Cerebral Mass Lesions Research Articles

CTNI-83. MATRIX REGIMEN FOR NEWLY DIAGNOSED PRIMARY CNS DIFFUSE B-CELL LYMPHOMA

Abstract We report our experience treating 25 patients with newly diagnosed primary CNS diffuse B-cell lymphoma with the MATRix regimen as reported by Ferreri, et al in 2016. The median age was 66 y (range 30-85); 12 patients were men and 13 were women. Twenty-three had cerebral mass lesions, 1 had a paraspinal mass, and 1 leptomeningeal disease. Treatment consisted of 4 cycles of rituximab 375 mg/sq m (Days 1, 6); methotrexate 3.5 g/sq m (Day 2); cytarabine 2 g/sq m every 12 h (Days 3, 4); and thiotepa 30 mg/sq m (Day 5). Patients without disease progression received carmustine 400 mg/sq m and thiotepa 5 mg/kg followed by autologous bone marrow transplantation. Four patients were switched to MATRix after 6 cycles of methotrexate and rituximab and received only 2 initial cycles including cytarabine and thiotepa. Overall, 18 patients completed initial chemotherapy. Sixteen of these 18 went on to transplantation; one patient had had early disease progression, and in one no stem cells could be collected. Five patients (median 73 y; range 64-85 y) died early after 1-2 cycles. The causes of early death were neutropenic fever, septic thrombophlebitis, and cardiac arrest. One of the 16 patients who completed the entire regimen including transplantation died of fungal pneumonia and the other 15 experienced no serious acute toxicity. One patient had late disease progression and 2 developed symptomatic leukoencephalopathy. Seventeen of the 25 patients remain alive. Median TTP for the cohort is 605 days, and OS 738 days. These preliminary data suggest that the MATRix regimen in safe and highly effective in the newly diagnosed setting. However, the high incidence of early death in patients over 65 y shows that the regimen is too toxic for older individuals.

Disseminated Nocardiosis with Ocular Involvement in a Patient with Anca-associated Vasculitis.

A 62-year male patient, diagnosed with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), developed proptosis and decrease in visual acuity while on rituximab treatment. As the ophthalmological examination and imaging studies could not exclude tumour of the orbit, enucleation of the orbit was performed. The histopathology displayed necrosis and inflammation. Because the clinical, laboratory and pathological findings of the patient suggested a vasculitis exacerbation, the immunosuppressive treatment was continued. However, the patient developed confusion and hemiplegia with cerebral mass lesions on imaging. The subsequent report of the pathology revealed a nocardial infection of the eye. The patient was diagnosed with nocardiosis with ocular and cerebral involvement. Despite efficient antimicrobial therapy, the disease progressed rapidly causing death. This case is unique as it describes disseminated nocardiosis with ocular and cerebral involvement in an AVV patient. Key Words: Immunosuppression, Nocardiosis, ANCA-associated vasculitis, Proptosis.

CTNI-59. MATRIX REGIMEN FOR NEWLY DIAGNOSED PRIMARY CNS DIFFUSE B-CELL LYMPHOMA

Abstract We report our experience treating 22 patients with newly diagnosed primary CNS diffuse B-cell lymphoma with the MATRix regimen as reported by Ferreri, et al in 2016. The median age was 64 y (range 45-85); 10 patients were men and 12 were women. Twenty had cerebral mass lesions, 1 had a paraspinal mass, and 1 leptomeningeal disease. Treatment consisted of 4 cycles of rituximab 375 mg/sq m (Days 1, 6); methotrexate 3.5 g/sq m (Day 2); cytarabine 2 g/sq m every 12 h (Days 3, 4); and thiotepa 30 mg/sq m (Day 5). Patients without disease progression received carmustine 400 mg/sq m and thiotepa 5 mg/kg followed by autologous bone marrow transplantation. Four patients were switched to MATRix after 6 cycles of methotrexate and rituximab and received only 2 initial cycles including cytarabine and thiotepa. Overall, 16 patients completed initial chemotherapy. Fourteen of these 16 went on to transplantation; one patient had had early disease progression, and in one no stem cells could be collected. Five patients (median 73 y; range 64-85 y) died early after 1-2 cycles. The causes of early death were neutropenic fever, septic thrombophlebitis, and cardiac arrest. One patient who completed the entire regimen including transplantation died of fungal pneumonia and the other 13 experienced no serious acute toxicity. One patient had late disease progression and one developed symptomatic leukoencephalopathy. Fourteen of the 22 patients remain alive. Median OS for the cohort is 663 days. These preliminary data suggest that the MATRix regimen in safe and highly effective in the newly diagnosed setting. However, the high incidence of early death in patients over 65 y suggests that the regimen may be too toxic for older individuals.

AML-372 Acute Promyelocytic Leukemia: A Case of Pseudotumor Cerebri Syndrome During Treatment Consolidation

Acute promyelocytic leukemia (APL) is a subtype of acute myeloid leukemia with distinctive morphological, clinical, and genetic features. Pseudotumor cerebri, also known as idiopathic intracranial hypertension, describes a neurologic syndrome characterized by headache, visual changes, papilledema without cerebral mass lesion, venous sinus thromboses, or obstructive hydrocephalus. Risk factors for the development of pseudotumor cerebri are obesity, women in the childbearing years, and hypervitaminosis A. The modified Dandy criteria are used for pseudotumor cerebri syndrome (PTCS) diagnosis. This complication has a high incidence in the pediatric population, particularly within 2-3 weeks of induction treatment for APL. Pseudotumor cerebri is a rare complication during consolidation therapy for APL. The reported incidence of "probable" PTCS is 1.7% in APL patients treated with ATRA. Case Report: We report here the case of a 20-year-old woman, obese with a BMI of 34.5 kg/m2, diagnosed with low-/intermediate-risk APL and treated with ATRA and ATO as induction treatment. After induction, she achieved MRD-negative complete morphological remission. During the first 2 weeks of the first cycle of consolidation-ATRA and ATO-the patient developed progressive diplopia and headaches without response to standard analgesic therapy. Additionally, she complained of nausea and vomiting. The ophthalmological exam with fundus examination revealed bilateral papilledema. The neurological exam was normal. There were no abnormal findings in the head CT scan or MRI of the brain. Thus, a diagnosis of "probable" pseudotumor cerebri was established. ATRA was discontinued and she received acetazolamide and dexamethasone with resolution of neurological symptoms within the next 7 days. Upon the next consolidation ATRA and ATO, the headaches resumed. The patient received all the ATRA consolidation cures as scheduled but with a dose reduction to 80% of the initial dose and in association with acetazolamide. She completed consolidation treatment and continues to be in molecular complete remission. Conclusions: This case report highlights the possibility of pseudotumor cerebri development in young adult patients with APL during consolidation therapy. The neurological symptoms were reversible, and the patient received all the ATRA consolidation. It is important to exclude other causes of intracranial hypertension including thromboses, infections, and disease involvement.

Mind the gap: IgG4-related disease mimicking infectious cerebral mass lesions

BackgroundCerebral intraparenchymal masses represent usually a neoplastic, or infectious differential diagnostic workup in neurology or infectious disease units.Case presentationOur patient was an 82-year-old male presenting with seizures, cerebral masses and a history of past treated pulmonary tuberculosis. Initial workup included a differential diagnosis of an infectious mass/multiple abscess. After exclusion of infectious or primary neoplastic origins by negative HIV serology, the absence of immune suppression, endocarditic lesions, negative results of blood cultures and bronchoalveolar lavage, negative cerebrospinal fluid workout on spinal tap led to exclusion of infectious causes. A surgical procedure was performed to access one of the lesions. This yielded a firm, cyst-like mass of histiocytic granulomatous tissue with a conspicuous plasmacellular component and a relevant IgG4 plasmacellular component consistent with IgG4-related disease.Steroid treatment determined conspicuous improvement and led to discharge of the patient.ConclusionParenchymal IgG4-related disease may be included as a new entity in the differential diagnosis of single or multiple cerebral masses in addition to infectious or neoplastic etiology.

CTNI-57. MATRix REGIMEN FOR NEWLY DIAGNOSED PRIMARY DIFFUSE B-CELL LYMPHOMA OF THE CENTRAL NERVOUS SYSTEM

Abstract We report our experience treating 20 patients with newly diagnosed primary diffuse B-cell lymphoma of the central nervous system with the MATRix regimen as reported by Ferreri, et al in 2016. The median age was 65 y; 8 patients were men and 12 were women. Eighteen had cerebral mass lesions, 1 had a paraspinal mass, and 1 leptomeningeal disease. Treatment consisted of 4 cycles of rituximab 375 mg/sq m (Days 1, 6); methotrexate 3.5 g/sq m (Day 2); cytarabine 2 g/sq m every 12 h (Days 3, 4); and thiotepa 30 mg/sq m (Day 5). Patients without disease progression received carmustine 400 mg/sq m and thiotepa 5 mg/kg followed by autologous bone marrow transplantation. Four patients were switched to MATRix after 6 cycles of methotrexate and rituximab and received only 2 initial cycles including cytarabine and thiotepa. Overall, 14 patients completed initial chemotherapy. Twelve of these 14 went on to transplantation; one patient had early disease progression, and in one no stem cells could be collected. All 4 patients over 70 y died early after 1-2 cycles; the median age of these was 80 y, and the causes of early death were neutropenic fever, septic thrombophlebitis, and cardiac arrest. One patient who completed the entire regimen including transplantation died of fungal pneumonia; the other 13 experienced no serious acute toxicity. One patient had late disease progression; one developed symptomatic leukoencephalopathy. Thirteen of the 20 patients remain alive. Median OS for the cohort is 698 days. These preliminary data suggest that the MATRix regimen in safe and highly effective in the newly diagnosed setting. However, the high incidence of early death in patients over 70 y suggests that the regimen may be too toxic for elderly individuals.

An unexpected intracerebral lesion \u2013 case report of a superinfected aspergillosis mimicking a brain metastasis

BackgroundInvasive aspergillosis of the central nervous system is a rare but increasingly prevalent disease. We present the unusual case of an immunosuppressed patient suffering from unexpected superinfected invasive aspergillosis with cerebral, pulmonal, and adrenal manifestations, mimicking a metastasized bronchial carcinoma. This report reveals the importance of including aspergillosis in the differential diagnosis of a cerebral mass lesion in the light of unspecific clinical findings.Case presentationA 58-year-old immunocompromised female presented to our emergency department with a single tonic-clonic seizure. Imaging showed a ring enhancing cerebral mass with perifocal edema and evidence of two smaller additional hemorrhagic cerebral lesions. In the setting of a mass lesion in the lung, and additional nodular lesions in the left adrenal gland the diagnosis of a metastasized bronchus carcinoma was suspected and the cerebral mass resected. However, histology did not reveal any evidence for a neoplastic lesion but septate hyphae consistent with aspergillus instead and microbiological cultures confirmed concomitant staphylococcal infection.ConclusionsA high index of suspicion for aspergillus infection should be maintained in the setting of immunosuppression. Clinical and radiological findings are often unspecific and even misleading. Definite confirmation usually relies on tissue diagnosis with histochemical stains. Surgical resection is crucial for establishing the diagnosis and guiding therapy with targeted antifungal medications.

Role of magnetic resonance spectroscopy in adequately differentiating neoplastic from non-noplastic and low-grade from high-grade lesions in brain masses

Objective: The aim of this study was to provide objective data on the clinical utility of MRS in difference diagnosis of brain lesion and also to provide metabolite ratios neoplastic from non-neoplastic lesion and low-grade from high-grade neoplasms. Method: A prospective study conducted at Sri Aurobindo Institute of Medical Sciences and PG Institute, Indore and Department of Radiology after approval from the ethical and research committee. The duration of this study was June 2018 to July 2020. A total of 102 patients (age range 21-60 years) Who were found to have cerebral mass lesion by computed tomography (CT) and convention MRI and from whom high quality MRS imaging data were obtained, were prospectively assessed and included in this study.Result: The mean age was 53.5±3.15 (range 28-70 years). Majority of the patients in the study were males forming 70 (68.6%) and females 32 (31.3%). Various spectroscopy ratios like Cho/Cr, Cho/NAA, Cho+Cr/NAA, NAA/Cr were calculated with the help of MRS. Conclusion: MRS gives information about the biochemical changes in tissues, which appear earlier than the structural changes, and so, in recent times, MRS has been used as a noninvasive method for the diagnosis and grading of brain tumors. All neoplastic lesion showed increase Cho and decreased NAA and this was more prominent in high-grade neoplasms. While we did not detect any LL peak in all 27 low-grade neoplasms, but 60 high-grade neoplasms showed an LL peak.

CTNI-64. MATRIX REGIMEN FOR NEWLY DIAGNOSED PRIMARY DIFFUSE B-CELL LYMPHOMA OF THE CENTRAL NERVOUS SYSTEM

Abstract We report our experience treating 16 patients with newly diagnosed primary diffuse B-cell lymphoma of the central nervous system with the MATRix regimen as reported by Ferreri, et al in 2016. The median age was 66 years (y); 8 patients were men and 8 were women. Fourteen had cerebral mass lesions, 1 had a paraspinal mass, and 1 leptomeningeal disease. Treatment consisted of 4 cycles of rituximab 375 mg/sq m (Days 1, 6); methotrexate 3.5 g/sq m (Day 2); cytarabine 2 g/sq m every 12 h (Days 3, 4); and thiotepa 30 mg/sq m (Day 5). Patients without disease progression received carmustine 400 mg/sq m and thiotepa 4 X 5 mg/kg followed by autologous bone marrow transplantation. Four patients were switched to MATRix after 6 cycles of methotrexate and rituximab and received only 2 initial cycles including cytarabine and thiotepa. Overall, 12 patients completed initial chemotherapy. Eleven of these 12 went on to transplantation; one patient had early disease progression and did not proceed to transplantation. All 4 patients over 70 y died early after 1–2 cycles; the median age of these was 80 y, and the causes of early death were neutropenic fever, septic thrombophlebitis, and cardiac arrest. One patient who completed the entire regimen including transplantation died of fungal pneumonia; the other 11 experienced no serious acute toxicity. One patient had late disease progression; one developed symptomatic leukoencephalopathy. Nine of the 16 patients remain alive. At a median follow-up of 23 months, median time to progression was 421 days and overall survival 564 days. These preliminary data suggest that the MATRix regimen in safe and highly effective in the newly diagnosed setting. However, the high incidence of early death in patients over 70 y suggests that the regimen may be too toxic for elderly individuals.

Primary Angiitis of the Central Nervous System Presenting as a Cerebral Mass Lesion: A Case Report and Literature Review

Primary angiitis of the central nervous system (PACNS) is a rare form of vasculitis and is confined entirely to the central nervous system (CNS)without systemic involvement. We report a rare case of PACNS in a 39-year-old female with new onset seizures and a right frontal enhancing mass. Initially the patient was thought to have a high-grade glioma and thus underwent a right frontal craniotomy for resection of right frontal mass. Intraoperatively, two fresh tissue samples were sent for intraoperative consultation. Sample 1 showed predominantly necrotic tissue and scant glial cells while sample 2 revealed glial tissue favoring gliosis versus low-grade neoplasm with necrosis and a few acute inflammatory cells. Final pathological diagnosis was consistent with PACNS. Postoperatively, the patient recovered well from surgery with no neurological deficits and was discharged on postoperative day 3. Two weeks after surgery the patient was started on cyclophosphamide and prednisone by Rheumatology. At one month follow up, the patient remained asymptomatic and seizure free.

Differentiation between neoplastic and nonneoplastic brain masses using intermediate echo time MR Spectroscopy

AbstractAim: The aim of this study was to evaluate the role of intermediate echo time (TE) Proton Magnetic Resonance Spectroscopy (1 H-MRS) in the differential diagnosis of neoplastic and nonneoplastic cerebral mass lesions.Materials and Methods: The research was done at Cukurova University in a 1.5 Tesla whole-body MR system. In point-resolved-single volume-spectroscopy (PRESS) localization method, Probe-P pulse sequence was studied with intermediate TE (TE=144). Of 55 cases whose diagnosis was confirmed with the histopathologically or clinical and radiological follow-up, MR Spectroscopy findings were discussed.Results: Twenty cases’ definite diagnosis were neoplastic and thirty-five cases’ were nonneoplastic lesions. For tumor detection in cerebral mass lesions, intermediate TE MRS was determined 100% sensitive and 91.7% specific. The positive predictive value was 86.4% and the negative one was 100%. In this study, in the neoplastic-nonneoplastic lesion differential diagnosis, NAA/Cho, NAA/Cr, Cho/Cr, and Cho/NAA ratios were evaluated and the most useful of them were found to be Cho/NAA ratio. Conclusion: It is concluded that intermediate TE MR Spectroscopy is a reliable imaging technique for the neoplastic-nonneoplastic differential diagnosis of the cerebral mass lesions.

ACTR-16. MATRix REGIMEN FOR NEWLY DIAGNOSED PRIMARY DIFFUSE B-CELL LYMPHOMA OF THE CENTRAL NERVOUS SYSTEM: PRELIMINARY RESULTS

Abstract We report our preliminary experience treating 9 patients with newly diagnosed primary diffuse B-cell lymphoma of the central nervous system with the MATRix regimen as reported by Ferreri, et al in 2016. The median age was 63 y; 5 patients were men and 4 were women. Seven had cerebral mass lesions, 1 a paraspinal mass, and 1 leptomeningeal disease. Five patients underwent biopsy; 2 imaging total resection; 1 subtotal resection; and in 1 the extent of resection was indeterminate. Treatment consisted of 4 cycles of rituximab 375 mg/sq m (Days 1, 6); methotrexate 4 g/sq m (Day 2); cytarabine 2 g/sq m every 12 h (Days 3, 4); and thiotepa 30 mg/sq m (Day 5). Patients without disease progression received carmustine 400 mg/sq m and thiotepa 5 mg/kg followed by autologous bone marrow transplantation. Two patients were switched to MATRix after 6 cycles of methotrexate and rituximab and received 2 additional cycles that included cytarabine and thiotepa. Overall, 7 patients completed initial chemotherapy, with 5 complete responses and 2 partial responses. Six of these 7 went on to transplantation and remain alive without disease progression after a median follow-up of 470 days. One patient with initial PR progressed and did not proceed to transplantation. Two patients died after 1 cycle; they were both over 70 y (76 and 84) and succumbed to sepsis with neutropenic fever. No serious acute toxicity occurred in the 6 patients who completed the entire regimen including transplantation; 1 patient has experienced symptomatic leukoencephalopathy. Overall progression free survival was 394 days. These preliminary data suggest that the MATRix regimen in safe and highly effective in the newly diagnosed setting. However, the high early mortality in patients over 70 years in this group suggests that the regimen may be too toxic for very elderly individuals.

Impact of anamnestic information and neurological deficits on the detection rate of secondary headaches

Headaches are avery common symptom and imaging is important to rule out symptomatic causes. For clinical differentiation between primary and secondary headaches an exact anamnesis and neurological examination are important. The aim of this study is therefore to identify anamnestic and neurological information that is associated with secondary headaches. Moreover, this study gives an overview of the causes and differential diagnoses of secondary headaches. We performed aretrospective analysis of 239 patients ≥18years with headaches who had undergone computed tomography or magnetic resonance imaging. The impact of basic characteristics such as age and gender as well as anamnestic (pain intensity, thromboembolic risk profile) and clinical information (neurological deficit, papilledema, reduced vigilance) was tested by χ2 test at the significance level p < 0.05. In all, 27 of the included patients (11.3%) showed intracranial pathologies that required treatment. The most frequent pathologies were intracranial hypertension (9patients), cerebral mass lesions (7patients) and thrombosis of the cranial sinus/veins (3patients). There was asignificant association of apathologic imaging finding and neurological deficits (p = 0.001) and apapilledema (p < 0.001). Reduced vigilance, pain intensity and thromboembolic risk factors as well as age and gender showed no significant association. Aneurological deficit and especially papilledema are hints towards secondary headaches and should result in computed tomography or magnetic resonance imaging. Other factors such as reduced vigilance, pain intensity, age and gender have no relevant impact on the occurrence of intracranial pathologies.

CT-guided Stereotactic Microsurgical Resection of Cerebral Mass Lesions

CT-guided Stereotactic Microsurgical Resection of Cerebral Mass Lesions

Takotsubo syndrome in hemodynamically unstable patients admitted to the intensive care unit - a retrospective study.

Takotsubo syndrome (TS) is an acute cardiac condition that is often triggered by critical illness but that has rarely been studied in the intensive care unit (ICU) setting. The aim of this study was to (i) estimate the incidence of TS in a hemodynamically unstable ICU-population; (ii) identify predictors of TS in this population; (iii) study the impact of TS on prognosis and course of hospitalization. Medical records from all patients admitted to our general ICU from 2012 to 2015 were analyzed. TS was defined as having transient regional wall motion abnormalities (RWMA) with a typical pattern not attributable to a history of coronary artery disease or acute coronary syndromes. Out of 6470 patients admitted to the ICU, echocardiography due to hemodynamic instability was performed in 1051 patients; 467 had LV dysfunction and 59 fulfilled TS criteria. Patients with TS had higher SAPS 3 scores on admission than patients with normal LV function. Septic shock, cardiac arrest, cerebral mass lesion, female sex and low pH were independently associated with TS on admission. Patients with TS needed more ICU resources measured by higher NEMS scores and longer ICU-stay. Crude mortality was higher in TS patients (32%) vs the ICU-population (20%, P = 0.020), but there were no differences in a SAPS 3 adjusted analysis. TS was not an uncommon cause of LV dysfunction in hemodynamically unstable ICU-patients. Furthermore, TS was associated with a more complex disease. TS is a complication to take in consideration in the critically ill.

Serum concentrations of glial fibrillary acidic protein (GFAP) do not indicate tumor recurrence in patients with glioblastoma.

Recent studies identified serum concentrations of the astroglial protein glial fibrillary acidic protein (GFAP) to be indicative of glioblastoma (GBM) in patients with newly diagnosed space occupying cerebral mass lesions. Until now, no data is available whether GFAP serum concentrations decrease after first therapy and whether GFAP may be used as a predictor of survival and an indicator of tumor recurrence. In this prospective study, we included 44 patients with a single space occupying cerebral mass lesion suspicious for GBM. GBM was histopathologically proven in 33 cases. After initial therapy, patients were followed up until tumor recurrence (defined according to the RANO criteria) or death (maximum observation period 78 weeks). Blood was sampled on a regular basis, and GFAP serum levels were determined using an immunofluorescence assay. Prior to any intervention, 14 of the 33 GBM patients had elevated GFAP serum concentrations (median 0.25µg/L, interquartile range 0.13-0.53), whereas only one out of 11 patients having other tumor entities revealed a slightly increased GFAP serum level (0.06µg/L). Following surgery (i.e., biopsy, full or partial resection), all initially GFAP positive GBM patients showed decreased serum concentrations. During the follow-up period, we found a minimal GFAP increase in one patient only (0.04µg/L; week 52), although 23 out of 31 available GBM patients developed tumor progression or died. No difference was found regarding the survival rate and the time to tumor recurrence between initially GFAP positive and GFAP negative GBM patients. In GBM patients, initially elevated GFAP serum concentrations decrease after the first diagnostic or therapeutic intervention. GFAP was not predictive for tumor recurrence.

The outcome of early decompressive craniectomy of rapidly evolving cerebral parenchymatous mass lesion: vascular or trauma

The outcome of early decompressive craniectomy of rapidly evolving cerebral parenchymatous mass lesion: vascular or trauma

Toxoplasmic Encephalitis in Patient with Acquired Immunodeficiency Syndrome.

Toxoplasmic encephalitis (TE) is an opportunistic infection found in immunocompromised patients and TE related cerebral mass lesion is often reported in acquired immunodeficiency acquired immunodeficiency syndrome (AIDS) patients. However, incidence of TE related AIDS in Korea is still rare and is unfamiliar to neurosurgeons. Differential diagnosis is needed to rule out other brain lesions. A 39-year-old man visited the emergency room with rapid progressive left hemiparesis. Magnetic resonance imaging showed a ring-enhanced mass lesion in his right frontal lobe. Human immunodeficiency virus and Toxoplasma gondii immunoglobulin G were tested positive by a serologic test. We report here a rare case of patient with TE related AIDS.

Neuro-Behçet’s disease presenting with tumour-like lesions and responding to rituximab

We describe a patient with neuro-Behçets disease (NBD) that presented with symptoms of raised intracranial pressure including papilloedema. MRI revealed tumour-like lesions which, on biopsy, confirmed an active vasculitis. Treatment was commenced with prednisone and cyclophosphamide which proved unsuccessful with enlargement of the cerebral mass lesions. Infliximab and mycophenolate were trialled also without benefit. The patient required ventriculoperitoneal shunts to relieve the symptoms of hydrocephalus. Rituximab was then commenced with significant symptomatic and imaging improvement. The case is unique, in our experience, in the need for shunting to relieve the symptoms of hydrocephalus related to vasculitis.

LINFOMA PRIMARIO DEL SISTEMA NERVIOSO CENTRAL COMO PRIMERA MANIFESTACIÓN DE SIDA. PRESENTACIÓN DE UN CASO Y REVISIÓN DE LA LITERATURA

El linfoma primario del sistema nervioso central (LPSNC) es una neoplasia infrecuente que comprende solo el 5% del total de los linfomas de localización extranodal. El LPSNC se define como un linfoma de tipo No Hodgkin (LNH) que solo infiltra el eje cerebro espinal sin compromiso sistémico. Esta neoplasia es más frecuente en sujetos con inmunodeficiencias congénitas y adquiridas. En los pacientes con SIDA, el LPSNC ocurre con igual incidencia en todos los grupos de riesgo y edades pero con una mayor prevalencia en aquellos con recuentos de linfocitos T CD4 + de &lt; de 50 células/µL. Se trata de un linfoma de células grandes, de alto grado de malignidad, y fuertemente asociado en su patogenia al virus de Epstein Barr (VEB) cuyo genoma se detecta en las células linfoides atípicas. La presencia del genoma del VEB se detecta en el 100% de los casos de LPSNC asociado al SIDA pero esto no ocurre en otras clases de pacientes con esta misma neoplasia. En pacientes con SIDA y lesiones de masa cerebral ocupante, la detección del genoma del VEB en el líquido cefalorraquídeo (LCR) a través de la reacción en cadena de la polimerasa (PCR) tiene una sensibilidad del 83% a 100% y una especificidad &gt; del 90% para el diagnóstico. El uso difundido de la terapia antirretroviral de gran actividad (TARGA) se asoció con una declinación en la incidencia de LPSNC. El pronóstico de esta neoplasia en pacientes con SIDA es pobre y se asocia con una mediana de supervivencia de 2 a 3 meses en la era previa a la TARGA con una discreta prolongación en la supervivencia con la TARGA. Se presenta un paciente que desarrolló un LPSNC como primera manifestación de SIDA y se efectúa una actualización de la literatura sobre el tema.