Reclassification of Mongolian Diffuse Gliomas According to the Revised 2016 World Health Organization Central Nervous System Tumor Classification

BACKGROUND: The 2016 World Health Organization (WHO) classification of central nervous system (CNS) tumors has been modified to incorporate the IDH mutation and 1p/19q co-deletion in the diagnosis of diffuse gliomas. In this study, we aimed to evaluate the feasibility and prognostic significance of the revised 2016 WHO classification of CNS tumors in Mongolian patients with diffuse gliomas. METHODS: A total of 124 cases of diffuse gliomas were collected, and tissue microarray blocks were made. IDH1 mutation was tested using immunohistochemistry, and 1p/19q co-deletion status was examined using fluorescence in situ hybridization analysis. RESULTS: According to the 2016 WHO classification, 124 cases of diffuse brain glioma were reclassified as follows: 10 oligodendroglioma, IDHmut and 1p/19q co-deleted; three anaplastic oligodendroglioma, IDHmut and 1p/19q co-deleted; 35 diffuse astrocytoma, IDHmut, 11 diffuse astrocytoma, IDHwt, not otherwise specified (NOS); 22 anaplastic astrocytoma, IDHmut, eight anaplastic astrocytoma, IDHwt, NOS; and 35 glioblastoma, IDHwt, NOS, respectively. The 2016 WHO classification presented better prognostic value for overall survival in patients with grade II tumors than traditional histological classification. Among patients with grade II tumors, those with oligodendroglioma IDHmut and 1p/19q co-deleted and diffuse astrocytoma IDHmut showed significantly higher survival than those with diffuse astrocytoma IDHwt, NOS (p<.01). CONCLUSIONS: Mongolian diffuse gliomas could be reclassified according to the new 2016 WHO classification. Reclassification revealed substantial changes in diagnosis of both oligodendroglial and astrocytic entities. We have confirmed that the revised 2016 WHO CNS tumor classification has prognostic significance in Mongolian patients with diffuse gliomas, especially those with grade II tumors.

Nimotuzumab y radioterapia en el tratamiento de tumores del tallo cerebral en niños y adolescentes / Nimotuzumab and radiotherapy in the treatment of brainstem tumors in children and adolescents

Introducción: Los tumores localizados en el tallo cerebral en los niños y adolescentes conllevan un mal pronóstico, especialmente aquellos infiltrantes y difusos. Con el tratamiento de radioterapia apenas llegan a más de 15 por ciento de supervivencia y no mejora la cifra con quimioterapia agregada. Objetivos: Estimar el efecto de la asociación del tratamiento radiante con el anticuerpo monoclonal Nimotuzumab en la supervivencia de niños y adolescentes con tumores del tallo cerebral. Método: Estudio clínico no aleatorizado, analítico, longitudinal y prospectivo. Se estudió una serie de 46 pacientes entre 2 y 18 años de edad que padecían de tumores del tallo cerebral, infiltrantes y difusos, desde enero de 2008 y en seguimiento hasta marzo de 2018. Todos se trataron con radioterapia, con dosis entre 54 y 59,8 cGrey, dosis diaria de 1,8 cGrey, y se irradiaban de lunes a viernes. Mientras duró el tratamiento radiante recibieron Nimotuzumab, en la dosis de 150 mg/m2 de superficie corporal, luego semanal con 8 dosis, y finalmente mensual durante uno o dos años. Resultados: Se alcanzó en la serie una supervivencia media de 18,4 meses, y una esperada de 42,9 por ciento a 2 años y 35,5 por ciento a 5 años, estabilizada hasta los 10 años. Conclusiones: La combinación de radioterapia y el anticuerpo monoclonal Nimotuzumab incrementa la supervivencia en niños y adolescentes con tumores del tallo cerebral y es bien tolerada, aun en periodos prolongados, e incluso en casos de recidiva(AU)

Introduction: Tumors localized in the brainstem of children and adolescents entail a bad prognosis, especially those that are intrinsic and diffuse. With radiotherapy treatment, patients barely get a 15 percent of survival, and the numbers don't improve with added chemotherapy. Objectives: To estimate the effect of the association of radiotherapy treatment with Nimotuzumab monoclonal antibody in the survival of children and adolescents with brainstem tumors. . Method: Non randomized, analytical, longitudinal and prospective clinical study that was authorized by the National Regulatory Authority. There was studied a group of 46 patients aged from 2 to 18 years that suffered from intrinsic and diffuse brainstem tumors, from January 2008 (and in follow up) to March 2018. All the patients were treated with radiotherapy, with doses among 54 and 59,8 Grey, daily doses of 1,8 Grey, and from Monday to Friday. While they were under radiotherapy treatment, they get Nimotuzumab, in doses of 150 mg/m2 of corporal surface; then weekly doses of 8 shots; and finally, monthly doses during one or two years. Results: In this group there was a survival mean of 18, 4 months, and an expected survival of 42, 9 percent for 2 years and 35, 5 percent for 5 years that can be stabilized to 10 years. Conclusions: Combination of radiotherapy and Nimotuzumab monoclal antibody can increase the survival from brainstem tumors in children and adolescents(AU)

Neuropsychiatry: Towards a Philosophy of Praxis / Neuropsiquiatría: hacia una filosofía de la práctica

Abstract Neuropsychiatry is a specialized clinical, academic and scientific discipline with its field located in the borderland territory between neurology and psychiatry. In this article, we approach the theoretical definition of neuropsychiatry, and in order to address the practical aspects of the discipline, we describe the profile of a neuropsychiatric liaison service in the setting of a large hospital for neurological diseases in a middle-income country. An audit of consecutive in-patients requiring neuropsychiatric assessment at the National Institute of Neurology and Neurosurgery of Mexico is reported, comprising a total of 1212 patients. The main neurological diagnoses were brain infections (21%), brain neoplasms (17%), cerebrovascular disease (14%), epilepsy (8%), white matter diseases (5%), peripheral neuropathies (5%), extrapyramidal diseases (4%), ataxia (2%), and traumatic brain injury and related phenomena (1.8%). The most frequent neuropsychiatric diagnoses were delirium (36%), depressive disorders (16.4%), dementia (14%), anxiety disorders (8%), frontal syndromes (5%), adjustment disorders (4%), psychosis (3%), somatoform disorders (3%), and catatonia (3%). The borderland between neurology and psychiatry is a large territory that requires the knowledge and clinical skills of both disciplines, but also the unique expertise acquired in a clinical and academic neuropsychiatry program.

Resumen La neuropsiquiatría es una disciplina médica cuyo campo clínico, académico y científico se localiza en el territorio fronterizo entre la neurología y la psiquiatría. En este artículo se aborda la definición teórica de la neuropsiquiatría y, con el objetivo de tratar los aspectos prácticos de la neuropsiquiatría, se describe el perfil de un servicio de interconsulta neuropsiquiátrica en el contexto de un hospital dedicado a las enfermedades del sistema nervioso, en un país de ingreso económico medio. Se incluyó a 1.212 pacientes evaluados consecutivamente en el Instituto Nacional de Neurología y Neurocirugía de México. Los diagnósticos principales fueron infecciones cerebrales (21%), neoplasias del sistema nervioso (17%), enfermedad cerebrovascular (14%), epilepsia (8%), enfermedades de la sustancia blanca (5%), neuropatías periféricas (5%), enfermedades extrapiramidales (4%), ataxias (2%) y traumatismos craneoencefálicos (1,8%). Los diagnósticos neuropsiquiátricos más frecuentes fueron síndrome confusional (36%), depresión (16,4%), demencia (14%), ansiedad (8%), síndromes frontales (5%), reacciones de ajuste (4%), psicosis (3%), trastornos somatoformes (3%) y catatonia (3%). El territorio fronterizo entre la neurología y la psiquiatría es extenso y requiere los conocimientos y fortalezas de ambas disciplinas, pero también la pericia entrenada mediante un programa clínico, científico y académico especializado.

Tumor Intraventricular de tipo astrocitoma subependimario de células gigantes en paciente con esclerosis tuberosa / Angiotomography in stenosis of the hepatic intraventricular tumor of subependymal giant cell astrocytoma in a patient with tuberous sclerosis

Paciente de sexo femenino, de 3 años de edad, con diagnóstico de esclerosis tuberosa y epilepsia refractaria a los farmacos antiepilépticos, que presenta en la resonancia magnética nuclear del cerebro (Figura1) un proceso ocupativo de aproximadamente 36x25x26 mm a nivel ependimario del asta frontal derecha, del núcleo caudado derecho y de la rodilla de la cápsula interna vecinal, que produce efecto de masa al septum pelucidum desplazándole a la izquierda. Es una imagen compatible con Astrocitoma. (AU)

Congenital hemangioma in spondylocostal dysostosis: a novel association

Abstract Congenital hemangioma is a benign tumor caused by dysfunction in embryogenesis and vasculogenesis, which progresses during fetal life to manifest as fully developed at birth. Although hemangiomas are the most common tumor of infancy, rapidly involuting congenital hemangioma has not been described in spondylocostal dysostosis. I report the novel association of congenital hemangioma and spondylocostal dysostosis in a Mexican newborn female patient with neural tube defects. Given the embryological relationship between skin and nervous system, I surmise that this association is not coincidental. I also propose that these morphologic alterations be incorporated to the spondylocostal dysostosis phenotype and specifically looked for in other affected children, in order to provide appropriate medical management and genetic counseling.

A modified method for locating parapharyngeal space neoplasms on magnetic resonance images: implications for differential diagnosis / 癌症

The parapharyngeal space (PPS) is an inverted pyramid-shaped deep space in the head and neck region, and a variety of tumors, such as salivary gland tumors, neurogenic tumors, nasopharyngeal carcinomas with parapharyngeal invasion, and lymphomas, can be found in this space. The differential diagnosis of PPS tumors remains challenging for radiologists. This study aimed to develop and test a modified method for locating PPS tumors on magnetic resonance (MR) images to improve preoperative differential diagnosis. The new protocol divided the PPS into three compartments: a prestyloid compartment, the carotid sheath, and the areas outside the carotid sheath. PPS tumors were located in these compartments according to the displacements of the tensor veli palatini muscle and the styloid process, with or without blood vessel separations and medial pterygoid invasion. This protocol, as well as a more conventional protocol that is based on displacements of the internal carotid artery (ICA), was used to assess MR images captured from a series of 58 PPS tumors. The consequent distributions of PPS tumor locations determined by both methods were compared. Of all 58 tumors, our new method determined that 57 could be assigned to precise PPS compartments. Nearly all (13/14; 93%) tumors that were located in the pre-styloid compartment were salivary gland tumors. All 15 tumors within the carotid sheath were neurogenic tumors. The vast majority (18/20; 90%) of trans-spatial lesions were malignancies. However, according to the ICA-based method, 28 tumors were located in the pre-styloid compartment, and 24 were located in the post-styloid compartment, leaving 6 tumors that were difficult to locate. Lesions located in both the pre-styloid and the post-styloid compartments comprised various types of tumors. Compared with the conventional ICA-based method, our new method can help radiologists to narrow the differential diagnosis of PPS tumors to specific compartments.

Tumores primarios del sistema nervioso central en personas de 15 años o más / Primary tumors of the central nervous system in people aged 15 years and older

Objetivo general: generar recomendaciones basadas en la mejor evidencia disponible acerca del manejo de personas con tumores primarios del sistema nervioso central o que hayan sido tratados por ello. Objetivos específicos: personas con tumores primarios del sistema nervioso central o que hayan sido tratados por ello, que reciben atención en el nivel secundario y nivel terciario de salud en el sector público y privado de salud. Usuarios de la guía: Médicos Generales, de Urgencia, Neurólogos, Neurocirujanos, Oncólogos, Radioterapeutas, y otros médicos y profesionales que participan en la atención integral de los pacientes con tumores primarios del sistema nervioso central o tratados por ello.

Estudio comparativo: prevalencia patologías bucales en pacientes pediátricos oncológicos 1997-2007 / Comparative study: oral pathologies prevalence in pediatric oncology patients 1997- 2007

Los pacientes pediátricos oncológicos con frecuencia presentan lesiones orales debido a su neoplasia o como efecto colateral del tratamiento. El objetivo de este estudio fue comparar la prevalencia de patologías de la mucosa oral en niños con cáncer que fueron hospitalizados y tratados con quimioterapia en el Hospital Regional de Concepción, en los años 1997 y 2007. Se realizó un estudio descriptivo retrospectivo longitudinal en datas de 148 pacientes (74 cada año) con patologías neoplásicas en tratamiento con quimioterapia (Leucemias, linfomas, tumores del Sistema Nervioso Central y otros), registrando sus datos generales y la patología bucal (mucositis (M), candidiasis (C), lesiones por Virus Herpes tipo 1 (VHS) y síndromes hemorragíparos (H) . Los datos se resumieron en tablas anuales y fueron sometidos a análisis estadísticos. Se encontró una disminución significativa del número de pacientes con patologías bucales en el año 2007 en relación al año 1997 (P<0.05, Tet de Fisher). Además se encontró una tendencia a la baja en los pacientes con candidiasis y con mucositis en el año 2007 en comparación con 1997. Es necesario seguir estudiando medidas para prevenir, diagnosticar y/o tratar tempranamente las patologías orales de los pacientes en tratamiento antineoplásico.

Pediatric oncology patients frequently have oral lesions due to malignancy or as a side effect of treatment. The aim of this study was to compare the prevalence of oral pathologies in oncology patients hospitalized and treated at the Regional Hospital of Concepción, Chile, in the years 1997 and 2007. A retrospective study was carried out in 74 patients each year. Patients suffered from acute lymphoblastic leukemia, acute myeloblastic leukemia, central nervous system tumors, lymphomas and other neoplasms. General data (age, gender, oncologic disease) and presence of oral pathologies (candidiasis, mucositis post-chemotherapy, herpetic lesions and hemorrhage) were obtained from their clinical records. Data was analyzed for statistical differences. A significant reduction in the number of patients with oral pathologies was found in 2007 in comparison to 1997 (P<0.05, Fisher´s test). In addition, candidiasis and oral mucositis showed less prevalence in 2007 as compared to 1997, although no significant differences were found. For the relevance of oral pathologies in the chemotherapy it´s important to continue studies about prevention, early detection and treatment of oral pathologies.

Atlas de mortalidad por cáncer en colombia 2010 / Atlas of cancer mortality in colombia 2010

El Instituto Nacional de Cancerología (INC), E. S. E., como ente asesor del Ministerio de la Protección Social, presenta la tercera edición del Atlas de mortalidad por cáncer en Colombia, que en esta oportunidad contempla el periodo 2000-2006. Este trabajo hace parte de la difusión periódica que hace el INC de la información producto de la vigilancia epidemiológica del cáncer en el país. La primera edición del Atlas de mortalidad fue publicada en 1994, con información correspondiente al periodo 1989-1991, donde se representó la cartografía de la mortalidad de cáncer en el país, por grandes regiones y departamentos. La segunda edición contempló información del periodo 1990-1996, y se desarrolló con la colaboración del Instituto Geográfico Agustín Codazzi (IGAC); en esa oportunidad se incorporaron aspectos novedosos desde el punto de vista metodológico, lo que permitió representar mapas temáticos de área pequeña, además de mapas a nivel departamental y mapas de tendencias. Esta nueva edición proporciona información para el periodo 2000-2006, con una metodología y una presentación similares a las de la edición anterior, con el objeto de facilitar la comparación entre ambas publicaciones. Un aspecto nuevo que se introdujo en esta entrega es que se presenta, en las tablas, la información de tendencias de mortalidad para dos periodos: 2000-2006 y 1998-2006. Esto permite al lector comparar el comportamiento de la mortalidad en un periodo corto y en un periodo largo, lo que para temas como el cáncer tiene particular relevancia. Al igual que la edición anterior, este trabajo obedece al esfuerzo coordinado con el IGAC, entidad encargada de producir la cartografía básica de Colombia. El insumo fundamental para su elaboración proviene del Departamento Administrativo Nacional de Estadística (DANE), en sus programas de estadísticas vitales, censos y proyecciones de población. Esperamos, una vez más, que el aporte realizado con la presente publicación constituya un elemento central para todas aquellas personas comprometidas con el control del cáncer en Colombia. Es nuestro deseo que esta publicación esté disponible para los tomadores de decisiones en salud en distintos ámbitos, así como para la comunidad científica, y que la información contenida en ella sea de valiosa ayuda no sólo para avanzar en el entendimiento sobre cómo se comporta cáncer en el país, sino, también, para orientar y evaluar las acciones departamentales y de país en el control de la enfermedad.

Incidencia estimada y mortalidad por cáncer en Colombia 2002-2006 / Estimated incidence and mortality from cancer in Colombia 2002-2006

El Instituto Nacional de Cancerología, como ente asesor del Ministerio de la Protección Social, presenta la segunda edición de la publicación sobre incidencia y mortalidad por cáncer en Colombia, con estimaciones departamentales para el periodo 2002-2006. Este esfuerzo corresponde al ejercicio que adelanta el Instituto en la difusión periódica de la información proveniente de la vigilancia epidemiológica del cáncer en el país. La anterior edición de la incidencia estimada y mortalidad por cáncer en Colombia, 1995-1999 fue realizada por la Subdirección de Investigaciones del Instituto Nacional de Cancerología en el 2005, con la colaboración de la Agencia Internacional para la Investigación en Cáncer (IARC), con el fin de proveer información de cáncer a escala departamental, como base para los tomadores de decisiones y planificadores en salud del país. Esta nueva edición proporciona información discriminada por departamentos, sexo, tipo de cáncer para el periodo 2002-2006, con una evaluación previa de las fuentes de información. En las tablas se presentan los casos nuevos anuales de cáncer y las muertes para el periodo con las correspondientes tasas crudas y ajustadas por edad, que dan cuenta de los patrones de comportamiento del cáncer en el orden departamental y nacional. Las tablas con información consolidada de incidencia para el periodo estarán disponibles en la página web del instituto.

Schwannoma: a propósito de un caso / Schwannoma: with regard to a case

El schwannoma es un tumor benigno de las vainas nerviosas, de crecimiento lento y doloroso. Muestra predilección por las extremidades y su localización en piel es infrecuente. Describimos el caso de una paciente de 16 años de edad, de sexo femenino, con diagnóstico de schwannoma en el tercer dedo de la mano izquierda y realizamos una revisión de la literatura.

Suprasellar germ cell tumor in a dog

A case of suprasellar germ cell tumor is described in a 6-year -old Airedale Terrier bitch that presented behavioral changes and abrupt onset of blindness. The neoplasm compressed the ventral surface of the cerebrum from the level of basal ganglia to the mesencephalon. Histologically the neoplasm consisted of nests and trabeculae of round to polygonal cells that occasionally surround tubules and cysts formed by columnar cells. Neoplastic cells are immunopositive for cytokeratin and alpha-fetoprotein. The diagnosis was based on location, histological appearance and on the immunohistochemical staining.

É descrito um caso de tumor de células germinativas supra-selar em numa cadela Airedale Terrier de seis anos de idade, que apresentou transtornos do comportamento e aparecimento abrupto de cegueira. O neoplasma comprimia a superfície ventral do cérebro desde a altura dos núcleos basais até o mesencéfalo. Histologicamente, o neoplasma consistia de ninhos e trabéculas de células redondas ou poligonais que ocasionalmente arranjavam-se ao redor de túbulos e cistos formados por células colunares. As células neoplásicas foram positivas na imunoistoquímica para citoqueratina e a-fetoproteína. O diagnóstico foi feito com base na localização do tumor, no seu aspecto histológico e nos resultados da marcação imunoistoquímica.

Bevacizumab en gliomas de alto grado recurrentes: reporte de un caso y revisión de la literatura / Bevacizumab in High Grade Gliomas: A Case Report and Review of the Literature

Los pacientes con astrocitoma anaplásico o glioblastoma (GB) presentan, en general, un pronóstico adverso. En la actualidad, el tratamiento estándar de los tumores gliales de alto grado consiste en el uso concomitante de radioterapia más temozolamida (TMZ), seguida de seismeses del agente alquilante. No obstante, los sujetos con tumores diagnosticados de novo o recurrentes tienen una supervivencia global que oscila entre los 3 y los 15 meses. Se han utilizado múltiples agentes quimioterapéuticos y biológicos para controlar la progresión de los gliomas, sin lograr mejorar la supervivencia, por estos motivos nos enfrentamos al desarrollo de nuevos medicamentos dirigidos contra blancos moleculares, entre ellos, el factor de crecimiento vascular endotelial (VEGF, por sus siglas en inglés vascular endothelial growth factor), que puede ser regulado, entre otros, por el bevacizumab (BEV). Recientemente se publicaron los resultados de un estudiofase II que demostró la eficacia de la combinación de BEV más irinotecan (CPT-11) en pacientes con gliomas de alto grado, al mejorar las tasas de respuesta, la supervivencia libre de progresión y global. A continuación se presenta el caso de una mujer con GB recurrente que logró respuesta completa después de iniciar el tratamiento con BEV/CPT-11.

Expression of motilin and its precursor mRNA in normal parenchyma, benign and malignant tumors of human thyroid / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate the expression of motilin and its precursor mRNA in normal human thyroid. To compare the expression differences of motilin and it precursor mRNA between normal thyroid and intestines. To study the expression of motilin and its precursor mRNA in human thyroid tumors and their clinical implications.</p><p><b>METHODS</b>RT-PCR, Southern blot and molecular cloning were used to detect motilin transcript expression in human thyroid and mucous membrane of small intestine. Real-time PCR and immunohistochemical techniques were used to quantify motilin precursor mRNA and motilin peptide in thyroid tissue samples including adenoma, medullary carcinoma, follicular carcinoma, papillary carcinoma and nodular goiter.</p><p><b>RESULTS</b>(1) The expression of motilin and its precursor mRNA in normal human thyroid was primarily in the thyroid C cells. (2) RT-PCR and Southern blot showed that motilin mRNA expressed in human thyroid was identical to that expressed in duodenum with identical sequence deposited in NCBI Genbank of America. (3) Immunohistochemistry, Western blot research and real-time PCR studies showed that motilin and its precursor mRNA were expressed in normal and tumor tissues of human thyroid. Thyroid tumors (acidophilic adenoma, medullary carcinoma, follicular carcinoma, papillary carcinoma and nodular goiter) showed intense and diffuse immunostaining for motilin peptide. Moreover, the expression of motilin and its precursor mRNA in thyroid medullar carcinoma and acidophilic adenoma were significantly higher than those of normal thyroid tissue (P < 0.05). The expression in thyroid follicular and papillary carcinomas were significantly lower than those of normal thyroid tissue (P < 0.05). There was no difference of the expression between nodular goiter and normal thyroid tissue (P > 0.05).</p><p><b>CONCLUSIONS</b>Motilin peptide and its precursor mRNA are expressed in C cells of human thyroid. The sequence of motilin is identical to that expressed in duodenum from NCBI Genbank of America. The expressions of both motilin and its precursor mRNA in thyroid medullary carcinoma and acidophilic adenoma are significantly increased. In contrast, their expressions in thyroid follicular and papillary carcinomas are significantly decreased. Motilin may regulate physiological functions of the thyroid through parafollicular cells. Motilin may be involved in the pathogenesis of medullary carcinoma and acidophilic adenoma of the thyroid.</p>

Mutational analysis of genes p14ARF, p15INK4b, p16INK4a, and PTEN in human nervous system tumors

The cancer is one of the most common and severe problems in clinical medicine, and nervous system tumors represent about 2% of the types of cancer. The central role of the nervous system in the maintenance of vital activities and the functional consequences of the loss of neurons can explain how severe brain cancers are. The cell cycle is a highly complex process, with a wide number of regulatory proteins involved, and such proteins can suffer alterations that transform normal cells into malignant ones. The INK4 family members (CDK inhibitors) are the cell cycle regulators that block the progression of the cycle through the R point, causing an arrest in G1 stage. The p14ARF (alternative reading frame) gene is a tumor suppressor that inhibits p53 degradation during the progression of the cell cycle. The PTEN gene is related to the induction of growth suppression through cell cycle arrest, to apoptosis and to the inhibition of cell adhesion and migration. The purpose of the present study was to assess the mutational state of the genes p14ARF, p15INK4b, p16INK4a, and PTEN in 64 human nervous system tumor samples. Homozygous deletions were found in exon 2 of the p15INK4b gene and exon 3 of the p16INK4a gene in two schwannomas. Three samples showed a guanine deletion (63 codon) which led to a loss of heterozygosity in the p15 gene, and no alterations could be seen in the PTEN gene. Although the group of patients was heterogeneous, our results are in accordance with other different studies that indicate that homozygous deletion and loss of heterozygosity in the INK4 family members are frequently observed in nervous system tumors.

Cryptococcus gattii fungemia: report of a case with lung and brain lesions mimicking radiological features of malignancy

A 64-year-old apparently immunocompetent white man developed lung and brain lesions of disseminated cryptococcosis. The radiologic features mimicked those of lung cancer metastatic to the central nervous system. C. gattii was recovered from cultures of bronchoalveolar lavage fluid, brain biopsy, and blood. The same fungus was recovered from pulmonary and brain specimens at autopsy. Serum and cerebrospinal fluid cryptococcal antigen tests were diagnostic in our case and should be included in the diagnostic evaluation of unexplained pulmonary and cerebral lesions. A literature search showed few reports of fungemia by this species of Cryptococcus, contrasting to C. neoformans.

Homem branco de 64 anos, aparentemente imunocompetente, desenvolveu lesões pulmonares e cerebrais por criptococose disseminada. Os achados radiológicos foram similares àqueles encontrados em pacientes com câncer de pulmão e metástase no sistema nervoso central. C. gattii foi isolado de cultivos de lavado broncoalveolar, biópsia cerebral e sangue. O mesmo fungo foi encontrado em fragmentos pulmonares e cerebrais obtidos da autópsia. Testes de antígeno no soro e no líquido cefalorraquidiano foram diagnóstico no nosso caso e devem ser incluídos na avaliação diagnóstica de lesões pulmonares e cerebrais indefinidas. Pesquisa na literatura mostrou poucos relatos de fungemia por esta espécie de Cryptococcus, contrastando com C. neoformans.

A Case of Early Gastric Adenocarcinoma and Intraabdominal Schwannoma in a Patient with Neurofibromatosis Type I / 대한소화기내시경학회지

Neurofibromatosis is an autosomal dominant hereditary disorder with an overall incidence of one in 3,000~4,000, and type 1 (Von Recklinghausen's neurofibromatosis) characterized by the presence of multiple cutaneous neurofibromas, axillary and groin freckling, and cafe- au-lait spot. The neurofibromatosis type 1 gene is a tumor suppressor gene. Patients with the neurofibromatosis type 1 are at increased risk of developing nervous system neoplasm, including plexiform neurofibromas, optic gliomas, ependymomas, meningiomas, astrocytomas, and pheochromocytomas. Neurofibromas may undergo secondary malignant degeneration and sarcomatous changes. Patients with neurofibromatosis type 1 show a high incidence of Wilm's tumor, rhabdomyosarcoma, nonlymphocytic leukemia, and pheochromocytoma but the gastrointestinal involvement appears to be relatively rare and usually consists of neurofibroma, ganglioneuroma, and leiomyoma. We have identified a case of early gastric adenocarcinoma and intraabdominal schwannoma in a 65-year-old man afflicted with neurofibromatosis type 1.

A Case of Rhabdoid Meningioma

Rhabdoid meningioma is an uncommon meningioma variant, which was initially described in 1998 by Perry et al.. It is classified as a variant corresponding to grade III in the recent World Health Organization(WHO) classification of tumors of the nervous system, because of its unique histopathological features and its highly aggressive clinical course. To date there were still a lot of arguments about their developmental patterns and the treatment strategy especially for rhabdoid meningiomas lacking other histological features of malignancy. Although the exact causes of rhabdoid transformation of a benign meningioma are unknown, malignant transformation long duration after surgical resection under an underlying predilection for malignancy is suggested in our case. And we suggest that any histological rhabdoid features in recurrent meningiomas or even in primary cases seem to indicate the malignant clinical course and the need for aggressive treatments, because transformation from a benign or atypical one to a malignant one seems to occur at last.

Neurogenic mediastinal tumours, a ten-year experience

Neurogenic tumours represent 20% of mediastinal tumors in adults and 35% of such tumours in children; as shown in most of previous studies. We reviewed the patients in whom we found surgical mediastinal tumours in our unit from 1[st] May, 1995 through June 2005. The aim is to evaluate management of cases proved to be neurogenic mediastinal tumours. During the last ten years, we admitted into our unit 132 patients with surgical mediastinal tumours. Full investigations including CT-scanning with contrast and CT- guided biopsy or ultrasound guided biopsy were performed. Thirty-three patients were proved to have neurogenic tumours. From these 132 patients with surgically-treatable mediastinal masses, we found 33 cases of neurogenic pathology [25% are neurogenic tumours]. All neurogenic tumours were in the posterior mediastinum, and represent 75% of all posterior mediastinal masses. There were 29 adults and 6 children [aged <16 years] Mean age was 36.4 years ranging from 5 to 67 years. Nineteen patients were females and only 14 were males. At operation, 27 had complete surgical resection while six patients had only biopsy. We did not find any dumbbell tumour among our patients. After pathological examination there were 14 benign schwannomas, 3 neurofibromas 2 ganglioneuromas, 4 malignant schwannomas, 3 neurobalstomas and one ganglioneuroblastoma. All patients with malignant lesions were from North Sinai. In our series, neurogenic tumours represented 25% of all mediastinal tumours, 39.4% in children and 21.3% in adults included in the study [132 patients]. Benign tumours had an excellent prognosis after resection and a five-year-follow up. In our series, malignant ones represented 22% of all neurogenic masses. Prognosis of these malignant tumours in children was better than in adults. The higher the grade of malignancy the poorer is the prognosis even after radio and/or chemotherapy. Neurogenic tumours are more frequent in females. They should be treated early to get a better prognosis. Incidence of tumours is markedly higher among patients from North Sinai. High resolution CT and/or US studies were quite enough for accurate diagnosis. MRI studies were not essential to diagnose any of our cases