Primary central nervous system angiosarcoma with recurrent acute subdural hematoma.

Angiosarcoma is an infrequent tumor among sarcomas, especially presenting as a primary tumor within the central nervous system, which can lead to a rapid neurological deterioration and death in few months. We present a 41-year old man with a right frontal enhancing hemorrhagic lesion. Surgery was performed with histopathological findings suggesting a primary central nervous system angiosarcoma. He was discharged uneventfully and received adjuvant chemotherapy and radiotherapy. At 5 months, the follow-up MRI showed two lesions with an acute subdural hematoma, suggesting a relapse. Surgery was again conducted finding tumoral membranes attached to the internal layer of the duramater around the right hemisphere. The patient died a few days later due to the recurrence of the subdural hematoma. This case report illustrates a rare and lethal complication of an unusual tumor. The literature reviewed shows that gross-total resection with adjuvant radiotherapy seems to be the best treatment of choice.

Primary central nervous system angiosarcoma with recurrent acute subdural hematoma.

Angiosarcoma is an infrequent tumor among sarcomas, especially presenting as a primary tumor within the central nervous system, which can lead to a rapid neurological deterioration and death in few months. We present a 41-year old man with a right frontal enhancing hemorrhagic lesion. Surgery was performed with histopathological findings suggesting a primary central nervous system angiosarcoma. He was discharged uneventfully and received adjuvant chemotherapy and radiotherapy. At 5 months, the follow-up MRI showed two lesions with an acute subdural hematoma, suggesting a relapse. Surgery was again conducted finding tumoral membranes attached to the internal layer of the duramater around the right hemisphere. The patient died a few days later due to the recurrence of the subdural hematoma. This case report illustrates a rare and lethal complication of an unusual tumor. The literature reviewed shows that gross-total resection with adjuvant radiotherapy seems to be the best treatment of choice.

Imaging and Radiologic-Pathologic Correlation in Granular Cell Astrocytomas: Report of 2 Cases.

BACKGROUND: Granular cell astrocytoma is a rare and aggressive subtype of astrocytoma that is histopathologically well defined in the literature. It is formed by polygonal cells with granular cytoplasm mixed with neoplastic astrocytes and usually a perivascular infiltrate of lymphocytes. Despite its unusual histologic appearance, relevant radiologic features have not yet been described. CASE DESCRIPTION: We report 2 middle-aged patients with neurologic symptoms secondary to a newly diagnosed brain tumor. The absence of central tumor necrosis as well as the presence of an atypical pattern of enhancement and areas of intense diffusion restriction on magnetic resonance imaging in both cases led to the diagnosis of primary central nervous system lymphoma. Histopathologic findings in both tumors showed an aggressive astrocytoma with a prominent granular cell population and perivascular lymphocytic cuffing in tissue, corresponding to a granular cell astrocytoma. Despite the favorable prognostic factors, including World Health Organization grades II and III astrocytomas and IDH mutations, the outcome was poor. CONCLUSIONS: Granular cell astrocytomas can show unusual aggressive radiologic features that do not correspond to their histopathologic grade of malignancy. The presence of perivascular lymphocytic infiltrate may alter the typical radiologic appearance of common astrocytomas.

Análisis del impacto clínico de la reclasificación diagnóstica de gliomas cerebrales según la clasificación de la Organización Mundial de la Salud (2016) / Analysis of the clinical impact of the diagnostic reclassification of brain gliomas according to the World Health Organization classification (2016)

Introducción: Desde la introducción de los criterios genéticos y moleculares en la clasificación de la Organización Mundial de la Salud (OMS) de tumores cerebrales de 2016, se ha producido una reclasificación diagnóstica entre determinados astrocitomas y oligodendrogliomas con discordancias histológicas y genéticas, cuyo pronóstico se desconoce. Objetivo: Analizar las implicaciones de la reclasificación diagnóstica de los gliomas cerebrales según los criterios de la OMS de 2016, especialmente según la mutación de la isocitrato deshidrogenasa (IDH) y la codeleción 1p19q. Pacientes y métodos. Estudio retrospectivo de los gliomas tratados desde el 1 de enero de 2012 hasta el 31 de diciembre de 2016, con análisis de los aspectos clinicorradiológicos y pronósticos, y con seguimiento disponible y completo hasta el 31 de marzo de 2019. Resultados: De 147 gliomas cerebrales, en 69 (astrocitomas u oligodendrogliomas de grados II-IV) se realizaron un diagnóstico molecular y una reevaluación diagnóstica. Se detectaron 24 gliomas reclasificados, habitualmente oligodendrogliomas que pasaron a astrocitomas, y que mostraron mayores supervivencias, derivadas de la no reclasificación en grado IV. Los gliomas reclasificados, todos de grados II/III, comenzaron mayoritariamente con crisis, sin focalidad, con lesiones únicas, <17 cm3 y con edema, aunque con similar supervivencia. Los factores pronósticos fueron: edad joven, focalidad, grado II y no captación de contraste o necrosis, o multiplicidad. No se detectaron variaciones según el patrón molecular con mutación en la IDH o codeleción. Conclusión: Los cambios diagnósticos tras la clasificación de la OMS de 2016 presentan características clinicorradiológicas específicas en esta serie, aunque no mayores supervivencias, si bien, por la supervivencia habitual en estos casos, precisarían un mayor tiempo de seguimiento

Introduction: Since the introduction of genetic and molecular criteria in the 2016 World Health Organization (WHO) classification of brain tumours, there has been a diagnostic reclassification between certain astrocytomas and oligodendrogliomas with histological and genetic discordances, the prognosis of which is unknown. Aim: To analyse the implications of the diagnostic reclassification of brain gliomas according to the 2016 WHO criteria, especially depending on isocitrate dehydrogenase (IDH) mutation and 1p19q codeletion. Patients and methods: We conducted a retrospective study of gliomas treated from 1 January 2012 to 31 December 2016, with analyses of clinicoradiological aspects and prognoses, and with available and complete follow-up until 31 March 2019. Results: From a total of 147 brain gliomas, a molecular diagnosis and a diagnostic re-evaluation were carried out in 69 cases (grade II-IV astrocytomas or oligodendrogliomas). Twenty-four reclassified gliomas were detected, usually oligodendrogliomas that became astrocytomas, and which showed greater survival, derived from their not being classified as grade IV. The reclassified gliomas, all grades II/III, mostly began with seizures, without focus, with single lesions, <17 cm3 and with oedema, although with similar survival rates. The prognostic factors were: young age, focus, grade II and no contrast enhancement or necrosis, or multiplicity. No variations were detected according to the molecular pattern with IDH mutation or codeletion. Conclusion: The changes in diagnosis after the WHO classification of 2016 present specific clinical-radiological characteristics in this series, but no greater survival, although, due to the habitual survival in these cases, they would require a longer follow-up time

[Mucin-producing urothelial type prostatic adenocarcinoma with signet ring cells and increased Ca 19.9 and Cea. Case report and literature review.] / Adenocarcinoma mucinoso de próstata tipo urotelial con células en anillo de sello y elevación del Ca 19.9 y Cea. Revisión de la literatura a propósito de un caso.

OBJECTIVE: Primary Mucin-producing Urothelial-type Adenocarcinoma of Prostate is extremely infrequent. The presence of signet ring cells is exceptional, more atypical in its mucinous variant. Anatomopathological and immunohistochemical study play a key role. METHODS: Bibliographic review and case report of a 66-year-old man with Ca 19.9 and CEA elevation, and normal PSA levels, associated with lower urinary tract symptoms (mucosuria, hesitancy and hematuria). He was diagnosed with mucin-producing urothelial- type adenocarcinoma of the prostate with signet ring cells by transrectal prostate biopsy after multiparametic MRI. RESULTS: We found 23 cases described in our review. No case diagnosed following an elevation of Ca 19.9 was found in the literature. In our case, after diagnosis, he was treated with retropubic radical prostatectomy and bilateral ilio-obturator lymph node dissection, with subsequent normalization of tumor markers; however, he presented secondary pulmonary involvement and pelvic tumor progression despite chemotherapy treatment. CONCLUSIONS: The elevation of associated tumor markers (Ca 19.9, CEA) is extraordinary. There is no treatment algorithm, however surgery (radical prostatectomy) with or without adjuvant chemotherapy treatment represents an alternative in its therapeutic management.

OBJETIVO: El adenocarcinoma primario de próstata de tipo urotelial es extremadamente infrecuente. La presencia de células en anillo de sello es excepcional, siendo más atípica aún en su variante mucinosa. Su estudio anatomopatológico e inmunohistoquímico juegan un papel fundamental.MÉTODOS: Revisión de la literatura a propósito del caso de un varón de 66 años con elevación de Ca 19.9 y CEA, y niveles de PSA normales, asociado a sintomatología del tracto urinario inferior (mucosuria, estranguria y hematuria) diagnosticado mediante biopsia prostática transrectal tras RMN multiparamétrica de un adenocarcinoma mucinoso de próstata tipo urotelial con células en anillo de sello. RESULTADOS: En la revisión efectuada se han encontrado descritos 23 casos. No se ha encontrado en la literatura ningún caso diagnosticado a raíz de una elevación del Ca 19.9. En nuestro caso, tras el diagnóstico fue tratado mediante prostatectomía radical retropúbica con linfadenectomía ilio-obturatriz bilateral, con normalización posterior de los marcadores tumorales; sin embargo, presentó afectación secundaria pulmonar y progresión tumoral pélvica a pesar de tratamiento quimioterápico. CONCLUSIONES: La elevación de marcadores tumorales asociada (Ca 19.9, CEA) es extraordinaria en este tipo de tumores. No existe un algoritmo de tratamiento, sin embargo la cirugía (prostatectomía radical) con o sin tratamiento adyuvante quimioterápico representa una alternativa en su manejo terapéutico.

Adenocarcinoma mucinoso de próstata tipo urotelial con células en anillo de sello y elevación del Ca 19.9 y Cea. Revisión de la literatura a propósito de un caso / Mucin-producing urothelial type prostatic adenocarcinoma with signet ring cells and increased Ca 19.9 and Cea. Case report and literature review

Objetivo: El adenocarcinoma primario de próstata de tipo urotelial es extremadamente infrecuente. La presencia de células en anillo de sello es excepcional, siendo más atípica aún en su variante mucinosa. Su estudio anatomopatológico e inmunohistoquímico juegan un papel fundamental. Métodos: Revisión de la literatura a propósito del caso de un varón de 66 años con elevación de Ca 19.9 y CEA, y niveles de PSA normales, asociado a sintomatología del tracto urinario inferior (mucosuria, estranguria y hematuria) diagnosticado mediante biopsia prostática transrectal tras RMN multiparamétrica de un adenocarcinoma mucinoso de próstata tipo urotelial con células en anillo de sello. Resultados: En la revisión efectuada se han encontrado descritos 23 casos. No se ha encontrado en la literatura ningún caso diagnosticado a raíz de una elevación del Ca 19.9. En nuestro caso, tras el diagnóstico fue tratado mediante prostatectomía radical retropúbica con linfadenectomía ilio-obturatriz bilateral, con normalización posterior de los marcadores tumorales; sin embargo, presentó afectación secundaria pulmonar y progresión tumoral pélvica a pesar de tratamiento quimioterápico. Conclusiones: La elevación de marcadores tumorales asociada (Ca 19.9, CEA) es extraordinaria en este tipo de tumores. No existe un algoritmo de tratamiento, sin embargo la cirugía (prostatectomía radical) con o sin tratamiento adyuvante quimioterápico representa una alternativa en su manejo terapéutico

Objective: Primary Mucin-producing Urothelial-type Adenocarcinoma of Prostate is extremely infrequent. The presence of signet ring cells is exceptional, more atypical in its mucinous variant. Anatomopathological and immunohistochemical study play a key role. Methods: Bibliographic review and case report of a 66-year-old man with Ca 19.9 and CEA elevation, and normal PSA levels, associated with lower urinary tract symptoms (mucosuria, hesitancy and hematuria). He was diagnosed with mucin-producing urothelial-type adenocarcinoma of the prostate with signet ring cells by transrectal prostate biopsy after multiparametic MRI. Results: We found 23 cases described in our review. No case diagnosed following an elevation of Ca 19.9 was found in the literature. In our case, after diagnosis, he was treated with retropubic radical prostatectomy and bilateral ilio-obturator lymph node dissection, with subsequent normalization of tumor markers; however, he presented secondary pulmonary involvement and pelvic tumor progression despite chemotherapy treatment. Conclusions: The elevation of associated tumor markers (Ca 19.9, CEA) is extraordinary. There is no treatment algorithm, however surgery (radical prostatectomy) with or without adjuvant chemotherapy treatment represents an alternative in its therapeutic management

Late recurrence of medulloblastoma after 17 years of complete remission.

BACKGROUND: To describe an exceptional case of late recurrence of medulloblastoma after 17 years of complete remission. CASE DESCRIPTION: A 42-year-old male consulted in ER for 10-day occipital headache. He had a previous history of cerebellar medulloblastoma 17 years ago treated with gross total resection, chemotherapy and radiotherapy. During his yearly follow-up he had maintained complete remission. MRi showed a cerebellar mass suggestive of medulloblastoma recurrence vs radio-induced tumor. Craniotomy and complete resection of the tumor was performed. The anatomopathological analysis confirmed the recurrence of medulloblastoma. The patient received high dose of adjuvant chemotherapy and he maintains complete remission after 18 months. CONCLUSION: Recurrence of medulloblastoma may occur despite more than 15 years of complete remission. Because of this fact it is mandatory to continue the follow-up of these patients. Aggressive management of recurrence is recommended in absence of disease dissemination.

Evaluación del impacto del cambio diagnóstico de los gliomas aplicando la nueva clasificación de la OMS de 2016 sobre una serie de casos / Assessment of the impact of glioma diagnostic reclassification following the new 2016 WHO classification on a series of cases

Antecedentes y objetivo: El objetivo de este trabajo es evaluar el cambio del diagnóstico molecular sobre el histológico de una serie de tumores gliales al revisar el diagnóstico con la clasificación de la OMS de 2016. Materiales y métodos: Se realiza un estudio retrospectivo de los tumores gliales (oligodendrogliomas y astrocitomas) tratados en nuestro centro entre enero de 2012 y junio de 2016, y una revisión diagnóstica según su estudio molecular. Se lleva a cabo el análisis estadístico de variables epidemiológicas, histológicas y de genética molecular (mutaciones en IDH y presencia de codeleción 1p19q), variación en el diagnóstico al introducir la nueva clasificación tumoral e impacto clínico de dicha reclasificación. Resultados: De los 147 casos de tumores gliales revisados, se obtuvo el diagnóstico molecular en 74 casos (50,3%). En 23 casos (31%) cambió el diagnóstico, predominando en 20 (87%) el diagnóstico previo de oligodendroglioma (69,6% grado II y 17,4% grado III). Solo 3 de los 23 casos cambiaron de diagnóstico inicial astrocitario al oligodendroglial. Respecto al patrón molecular en estos 23 casos, se detectó IDH mutado en 16 (69,6%) y codeleción 1p19q negativa en 20 (87%). Según la estirpe celular, de los 27 oligodendrogliomas de esta serie, 20 (74%) cambiaron de diagnóstico por tener la codeleción negativa, pasando a ser astrocitomas. Se observó una tendencia a un mayor cambio de diagnóstico en pacientes jóvenes (<40 años), p=0,065, mayoritariamente con diagnóstico previo de oligodendrogliomas, sin relación con el sexo. Además, se detectó una mayor frecuencia de cambio de diagnóstico entre los tumores con IDH mutado (69,6%), p=0,003. Respecto a la supervivencia o el patrón clínico, no se detectaron cambios significativos entre los tumores con o sin cambio diagnóstico, a pesar de no recibir tratamiento de elección, tras un seguimiento medio de 16 meses, en probable relación con el bajo grado lesional. Conclusiones: Dentro del espectro de tumores astrocitarios y oligodendrogliales en nuestro centro, la clasificación diagnóstica con genética molecular evidencia importantes cambios respecto al diagnóstico morfológico. Estos cambios afectan especialmente a los diagnósticos previos de oligodendrogliomas y a los pacientes jóvenes en los casos revisados, y con patrones moleculares de mutación en la IDH y de ausencia de codeleción 1p19q. Si bien se pueden plantear dudas respecto a la clínica, el pronóstico y el tratamiento realizado en estos casos, se requieren estudios específicos en estos aspectos para lograr unas conclusiones apropiadas

Background and objectives: The aim of this project is to assess diagnostic reclassification based on molecular data over morphology in a series of glial tumours since the introduction of the 2016 WHO classification of brain tumours. Materials and methods: Retrospective review of glial tumours (oligodendrogliomas and astrocytomas) treated in our centre between January 2012 and June 2016 in which a review of diagnosis was performed when molecular studies were added. Statistical analysis included evaluation of variables of epidemiology, morphology and molecular data (mainly IDH mutation and 1p19q codeletion), diagnostic changes after new classification was considered, and clinical impact in cases of diagnostic reclassification. Results: From a total of 147 glial tumours reviewed in our centre, molecular diagnosis was obtained in 74 cases (50.3%). Initial diagnosis changed in 23 cases (31%), and 20 (87%) of them had a prior histological diagnosis of oligodendroglioma (69.6% grade II - and 17.4% grade III). Only 3 of these 23 cases diagnosis changed from astrocytoma to oligodendroglioma. Among reclassified tumours, there was a common molecular pattern, as findings showed mutant IDH in 16 cases (69.6%) and no codeletion in 20 cases (87%). According to the cell of origin, of the whole group of 27 oligodendrogliomas in our series (reclassified and non-reclassifed), 20 cases (74%) became astrocytomas, despite typical oligodendroglial morphology, due to absence of 1p19q codeletion. There was a trend for diagnosis reclassification in younger patients (<40 years), P=.065, mainly in those with a prior diagnosis of oligodendroglioma, with no statistical differences based on gender or clinical data. Besides, reclassification was more common among tumours with mutant IDH (69.6%), P=.003, than those with wild type IDH. In terms of survival, despite receiving different treatments, no significant changes were detected between reclassified and non-reclassified tumours after a mean follow-up of 16 months, partly related to lower grade of these lesions. Conclusions: Within the spectrum of the glial tumours treated in our institution, this new classification including molecular genetics over morphological data has provided marked diagnostic changes. These changes appear mainly in tumours previously diagnosed as oligodendrogliomas and in younger patients, with molecular patterns of mutant IDH and 1p19q codeletion. Although diagnosis reclassification may affect clinic, prognosis or therapeutic management of these tumours, deeper and prospective studies on these specific aspects are needed

Assessment of the impact of glioma diagnostic reclassification following the new 2016 WHO classification on a series of cases. / Evaluación del impacto del cambio diagnóstico de los gliomas aplicando la nueva clasificación de la OMS de 2016 sobre una serie de casos.

BACKGROUND AND OBJECTIVES: The aim of this project is to assess diagnostic reclassification based on molecular data over morphology in a series of glial tumours since the introduction of the 2016 WHO classification of brain tumours. MATERIALS AND METHODS: Retrospective review of glial tumours (oligodendrogliomas and astrocytomas) treated in our centre between January 2012 and June 2016 in which a review of diagnosis was performed when molecular studies were added. Statistical analysis included evaluation of variables of epidemiology, morphology and molecular data (mainly IDH mutation and 1p19q codeletion), diagnostic changes after new classification was considered, and clinical impact in cases of diagnostic reclassification. RESULTS: From a total of 147 glial tumours reviewed in our centre, molecular diagnosis was obtained in 74 cases (50.3%). Initial diagnosis changed in 23 cases (31%), and 20 (87%) of them had a prior histological diagnosis of oligodendroglioma (69.6% grade ii and 17.4% grade iii). Only 3 of these 23 cases diagnosis changed from astrocytoma to oligodendroglioma. Among reclassified tumours, there was a common molecular pattern, as findings showed mutant IDH in 16 cases (69.6%) and no codeletion in 20 cases (87%). According to the cell of origin, of the whole group of 27 oligodendrogliomas in our series (reclassified and non-reclassifed), 20 cases (74%) became astrocytomas, despite typical oligodendroglial morphology, due to absence of 1p19q codeletion. There was a trend for diagnosis reclassification in younger patients (<40 years), P=.065, mainly in those with a prior diagnosis of oligodendroglioma, with no statistical differences based on gender or clinical data. Besides, reclassification was more common among tumours with mutant IDH (69.6%), P=.003, than those with wild type IDH. In terms of survival, despite receiving different treatments, no significant changes were detected between reclassified and non-reclassified tumours after a mean follow-up of 16 months, partly related to lower grade of these lesions. CONCLUSIONS: Within the spectrum of the glial tumours treated in our institution, this new classification including molecular genetics over morphological data has provided marked diagnostic changes. These changes appear mainly in tumours previously diagnosed as oligodendrogliomas and in younger patients, with molecular patterns of mutant IDH and 1p19q codeletion. Although diagnosis reclassification may affect clinic, prognosis or therapeutic management of these tumours, deeper and prospective studies on these specific aspects are needed.

Misdiagnosis of posterior sequestered lumbar disc herniation: report of three cases and review of the literature.

INTRODUCTION: Posterior migration of sequestered disc is an extremely rare event that mimics more common spinal lesions as spinal tumors, making difficult its preoperative diagnosis and appropriate management. We retrospectively reviewed all lumbar disc herniations treated by surgery at our institution from 2006 to 2016 to identify cases with posterior sequestered disc fragments and possible misdiagnosis for other spinal lesions. Complementarily, a literature review of misdiagnosed cases of posterior migrated discs was undertaken. CASE REPORT: Three posterior sequestered lumbar disc cases (one intradural), were found among the 1153 reviewed surgeries. Two of them, presenting with progressive neurological deficit, were respectively misdiagnosed as pseudotumoral lesion and meningioma/neurogenic tumor on MRI. After intraoperative diagnosis and emergent resection, histology confirmed intervertebral disc tissue. The remaining case had an accurate preoperative diagnosis and after an initial conservative management finally underwent surgery because of refractory pain. Full recovery was achieved months after surgical treatment in all cases. DISCUSSION: Non-tumoral lesions are the most frequent misdiagnosis of posterior sequestered lumbar disc described in the literature. Early surgical treatment is the standard management due to high incidence of cauda equine syndrome (CES); however, spontaneous regression of posterior sequestered lumbar disc herniations has been recently reported. In conclusion low incidence and similar clinical and radiological features with other more common posterior spinal lesions like hematomas, synovial cyst or abscess turns posterior sequestered disc herniations a diagnosis challenge. Despite high incidence of CES, an initial conservative management should be evaluated in selected patients without neurological deficit and well-controlled pain.

Multinodular and vacuolating neuronal tumor of the cerebrum. A rare entity. New case and review of the literature / Tumor neuronal multinodular y vacuolizante cerebral, una entidad rara. Descripción de un nuevo caso y revisión sistemática de la literatura

Background: Multinodular and vacuolating neuronal tumor has been recently described and included in the World Health Organization Classification of Tumors of The Central Nervous System, even though its consideration as a true tumor is controversial. Patients with these lesions usually present with refractory seizures and inconclusive imaging findings that may be confused with other more common diagnoses such as dysembryoplastic neuroepithelial tumors or low-grade gliomas. Therefore, surgical resection is warranted to reach a pathologic diagnosis and seizure control. To the best of our knowledge, only 16 cases have been published in the English literature. Case description: We present the case of a 52-year-old male who presented at our institution with a 2-year-history of absence of seizures. Brain MRI showed a T2-hyperintense lesion with no contrast enhancement affecting his temporal lobe. Temporal craniotomy and microsurgical resection was scheduled. The procedure was uneventful and a grayish, gluey mass was sent for pathologic analysis. The tumor was formed by immature neuronal cells organized in nodules with a vacuolated matrix. A thorough immunohistochemical analysis showed positivity for: Protein Gene Product 9.5. ATRX. OLIG2. SOX10. p16. Nestin. Synaptophysin. The findings were consistent with multinodular and vacuolating neuronal tumor. The patient has been seizure-free after surgery and with no signs of tumor progression. Conclusion: We present a thorough review addressing this uncommon tumor along with a description of the 17th reported case of MVNT, a tumor that was described for the first time in 2013. Further studies and case studies are necessary to establish a well-defined morphological and immunohistochemical profile along with knowledge about its natural history

Antecedentes: El tumor multinodular y vacuolizante cerebral se ha descrito recientemente y ha sido incluido en la clasificación de los tumores del sistema nervioso central de la Organización Mundial de la Salud. No obstante, cabe destacar que su catalogación como «verdadero tumor» es controvertida. Los pacientes con esta lesión suelen presentar clínica de crisis convulsivas refractarias al tratamiento médico. Dada su rareza y, sobre todo, su reciente descripción y clasificación, este tipo de tumores puede confundirse con entidades más frecuentes, tales como los tumores disembrioplásicos neuroepiteliales o los gliomas de bajo grado. Por consiguiente, la resección quirúrgica es el tratamiento recomendado, dado que con ella se consigue un diagnóstico anatomopatológico y la posibilidad de intentar controlar las crisis convulsivas y la sintomatología derivada del tumor. Solamente 16 casos han sido publicados previamente en la literatura médica en lengua inglesa.Descripción de un caso: Presentamos el caso de un paciente varón de 52 años de edad valorado en nuestro centro por un cuadro de 2 años de evolución de crisis de ausencia. La resonancia magnética cerebral mostró una lesión ocupante de espacio en el lóbulo temporal derecho, hiperintensa en secuencias T2 y FLAIR. La lesión no mostraba realce tras la administración de gadolinio. Se propuso una craneotomía temporal y resección microquirúrgica guiada con neuronavegación. La cirugía transcurrió sin incidencias, consiguiéndose una resección macroscópica total de una lesión gomosa y grisácea. El análisis anatomopatológico describió una lesión de tipo tumoral formada por células neuronales de aspecto inmaduro que se agrupaban en nódulos sobre una matriz con microvacuolizaciones. En el estudio inmunohistoquímico se halló positividad para protein gene product 9.5, ATRX, OLIG2, SOX10, p16, nestina y sinaptofisina. Los hallazgos descritos eran congruentes con un tumor neuronal multinodular y vacuolizante. El paciente está en seguimiento en consultas y libre de crisis, con desaparición de sus síntomas previos y sin aparentes datos clinicorradiológicos de recidiva o progresión de su enfermedad.Conclusiones: Presentamos una revisión sistemática que versa sobre el tumor neuronal multimodular y vacuolizante cerebral, un tumor muy inusual y de reciente publicación. Asimismo, describimos un caso adicional de este tipo de lesión, que resulta ser el decimoséptimo caso descrito en la literatura médica en lengua inglesa. Más series de casos y estudios son necesarios para mejorar la caracterización y definición de estas lesiones, así como para conocer su historia natural, inmunohistoquímica, genética y manejo diagnóstico-terapéutico más adecuado

Multinodular and vacuolating neuronal tumor of the cerebrum. A rare entity. New case and review of the literature.

BACKGROUND: Multinodular and vacuolating neuronal tumor has been recently described and included in the World Health Organization Classification of Tumors of The Central Nervous System, even though its consideration as a true tumor is controversial. Patients with these lesions usually present with refractory seizures and inconclusive imaging findings that may be confused with other more common diagnoses such as dysembryoplastic neuroepithelial tumors or low-grade gliomas. Therefore, surgical resection is warranted to reach a pathologic diagnosis and seizure control. To the best of our knowledge, only 16 cases have been published in the English literature. CASE DESCRIPTION: We present the case of a 52-year-old male who presented at our institution with a 2-year-history of absence of seizures. Brain MRI showed a T2-hyperintense lesion with no contrast enhancement affecting his temporal lobe. Temporal craniotomy and microsurgical resection was scheduled. The procedure was uneventful and a grayish, gluey mass was sent for pathologic analysis. The tumor was formed by immature neuronal cells organized in nodules with a vacuolated matrix. A thorough immunohistochemical analysis showed positivity for: Protein Gene Product 9.5. ATRX. OLIG2. SOX10. p16. Nestin. Synaptophysin. The findings were consistent with multinodular and vacuolating neuronal tumor. The patient has been seizure-free after surgery and with no signs of tumor progression. CONCLUSION: We present a thorough review addressing this uncommon tumor along with a description of the 17th reported case of MVNT, a tumor that was described for the first time in 2013. Further studies and case studies are necessary to establish a well-defined morphological and immunohistochemical profile along with knowledge about its natural history.

Angiocentric Glioma in an Elderly Patient: Case Report and Review of the Literature.

BACKGROUND: Angiocentric glioma is a very uncommon low-grade tumor, predominantly occurring in pediatric patients, that was first described in 2005 and was codified 2 years later as a new central nervous system primary tumor. We herein report an exceptionally rare case of an elderly patient with angiocentric glioma. Only one additional case of angiocentric glioma in a patient older than 65 years has been hitherto reported. CASE DESCRIPTION: An 83-year-old male patient presented at our institution complaining of a 1-month history of progressive weakness of his right hand and difficulty performing fine movements. Magnetic resonance imaging of the brain was performed, and fluid-attenuated inversion recovery and T2-hyperintense diffuse cortico-subcortical lesion were reported. A neuronavigation-guided frontal craniotomy was performed to expose the premotor cortex, motor cortex, Rolandic sulcus, and postcentral gyrus. Intraoperative mapping showed that the tumor was close to the shoulder area. Therefore, only partial resection was safely feasible. Pathology report described astrocytic neoplastic cells affecting mainly the cortex and piamater with the classic finding of subpial palisading, with no endothelial invasion or atypia. Neoplastic cells were positive for glial fibrillary acidic protein, epithelial membrane antigen, Wilms tumor protein-1, P16, and P53. Low proliferative activity was seen (Ki-67 < 2%). Abundant gliovascular structures were also reported. CONCLUSIONS: Considering the morphologic and immunohistochemical data, the final pathologic diagnosis was angiocentric glioma. Furthermore, a thorough review of the literature was performed with the purpose of updating and summarizing the main clinical, radiologic, and pathologic features of this rare tumor.

Expanded Endoscopic Transclival Approach for Resection of a Chordoid Meningioma without Dural Attachment (MWODA) Located in the Prepontine Cistern.

BACKGROUND: Meningiomas without dural attachment (MWODA) located in the posterior fossa are an unfrequent entity. They are usually located in the fourth ventricle, and their occurrence outside of this anatomic structure is an even more uncommon finding. Chordoid meningiomas are a rare subtype of meningioma, and they have been reported to account for 0.5%-1% of all meningiomas. CASE DESCRIPTION: We report the unusual case of a 36-year-old female patient, with unremarkable past medical history, who presented at our institution complaining of acute binocular diplopia. Right cranial nerve VI paresis was observed on physical examination. Imaging studies revealed an intradural retroclival solid mass that enhanced homogeneously after contrast administration. Interestingly, no dural tail was present. Expanded endonasal endoscopic resection of her retroclival lesion was performed. We used a 4-hand technique with 0 and 30 degrees endoscopes, with intradural pituitary transposition. Gross total resection was achieved and the pathology report described findings consistent with chordoid meningioma. The patient is recurrence-free and in good condition at 1-year follow-up. CONCLUSIONS: We performed a thorough review of the literature, and we found 10 reported cases describing extraventricular MWODA in the posterior fossa. To our knowledge, this is the first reported case of retroclival MWODA with pathologic findings consistent with chordoid meningioma.