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ABSTRACT

We present a 9 year-old boy complaining from headache andvomiting. Physical examination, lumber punction and cranialCT were compatible with tuberculosis meningitis. Clinicalmanifestations did not resolve with treatment for tuberculosis.After nine-months, spinal MRI revealed a mass in conusmedullaris, and the consequent biopsy from the mass revealedependymoma. Spinal cord tumors with retrograde seeding mustbe kept in mind in differential diagnosis of tuberculosis meningitis.

Spinal Ependymoma Mimicking TuberculousMeningitis in Childhood Age: A Case Report**Celkan Tiraje, *Ergul Yakup, ***Akcakaya Necla, ***Camcioglu Ylldlz, ****Albayram Said,

*****Oz Buge, *****Sar Mehmet, **Yildiz Inci* Assistant in Pediatrics

** Associate Professor in Department of Pediatric Hematology and Oncology,*** Professor in Department of Pediatric Infectious Disease and Immunology,

**** Associate Professor in Department of Radiology,***** Professor in Department of Pathology.

Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, TURKEY

Keywords: childhood, spinal ependymoma, tuberculousmeningitis

Correspondence to: Tiraje CELKAN, MD, Associate Professor, Department of Pediatric Hematology and Oncology, Cerrahpasa Faculty of Medicine, IstanbulUniversity, Istanbul, TURKEYPhone: 00 90 212 414 30 00/ 21956, Fax: 00 90 212 588 31 02, E-mail: tirajecelkan@yahoo.com

INTRODUCTION

Intramedullary spinal cord tumors are uncommon at anyage and quite rare in children; these tumors account for 4-10 % of neoplasms of the central nervous system (CNS),and essentially reflect the volume of the spinal cord inrelation to the volume of the remainder of the CNS.1-4

Ependymomas account for about 30% of spinal tumors,and 50% or more of intraspinal ependymomas are locatedin the caudal region.5-7 There could be basilar involvementsecondary to spinal CSF seeding, so a differential diagnosiswith meningitis should be performed.8 This tumors show atendency for CSF seeding and they are low-grade tumors,both features make this disease more difficult to diagnose.Here we present a case with spinal ependymoma, aiming toemphasize the cases whom were thought as tuberculosismeningitis due to clinical and radiographic features, andthe progression of disease could not be ameliorated,ependymoma should also be added in the differentialdiagnosis.

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CASE REPORT

A 9-year-old boy admitted to our clinic with headache,vomiting, and neck stiffness. His physical examinations werecompatible with meningitis. Lumber puncture wasxantochromic, protein content was increased (630 mg/dl),although glucose content was normal (CSF glucose 75 mg/dl, synchronous blood glucose 80 mg/dl). In microscopic

examination of the fluid, there were 260 leukocytes /mm3(%80 lymphocyte, %20 polymorphonuclear leukocyte), andabundant red blood cells. Cranial CT(computedtomography) revealed cranial basilar involvement (Figure

Figure 1. Axial CT image shows basal infiltration and contrastenhancement compatible with tuberculous meningitis.

Figure 2. An Axial T1-weighted contrast enhanced MR imageshows diffuse subarachnoid gelatinous material located basalcistern of brain. Note that prominent compression effect ofgelatinous material against to brain stem.

Figure 3. A, B . Saggital T2-weigted and T1-weighted contrast enhanced MRimages reveal an expansile mass lesion originating from conus medullaris. Notethat spinal canal widening due to chronic compression effect of gelatinousmaterial.

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1), suggesting the diagnosis of tuberculous meningitis,although the bacilli could not be isolated from the cultures.He was treated as tuberculous meningitis, and a ventriculo-peritoneal shunt was applied because of hydrocephalusdetected ten days after his presentation. No regression ofclinical manifestations with therapy was achieved,furthermore his neurological findings progressed (staggering,walking disturbances, urinary incontinence). Nine monthsafter diagnosis, spinal and cranial MRI (magnetic resonanceimaging) studies were performed. An expansile mass lesionwas detected in conus medullaris. Diffuse gelatinous softtissue material was noted in all spinal subarachnoid spaceand in cranial subarachnoid space specially located ventralportion of brain (Figures 2,3). Spinal biopsy revealed aGrade 2 ependymoma (according to the classification ofWorld Health Organization) originating from spinal cord(Figures 4,5). He was accepted as inoperable, a combinationof spinal and cranial radiotherapy with chemotherapy wasdecided for treatment regimen. After radiotherapy, therewas no clinical regression and we decided to begintemozolomide (100 mg/kg) treatment. After three cycles ofchemotherapy, his cranial lesions partially regressed, howeverhis spinal lesions continued to progress. We added etoposide(50 mg/m2 p.o.), vincristine (1.5 mg/m2 i.m. weekly) andmethotrexate ( 10 mg/m2 intratechal) to our treatmentprotocol. After 2 months, patient stabilized and refusedany more therapy. Patient is stable for two years with gaitdisturbance and no progression in his neurological findings,till he was lost .

DISCUSSION

Brain tumors are the second most common tumors of thechildhood age group in developed countries. In developingcountries such as Turkey, this group follows lymphomas.

However, ependymomas consist only 10 percent of braintumors diagnosed in childhood age group, and of these,only 10% is originated solely from spinal cord.9 It is alsocompatible with the literature that, these slowly-growingtumors may be confused with tuberculous meningitis in theearly stage of disease.8 In the presence of slowly progressingneurological findings, and no response to treatment,malignant situations should be thought. The signs andsymptoms may not be marked, due to low mitotic index ofthese slowly-growing t umors. In many spinal ependymomacases, the first symptoms of disease are lumber pain,lumbosciatic neuralgia, motor deficits, and sphincterdeficits.6 In contrast, our case presented with meningitisfindings of vomiting, headache, and neck stiffness. Thispresentation is very unusual for spinal cord tumors. Classicfindings emerged nine months after and up to our searchthis sit uation has not been previously reported. Due to thiscondition, patient is diagnosed as meningitis depending onthe absence of classic findings of spinal ependymomas, inaddition, his lumber puncture and cranial CT findings werecompatible with meningitis.9,10 When we re-evaluate to ourcase retrospectively, we saw that the initial CSF glucose levelwas within normal range, there was no growth in cult ures,and there was no response to treatment after nine months.We have given anti tuberculo sis treatment to this boy as thisdisease is endemic in Turkey, although the diagnosis was notproven by cultures or not detected by histologically. Thesepoints contradict with our first diagnosis. The treatmentmodalities for ependymomas consist of surgery, radiotherapyand chemotherapy. Prognosis may be v ery good if the tumorcan be resected totally by surgery.8,11,12 The response oftumor to radiotherapy and chemotherapy is not good.13,14

Our case also gave no proper response to radiotherapy orchemotherapy except temozolomide. Temozolomide(TMZ,3,4-dihydro-3-methyl-4-oxoimidazo 5,1-d]-as-

Figure 4. Perivasculer pseudorosette formation of ependymaltumor cells (HE x 400)

Figure 5. Low Ki-67 score (Ki-67 x 400)

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44Austral - Asian Journal of Cancer ISSN-0972-2556, Vol. 7, No.1, January 2008

tetrazine-8-carboxamide) is a new oral alkylating agent thathas recently demonstrated efficacy in the treatment ofmalignant gliomas.15 Despite moderate toxicity, objectiveresponse rates to temozolomide have been low, indicatingthat temozolomide has minimal activity in the high-gradegliomas of childhood.16 Although we did not achieve acomplete remission with temozolomide, we thought that ithad a great affect on stabilization of neurological symptomsand interrupting progression.

In conclusion, spinal cord ependymomas should bethought in differential diagnosis of patients with clinical andradiological findings compatible with tuberculosis meningitiswhose disease progression could not be altered with therapy.

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