ABSTRACT
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 (AU)
El angiosarcoma es un tumor infrecuente entre los sarcomas, especialmente cuando se trata de una lesión primaria en el sistema nervioso central, que puede conducir a un rápido deterioro neurológico y a la muerte en pocos meses. Se presenta a un varón de 41 años con una lesión hemorrágica frontal derecha. Se realiza cirugía con resultados histopatológicos que sugieren un angiosarcoma cerebral primario. El paciente recibió tratamiento complementario con quimioterapia y radioterapia. A los cinco meses, el seguimiento con RM mostró dos lesiones y un hematoma subdural agudo, sugiriendo recaída. Se realizó una nueva cirugía hallando membranas tumorales unidas a la cara interna de la duramadre, alrededor del hemisferio derecho. El paciente acabó falleciendo debido a la recurrencia del hematoma subdural. Este caso ilustra una complicación rara y letal de un tumor infrecuente. La literatura revisada muestra que su tratamiento de elección es la resección completa y la radioterapia adyuvante (AU)
Subject(s)
Humans , Male , Adult , Hemangiosarcoma/surgery , Brain Neoplasms/surgery , Hematoma, Subdural, Acute/diagnostic imaging , Tomography, X-Ray Computed , Magnetic Resonance Imaging , Fatal OutcomeABSTRACT
The molecular biology of human glioma is a complex and fast-growing field in which basic research needs to meet clinical expectations in terms of anti-tumor efficacy. Although much effort is being done in molecular biology research, significant contribution to the quality of life and overall survival still lacks. The vastness of molecular biology literature makes it virtually impossible for clinicians to keep up to date in the field. This paper reviews some practical concepts regarding glioma tumorigenesis from the clinician's perspective. Five main aspects are discussed: major intracellular signaling pathways involved in glioma formation; genomic, epigenetic and transcriptomic relevant features of glioma; the prognostic and predictive values of molecular markers according to the new WHO classification of glial tumors; the importance of molecular and cellular heterogeneity in glioblastoma, responsible for its therapy resistance; and the interaction between glioma and the immune system, in view of the novel and promising targeted therapies.
Subject(s)
Brain Neoplasms/genetics , Glioma/genetics , Brain Neoplasms/blood supply , Brain Neoplasms/etiology , Brain Neoplasms/pathology , Glioma/blood supply , Glioma/etiology , Glioma/pathology , Humans , Molecular Biology , Signal Transduction/physiology , TranscriptomeABSTRACT
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 oligodendro-gliomas 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 oligodendro-gliomas 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.
TITLE: Análisis del impacto clínico de la reclasificación diagnóstica de gliomas cerebrales según la clasificación de la Organización Mundial de la Salud (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.
Subject(s)
Astrocytoma/classification , Astrocytoma/diagnosis , Brain Neoplasms/classification , Brain Neoplasms/diagnosis , Oligodendroglioma/classification , Oligodendroglioma/diagnosis , Adult , Astrocytoma/genetics , Brain Neoplasms/genetics , Female , Humans , Isocitrate Dehydrogenase/genetics , Male , Middle Aged , Mutation , Neoplasm Grading , Oligodendroglioma/genetics , Retrospective Studies , World Health OrganizationABSTRACT
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