Primary Spinal Intramedullary Lymphoma: a Case Report and Review of the Litera- Ture

Central JSM Neurosurgery and Spine Case Report *Corresponding author Feyza Karagoz Guzey, Topkapi Mahallesi, Kahalbagi Sokak 46/2 Fatih Istanbul, Turkey, 34093, Tel: Primary Spinal Intramedullary 905326334032; Email: Submitted: 28 November 2014 Lymphoma: A Case Report and Accepted: 05 January 2015 Published: 08 January 2015 Copyright Review of the Literature © 2015 Guzey et al. Feyza Karagoz Guzey1*, Yucel Hıtay1, Cihan Isler1, Abdurrahim OPEN ACCESS Tas1, Ozgur Aktas1, Azmi Tufan1, Mustafa Vatansever1 and Aslı Keywords 2 Kahraman Akkalp • Central nervous system lymphoma 1Department of Neurosurgery, Bagcilar Research and Training Hospital, Turkey • Lymphoma 2Department of Neurosurgery and Pathology, Bagcilar Research and Training Hospital, • Nonhodgkin lymphoma Turkey • Spinal intramedullary lymphoma • Spinal intramedullary tumor Abstract Background: Primary spinal intramedullary lymphoma is a very rare lesion. It has not specific laboratory or radiologic findings. Therefore false and late diagnosis is frequent. Case Report and Literature Review: A 55 year-old lady with multifocal primary spinal intramedullary B-cell non-Hodgkin’s lymphoma was reported. Review of literature yielded 45 cases with primary spinal intramedullary lymphoma and clinical and radiological characteristics of these 46 cases were evaluated. Results: Male/female ratio was 24/22. Median age of the patients was 51.5 years for all cases except a series consisting 14 cases to be reported in an article in which detailed data case by case was not given. Median age of those 14 cases was 62.5. Most of the cases were localized in the cervical and then thoracic segments. Multifocal spinal tumors were found in 12 patients. Most of the patients were admitted with rapidly progressing myelopathy symptoms and signs. Most frequently seen magnetic resonance appearance was T2 hyperintensity and dense and homogeneous contrast enhancement with spinal cord enlargement. Misdiagnosis was frequent (23 cases) and certain diagnosis could be provided with autopsy in 5 cases. Tissue sampling was performed mostly with spinal cord biopsy (27) and brain biopsy (10 cases). Most of the cases had B-cell lymphoma (26 cases). Patients were treated with various types of chemotherapy, radiotherapy or combination of them. Twenty-six cases were died during follow-up median 16.5 months (for 10 cases of the largest series consisting 14 cases) and 10 months (for other cases) after onset of symptoms and signs. Survival time was significantly different between neither cell types (B cell and other lymphomas) nor initial treatment modalities (chemotherapy, radiotherapy or combined of them). Conclusions: Primary spinal intramedullary lymphoma diagnosis is difficult, and misdiagnosis is frequent. It must be kept in mind for differential diagnosis especially in middle aged patients with rapidly progressing myelopathy findings. Prognosis is poor despite aggressive treatment. INTRODUCTION other CNS sites sequentially or concurrently [2]. It is not a rare event in the patients with brain lesions because of spread via Primary central nervous system (CNS) lymphoma sourced direct invasion from the medulla oblongata or dissemination with from brain, leptomeninges, eyes or spinal cord is an uncommon type of extranodal non-Hodgkin’s lymphoma which constitutes cerebrospinal fluid (CSF) [3]. Kawasaki et al [4] reported that as “primary” if there is no evidence of systemic lymphoma by brain lymphomas. However, isolated spinal cord involvement is there was spinal cord involvement in 4 of 14 cases with primary screeningonly 1% of at all the lymphomas time of diagnosis in the body [2]. [1]. Spinal Patients cord are lymphomas classified may be primary and originate in the spinal cord or accompany CNS lymphomas [5,6]. exceedingly rare and observed in 1-3.3% of all cases of primary Cite this article: Guzey FK, Hıtay Y, Isler C, Tas A, Aktas O, et al. (2015) Primary Spinal Intramedullary Lymphoma: A Case Report and Review of the Litera- ture. JSM Neurosurg Spine 3(1): 1049. Guzey et al. (2015) Email: Central Because of rarity of these lesions, primary intramedullary lymphomas (PIML) are usually not suggested in differential diagnosis in the patients with spinal intramedullary masses. Therefore, delayed diagnosis and misdiagnosis is very frequent. However, early diagnosis is important because effective treatment in early period of the disease may provide quite long survival. We reported a case with primary spinal intramedullary B-cell lymphoma and also we found 45 cases with detailed data in literature.CASE REPORT Characteristics of these 46 cases were discussed. A 55 year-old lady who had not any disease previously was referred to our clinic for spinal intramedullary mass at the Figure 1a Lumbar T1 (a) weighted sagittal and T2 weighted axial. lumbar segments of the spinal cord and conus region. She had a monoparesis of her left leg for one month. On her physical examination, there was a left leg paresis with 1-2/5 strength not hypoesthesia or sphincteric disturbances. Spinal magnetic resonanceon proximal imaging and 3/5 (MRI) strength revealed on distal an intramedullary muscles. There mass, was cord and conus medullaris at T12 to L1 levels. It extended into the41x14 left mm neural in size, foramen locating along in the with lumbar spinal segments root. The of massthe spinal was hypointense on T1-weighted, and slightly hyperintense on T2- weighted sections and it was enhanced homogeneously after intravenous contrast injection (Figure 1 a-d). In addition, there MRIswas a did small not mass reveal with other same pathologies. imaging characteristics in the filum terminale at L2 level (Figure 1 c). Brain, cervical and thoracic Routinely performed blood biochemical parameters, infection markers and peripheral blood cell counts were at normal levels Figure 1b MRI sections showed an intramedullary mass with T1 iso and T2 hyperintensity. (HIV) and hepatitis viruses were negative. Abdominal and and serologic tests including human immunodeficiency virus performed for primary tumor screening were also normal. thoracic computerized tomography and breast investigations She operated on for especially histological diagnosis. The borders intraoperatively. Only biopsy and decompressing duraplastyintramedullary could tumor be wasperformed. infiltrative On andthe it dayhad ofnot surgery,distinct prednisolone 250 mg intravenously was performed and it was continued for the day after and then gradually decreased. Just after operation, her leg paresis partly improved. Pathological investigation showed that the tumor was large B-cell lymphoma (Figure 2). Bone marrow aspiration and thorax and abdomen riskcomputerized factor. tomographies revealed no pathology. The tumor was accepted as a PIML. It could not be find any immunological Her treatment was continued in an oncology center. She was grays in 20 fractions and additional doses to the posterior fossa Figure 1c (9treated grays with in 5 fractions)whole craniospinal and to the radiotherapyoperation site (RT) (T12-L2) consisting (9 grays 36 in 5 fractions). However she died because of dissemination of the the disease). the study. The cases that had been reported in CNS lymphoma disease after 3 months from operation (4 months from onset of seriessystemic were disease introduced during firstinto admissionthe study wereonly notif theirincluded detailed into REVIEW OF LITERATURE Medline were searched for PIML. All found papers were characteristics had been reported in the articles. Forty-five cases evaluated. Cases with previously known lymphoma story or were evaluated for their clinical and radiological characteristics found in the literature [2,3,6-34] and total 46 cases with our one JSM Neurosurg Spine 3(1): 1049 (2015) 2/8 Guzey et al. (2015) Email: Central Table 1: Articles reporting the cases with primary spinal intramedullary lymphoma. Literature Year n Herbst et al [7] 1976 1 Bruni et al [8] 1977 1 Mitsumoto et al [9] 1980 1 Slager et al [10] 1982 1 1 HautzerItami et et al al [12] [11] 19831986 1 1987 1 Figure 1d It had indistinct borders and enhanced homogeneously Landan et al [13] 1990 1 after intravenous contrast injection (c and d). In addition, there was a Bluemke and Wang [14] 1990 1 at L2 level (c). NakaoSlowik et et al al [15] [3] 1 second small mass with similar characteristics in the filum terminale Schild et al [16] 19941995 1 McDonald et al [17] 1995 1 Urasaki et al [18] 1996 1 1996 1 CarusoKim et et al al [19] [20] 1998 1 Drouet et al [21] 1999 1 Pels et al [22] 2000 1 2000 1 Thurnher et al [23] 2001 1 Bekar et al [24] 2002 2 HerrlingerNakamizo et al [25][26] 2002 1 Legais et al [27] 2002 1 2002 1 Figure 2 Diffuse and strong CD20 immunoreactivity in the neoplastic SchwarzLee et alet [29]al [28] 2002 1 2007 1 cells (CD20 X400). 2007 1 (Table 1). Intravascular lymphoma cases [26,27] were not Machiya et al [30] excluded in spite of some peculiar characteristics of this entity Peltier et al [31] 2010 1 Toshkezi et al [32] 2011 Flanagan et al [2] 2012 141 spinalbecause intramedullary there were 3 caseshistiocytic with intravascular lymphoma without B cell lymphoma systemic in the series of Flanagan et al [2]. Also, two cases with primary LinSato et et al al [33] [6] 1 was included into the study despite the paper could not be found. 2013 1 involvement [9,32] were included into the study. One case [15] However, characteristics of that case were given in detail in other BhushanamOur case et al [34] 2014 1 papers. Total 2014 46 n: number of patients reported in articles wereFourteen

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