013 Available online 6 August 2013 KEYWORDS Abstract Paraneoplastic cerebellar degeneration (PCD) is a type of paraneoplastic neurological disorder (PND) that is associated with many solid tumors, Hodgkin’s lymphoma (HL) and very Gastric lymphoma; rarely with non-Hodgkin’s lymphoma (NHL). We report a case of PCD associated with gastric dif- Paraneoplastic cerebellar fuse large B-cell lymphoma (DLBCL) in a patient who presented with acute onset of giddiness and degeneration; Paraneoplastic neurological double vision and had complete remission of the gastric lesion and marked improvement of cere- disorders; bellar syndrome with rituximab-based combination chemotherapy. A brief review of the literature Rituximab; is also presented. Auto-immune antibodies; ª 2013 Production and hosting by Elsevier B.V. on behalf of National Cancer Institute, Cairo University. Diffuse large B-cell lymphoma * Corresponding author. Address: Department of Medical Oncology, Introduction Kidwai Memorial Institute of Oncology, Dr. M.H. Marigowda Road, Bangalore 560 029, Karnataka, India. Tel.: +91 080 26094000; fax: +91 0806560723. Paraneoplastic cerebellar degeneration (PCD) and other forms E-mail address: [email protected] (B.K. Viveka). of paraneoplastic neurological disorders (PNDs) have been de- q This particular case is unique for its rarity and presentation. To the scribed in association with many solid tumors (like small-cell best of our knowledge, this is the first case report of gastric DLBCL lung cancer and adenocarcinoma of the breast and ovary), associated with paraneoplastic cerebellar degeneration. We hope that Hodgkin’s lymphoma (HL) and very rarely with non- this report would help oncologists recognize and treat such rare cases. Hodgkin’s lymphoma (NHL) [1]. Symptoms of neurological Peer review under responsibility of The National Cancer Institute, disturbance may precede those of the underlying malignancy. Cairo University. We report a patient who presented with rapidly progressive pan-cerebellar syndrome with encephalopathy and was subsequently diagnosed to have gastric diffuse large B-cell Production and hosting by Elsevier lymphoma (DLBCL). It is a clinical challenge to diagnose such 1110-0362 ª 2013 Production and hosting by Elsevier B.V. on behalf of National Cancer Institute, Cairo University. http://dx.doi.org/10.1016/j.jnci.2013.07.001 232 K.C. Lakshmaiah et al. a rare presentation of a neoplastic condition. There are no spe- and serum sample was negative for human immunodeficiency cific therapies for PCD described [2]. virus (HIV) antibodies and Venereal Disease Research Labo- ratory (VDRL) test. Magnetic resonance imaging of the brain Case history including diffusion weighted imaging (DWI) and apparent dif- fusion coefficient (ADC) and electroencephalogram (EEG) A 68-year-old male presented with a two month history of pro- were unremarkable. Routine analysis of the cerebrospinal fluid gressive unsteadiness while walking, tendency to fall to either (CSF) showed 10 cells (9 lymphocytes, 1 polymorph), normal side, and slurred speech. He had lost about 5 kg of weight in protein and glucose. Repeated CSF cytospin failed to yield the last 2 months. This was followed by altered behaviour with any neoplastic cells. Since the above investigations did not pro- fearfulness and talking to self for last four days. Patient had vide a clue to the diagnosis, he was evaluated further for pos- progressive difficulty in walking due to gait ataxia and postural sible paraneoplastic aetiology in view of rapidly progressing instability and required support for ambulation. There were no symptoms. Stool occult blood was positive, while serological other systemic or neurological symptoms during this period. markers for paraneoplastic cerebellar degeneration including There was no significant past medical history. Examination re- anti-Yo, anti-Hu, anti-Tr, anti-Ma and anti-Ri antibodies were vealed titubation, upbeat nystagmus, truncal and gait ataxia, all negative. Antibodies for autoimmune encephalopathy like bilateral limb ataxia, normal sensory examination, brisk deep N-methyl D-aspartate (NMDA) and LGI-1 were not tested. tendon reflexes and flexor plantar response. He was noted to Whole body positron emission tomography (PET)–com- have fluctuating sensorium, with multifocal rest and action in- puted tomography (CT) scan performed using 10 mCi of intra- duced myoclonus of limbs and facial muscles. He developed venous fluorodeoxyglucose (FDG) showed a 2.3 · 1.8 cm significant worsening of behavioural disturbances during eval- metabolically active focal mural thickening of the gastric fun- uation in hospital with visual hallucinations, and disturbed dus and two small pulmonary nodules in the left lower lobe sleep. and the right middle lobe measuring 4 and 3 mm in diameter, He was evaluated for subacute onset pan-cerebellar respectively (Fig. 1). Upper gastrointestinal endoscopy showed syndrome with encephalopathy; the differential diagnoses an elevated lesion with surface ulceration in the gastric fundus considered included paraneoplastic or non-paraneoplastic (Fig. 2A). Histopathology of the lesion showed gastric mucosa autoimmune encephalopathy, neurosyphilis, intracranial space effaced with sheets of large mononuclear lymphoid cells with occupying lesion, and Creuzfeldt Jakob disease. Investigations scant cytoplasm and vesiculated nuclei with prominent nucle- for the same were carried out. Routine investigations including oli. The cells were CD20 positive, cytokeratin (CK) negative, haemogram, renal and hepatic function tests, serum electro- and CD3 negative, suggestive of DLBCL of the stomach lytes, serum ammonia (27.8; ref: 11–35 lmol/l) and lactate (Fig. 2B and C). (12.7; ref: 4.5–20 mg/dl) were normal. Thyroid function tests Colonoscopy and bone marrow aspiration were non-con- were normal [T3: 83.8, (ref: 71–178 ng/dl); T4: 8.42, (ref: 4.5– tributory. A diagnosis of gastric DLBCL stage IV (pulmonary 12.5 lg/dl); TSH: 1.17, (ref: 0.4–5.5 lIU/ml)], anti thyroid per- metastases) with an International Prognostic Index (IPI) score oxidase antibody was borderline (35.9; ref: upto 34 IU/ml), of 4/5 and PCD and limbic encephalopathy was thus made. He Figure 1 Whole body PET–CT showing stomach wall thickening (A) and areas with increased metabolic activity (B–D). Gastric diffuse large B cell lymphoma presenting as paraneoplastic cerebellar degeneration 233 Figure 2 Gastroscopy showing elevated lesion with ulceration (A), Hematoxylin and eosin (H&E) stain of the stomach showing large B cells within the glandular structure (B) and immunochemistry (IHC) showing CD20 positive cells (C). initially received intravenous methyl prednisolone (1