Pleomorphic Xanthoastrocytoma with Oligodendroglioma-Like Areas with Negative 1p19q Co-Deletion

The most common mixed glioma encountered in routine surgical practice is oligoastrocytoma (OA); however, its is currently considered a vanishing entity. The 2016 classification of the World Health Organization (WHO) discourages the diagnosis of tumors as mixed glioma. The recommendations are that diffuse gliomas, including those withmixed or ambiguous histological features, should be subjected tomolecular testing. Dual-genotype OAs are not yet a distinct entity or variant in the classification. We report a case ofmixed glioma: a pleomorphic xanthoastrocytoma (PXA)mixed with an oligodendroglioma. The immunohistochemistry (IHC) pattern of isocitrate dehydrogenase 1 (IDH1) negativity with retained nuclear expression of the alpha-thalassemia x-linked intellectual disability syndrome (ATRX) protein, and 1p19q co-deletion negativity in both the components enabled its identification as a mixed glioma rather than a collision tumor. To the best of our knowledge, the case herein presented is the fourth case of PXA with oligodendroglioma. Out of the other three reported cases, only one was of a collision tumor with a dual genotype, and the other two showed similar molecular signatures in both components. The present article discusses the histological, immunohistochemical and molecular features of the aforementioned case.

A Diffuse Leptomeningeal Glioneural Tumor Case Producing Hydrocephalus and Polyradiculopathy

The present report describes the case of a male 17-year-old patient who progressively developed a hydrocephalus and polyradiculopathy due to involvement of central nervous system (CNS) by a diffuse leptomeningeal glioneuronal tumor (DLGNT). The tumor had partial remission in response to the treatment with radiotherapy plus procarbazine, lomustine, and vincristine (PCV) chemotherapy, and the patient had improvement in function and pain levels. The current knowledge about DLGNT, including its clinical manifestations, imaging findings, histological characteristics, and treatment are revised and discussed in the present paper.

Oligodendroglioma anaplásico quístico: reporte de caso / Anaplastic cystic oligodendroglioma: case report

Resumen: Los oligodendrogliomas anaplásicos son gliomas infiltrantes grado III de la organización mundial de la salud (OMS). Son tumores poco frecuentes y representan el 5-10% de todas las neoplasias intracraneales primarias. Su incidencia es de 0.3 por 100.000 habitantes por año en Estados Unidos. Con frecuencia se presentan en adultos entre los 40-60 años de edad. Los síntomas principales pueden ser déficit motor, déficit cognitivos y síntomas de aumento de la presión intracraneal. Su comportamiento en resonancia magnética muestra un aspecto heterogéneo con necrosis, degeneración quística y hemorragia intratumoral. Las presentaciones quísticas extensas son poco frecuentes. Reportamos el caso de un oligodendroglioma anaplásico de aspecto predominantemente quístico en una mujer joven.

Abstract: Anaplastic oligodendrogliomas are grade III infiltrating gliomas of the World Health Organization (WHO). They are rare tumors and represent 5-10% of all primary intracranial neoplasms. Its incidence is 0.3 per 100.000 inhabitants per year in the United States. They often occur in adults between 40-60 years of age. The main symptoms may be motor deficit, cognitive deficits and symptoms of increased intracranial pressure. Its behavior in MRI shows a heterogeneous appearance with necrosis, cystic degeneration and intratumoral hemorrhagic. Extensive cystic presentations are rare. We report the case of an anaplastic oligodendroglioma of predominantly cystic appearance in a young woman.

Semi-quantitative evaluation of brain gliomas in adults: A focus on neuropathological characteristics

Introduction: Gliomas are neoplasms with high recurrence and mortality. Due to the difficulty to apply the World Health Organization (2016) classification, developing countries continue to use histological evaluation to diagnose and classify these neoplasms. Objective: To develop a semi-quantitative scale to numerically grade gliomas by its morphological characteristics. Method: A cohort of patients with gliomas was assessed and followed for 36 months. Tumor tissue sections were analyzed and graded, including aspects such as cell line, cellularity, nuclear pleomorphism, mitosis, endothelial hyperplasia, hypoxic changes, apoptotic bodies, necrosis, hemorrhage and proliferation index. Results: 58 cases were analyzed. Low-grade gliomas median score was 12 points (9 and 13.5 for percentiles 25 and 75, respectively), whereas for high-grade gliomas it was 17 points (16 and 20.5 for percentiles 25 and 75, respectively) (p < 0.0001). Thirty-six-month survival of patients with low (13/17) and high grade gliomas (6/41) was also significantly different (p < 0.0001). Conclusions: The semi-quantitative morphological scale allows an objective evaluation of gliomas, with an adequate correlation between the score, tumor grade and survival time.

Oligodendroglioma with Sarcomatous Transformation: Case Report and Literature Review

Oligodendrogliomas are infiltrative tumors of the central nervous systemconsidered to be morphologically stable and to offer a better prognosis. Here, we describe the case of a 36- year-old man with an initial diagnosis of oligodendroglioma, World Health Organization (WHO) grade II, who presented transformation to a sarcomatous form, while maintaining the oligodendroglial component as well as the genetic characteristics of the initial tumor without having undergone any complementary treatments previously. Despite the favorable genetic characteristics, the tumor presented poor response to complementary treatments, and rapid progression, including spinal metastasis.

Dysembryoplastic neuroepithelial tumor originally diagnosed as astrocytoma and oligodendroglioma / Tumor neuroepitelial disembrioplásico diagnosticado originalmente como astrocitoma ou oligodendroglioma

Dysembryoplastic neuroepithelial tumor (DNT), described in 1988 and introduced in the WHO classification in 1993, affects predominantly children or young adults causing intractable complex partial seizures. Since it is benign and treated with surgical resection, its recognition is important. It has similarities with low-grade gliomas and gangliogliomas, which may recur and become malignant. OBJECTIVES: To investigate whether DNT was previously diagnosed as astrocytoma, oligodendroglioma, or ganglioglioma and to determine its frequency in a series of low-grade glial/glio-neuronal tumors. METHODS: Clinical, radiological, and histological aspects of 58 tumors operated from 1978 to 2008, classified as astrocytomas (32, including 8 pilocytic), oligodendrogliomas (12), gangliogliomas (7), and DNT (7), were reviewed. RESULTS: Four new DNT, one operated before 1993, previously classified as astrocytoma (3) and oligodendroglioma (1), were identified. One DNT diagnosed in 2002 was classified once more as angiocentric glioma. Therefore, 10 DNT (17.2%) were identified. CONCLUSIONS: Clinical-radiological and histopathological correlations have contributed to diagnose the DNT.

O tumor neuroepitelial disembrioplásico (DNT), descrito em 1988 e incorporado na classificação da OMS em 1993, acomete predominantemente crianças ou adultos jovens, causando crises convulsivas parciais complexas farmacorresistentes. Como é benigno e tratável com ressecção cirúrgica, seu reconhecimento é importante. Tem semelhanças com gliomas de baixo grau e gangliogliomas, que podem recidivar e malignizar. OBJETIVOS: Investigar se o DNT foi originalmente diagnosticado como astrocitoma, oligodendroglioma ou ganglioglioma e determinar sua frequência numa série de neoplasias gliais/glioneuronais de baixo grau. MÉTODOS: Foram revistos aspectos clínicos, radiológicos e histológicos de 58 neoplasias operadas entre 1978 e 2008, classificadas como astrocitomas (32, sendo 8 pilocíticas), oligodendrogliomas (12), gangliogliomas (7) e DNT (7). RESULTADOS: Foram identificados quatro novos DNT, um operado antes de 1993, originalmente diagnosticado como astrocitoma (3) e oligodendroglioma (1). Um DNT diagnosticado em 2002 foi reclassificado como glioma angiocêntrico. Portanto, 10 DNT (17,2%) foram identificados. CONCLUSÕES: Correlações clínico-radiológicas e histopatológicas contribuíram para o diagnóstico do DNT.

Current concepts in the pathology and genetics of gliomas.

In recent years, there has been a marked improvement in our understanding of molecular genetics of gliomas. These advancements offer hope for development of tailored therapies targeting a tumor's unique molecular profile, and may also translate into improved classification and identification of newer prognostic markers. This review focuses on the neuropathological features of different types of glial neoplasms according to the World Health Organization classification, and the recent advances in their molecular biology with emphasis on the genetic mechanisms underlying tumor progression, diagnostic and prognostic markers and potential therapeutic targets.

Current concepts and newer developments in the treatment of malignant gliomas.

Primary malignant brain tumors account for only 2% of all adult cancers but they cause a disproportionately high cancer-related disability and death. Survival of malignant glioma patients has changed only modestly over the past three decades despite the emergence of new treatment strategies for these tumors. In this review, we describe the standard treatment modalities for malignant glioma, which include surgery, radiation therapy and chemotherapy, as well as the status of novel therapies that have been developed to target various aspects of glioma cell biology. We also address this issue of drug delivery as a factor limiting the efficacy of systemic administration of therapeutics and attempts to overcome this barrier. Further progress towards a cure for malignant gliomas will require a greater understanding of the underlying mechanisms driving the growth, and resistance to therapy, of these challenging tumors.

Imunoistoquímica em oligodendrogliomas / Immunohistochemstry in oligodendrogliomas

Os oligodendrogliomas (OL) são tumores gliais caracterizados histologicamente pela presença de núcleo redondo e homogêneo com halo claro perinuclear. A diferenciação microscópica desses tumores com neurocitoma central, DNT e algumas vezes com ependimoma de células claras pode ser difícil. O estudo imunoistoquímico com marcadores glial e neuronal tem sido utilizado e pode auxiliar no diagnóstico diferencial. O objetivo do presente estudo foi determinar a diferenciação neuronal e glial por meio de técnica imunoistoquímica utilizando anticorpos de rotina em tumores com características microscópicas de OL. Foram estudados 42 pacientes com idade entre 4 e 60 anos. Dez apresentavam sinais de maior malignidade (anaplásico). Trinta e três casos (78,5%) mostraram positividade para GFAP, sendo em 10 focal e 6 casos com expressão intensa. Doze casos (28,5%) apresentaram positividade para NSE e/ou sinaptofisina, demonstrando alguma diferenciação neuronal, principalmente focal. Trinta e quatro casos (80,9%) foram positivos para S-100 e três casos (7,1%) foram positivos focalmente para NeuN. Concluimos que áreas focais de diferenciação neuronal e/ou glial podem estar presente em OL típicos e, portanto, é necessário cautela no diagnóstico diferencial em amostras pequenas de tumor. A positividade difusa para marcadores neuronais deve sugerir o diagnóstico de neurocitoma central.

Oligodendroglioma in a French Bulldog

A 5-year-old, male French bulldog with bradycardia, dyspnea, and decerebrate rigidity was necropsied. Macroscopic findings were restricted to the brain, and a single mass, 1.5x2.0x1.5 cm in size, was observed mainly at the right cingulum with prominently protruding into the dilated right lateral ventricle. The mass was grayish white in color, soft and gelatinous, but not clearly delineated. Microscopically, the mass consisted of diffuse proliferated neoplastic oligodendroglial cells characterized by small, round, and hyperchromatic nuclei with clear cytoplasm and the cells aggressively invaded into the adjacent parenchyma. Immunohistochemistry demonstrated that most of the neoplastic cells were positive for S-100 protein, vimentin, neuron specific enolase (NSE), and neurofilament protein (NFP). From these findings, the mass was diagnosed as oligodendroglioma.

Oligodendroglioma Arising in a Sacrococcygeal Immature Teratoma

Tumors of neuroepithelial origin are extremely rare in teratoma and tend to be derived from glial or primitive neuroectodermal cells. We describe a case of 2- month-old baby girl with an oligodendroglioma arising in an immature teratoma of the sacrococcygeal region. Histologically, the tumor was identical in appearance to low grade oligodendroglioma within the adult brain. Because immature teratoma was grade II, the patient received adjuvant chemotherapy. The patient died of progression of the intra-abdominal tumor 6 months after surgical excision. The authors believe this to be the first presentation in the world literature.

Oligodendrogliomas: estudo anatomopatológico e clínico de 15 casos / Oligodendroglioma: a pathological and clinical study o 15 cases

Oligodendrogliomas correspondem a 4-5 por cento dos tumores primários do sistema nervoso central apresentando crescimento infiltrativo e lento. Relatamos os achados anatomopatológicos e clínicos de 15 casos de oligodendrogliomas. Oito pacientes eram do sexo masculino e 7 do feminino. As idade oscilaram entre 17 e 66 anos, apresentando média de 39,73 anos. A sintomatologia apresentada correspondeu ao crescimento expansivo, sendo cefaléia (60 por cento) e crises convulsivas (60 por cento) os sintomas mais frequentes. O lobo frontal (n=6) foi o sítio anatômico mais acometido, seguido pelo parietal (n=2), temporal (n=1) e occipital (n=1). Cinco pacientes foram submetidos a ressecção total do tumor e 10 pacientes a exerese parcial; dentre estes, 3 foram submetidos a radioterapia adjuvante, 1 a quimioterapia e 1 a quimio e radioterapia. Evidenciou-se taxa de recidiva tumoral total de 60 por cento em período médio de 32 meses de acompanhamento. Cinco recidivas tumorais ocorreram nos pacientes submetidos apenas ao tratamento cirúrgico e quatro nos pacientes submetidos a quimio ou radioterapia adjuvante. Estes achados aproximam-se dos encontrados na literatura, auxiliando na compreensão do comportamento biológico deste raro tumor cerebral.

Anaplastic oligodendroglioma with cervical lymphonode metastasis: report of a case

A case of a patient operated on twice for a temporoparietal anaplastic oligondendroglioma, followed by radiotherapy, is reported. Although no intracranial recurrence has happened, a biopsyproven extracranial cervical lymphnode metastasis was diagnosed 15 months later. Surgical manipulation of the primary lesion by lymphatic and/or blood vessel invasion seem to be the main suspected factors in the development of CNS tumors metastasis in the head and neck region.

Intraventricular neurocytoma: a report of two cases.

Central neurocytomas are uncommon intracranial neoplasms. More than one hundred cases are documentated in the literature. In this report we describe the clinical and histopathological features in two patients with intraventricular neurocytoma. As the light microscopic features of neurocytoma resemble with that of an oligodendroglioma, it is essential to differentiate these two tumours, using either ultrastructural or immunohistochemical techniques.

Oligodendrogliomas: acerca de 33 casos / Oligodendrogliomas: a review of 33 cases

De 1.000 tumores intracraneanos, oligodendroglioma fue el diagnóstico en 20 varones y 13 mujeres (3,3% incidencia). Destacaban 6 niños (de 7 a 15 años) que fueron un 18% de la serie. La epilepsia fue el síntoma inicial en 18 casos que llegaron a tratamiento en un plazo menor que quienes se presentaron con otros síntomas. Los resultados mostraron una mortalidad de 83% a 3 años plazo para 6 pacientes en los que se hizo biopsia y radioterapia. La mortalidad de 5 años plazo mejoró a un 37,5% en 8 pacientes operados con resección parcial y radioterapia y en otros 18 pacientes con resección total o subtotal, más radioterapia, la mortalidad a 5 años fue 27,7%. Los factores que influyen en los resultados parecen ser el tamaño y la localización del tumor, lo que permite diferentes aproximaciones quirúrgicas

Quimioterapia sistêmica para a disseminaçäo intraventricular de oligodendroglioma frontal / Systemic chemotherapy for intraventricular dissemination of frontal oligodendroglioma: report of a case

Recidiva intraventricular de oligodendroglioma frontal foi tratada com sucesso através de quimioterapia sistêmica

Oligodendrogliomas: análise de 24 casos e revisäo de literatura / Oligodendrogliomas: report of 24 cases and review of the literature

Oa autores apresentam 24 casos de doentes portadores de oligodendrogliomas e revisam aspectos relacionados a classificaçäo, quadro clínico, comportamento de oligodendrofliomas e revisam aspectos relacionados a classificaçäo, quadro clínico, comportamento biológico, patologia e tratamento desta neoplasia