Valor pronóstico de la perfusión cerebral por RM en el estudio inicial de los gliomas de alto grado / Prognostic value of brain perfusion by MRI in the initial study of high grade gliomas

Objetivos: Valorar si la perfusión tumoral en el estudio diagnóstico inicial de RM es un marcador pronóstico para la supervivencia en pacientes diagnosticados de gliomas de alto grado. Analizar los factores de riesgo que influyen en la mortalidad por gliomas de alto grado para poder cuantificar la supervivencia global esperada del paciente. Pacientes y métodos: Se seleccionaron las RM de todos los pacientes diagnosticados de glioma de alto grado en un hospital de tercer nivel entre los años 2017 y 2019. Se recogieron variables clínicas y tumorales. Se usó el análisis de supervivencia para determinar la asociación entre la perfusión tumoral y el tiempo de supervivencia. Se estudió la relación entre las variables recogidas y la supervivencia mediante el estadístico de Wald, cuantificando esta relación mediante la regresión de Cox. Por último, se analizó el tipo de relación existente entre la perfusión tumoral y la supervivencia a través del estudio de regresión lineal. Estos análisis estadísticos se realizaron con el software SPSS v.17. Resultados: Se incluyeron 38 pacientes (media de edad 61,1años). La supervivencia media global fue de 20,6meses. Se observó asociación entre la perfusión tumoral en la RM diagnóstica y la supervivencia global, mostrando el grupo con valores intratumorales de volumen sanguíneo cerebral relativo (rVSC) >3,0 una disminución significativa en el tiempo medio de supervivencia respecto al grupo con valores <3,0 (14,6meses vs 22,8meses, p=0,046). También han demostrado influir significativamente en la supervivencia media variables como la escala de Karfnosky y el tiempo de recidiva desde la intervención. Conclusiones: Se ha evidenciado que la perfusión tumoral por RM tiene valor pronóstico en el estudio inicial de los gliomas de alto grado.(AU)

Objectives: To evaluate if the tumour perfusion at the initial MRI scan is a marker of prognosis for survival in patients diagnosed with high grade gliomas (HGG). To analyse the risk factors which influence on the mortality from HGG to quantify the overall survival to be expected in patients. Patients and methods: The patients diagnosed with HGG through a MRI scan in a third-level hospital between 2017 and 2019 were selected. Clinical and tumour variables were collected. The survival analysis was used to determine the association between the tumour perfusion and the survival time. The relation between the collected variables and the survival period was assessed through Wald's statistical method, measuring the relationship via Cox's regression model. Finally, the type of relationship that exists between the tumour perfusion and the survival was analysed through the lineal regression method.Those statistical analysis were carried out using the software SPSS v.17. Results: Thirty-eight patients were included (average age: 61.1years old). The general average survival period was 20.6months. A relationship between the tumour perfusion at the MRI scan and the overall survival has been identified, in detail, a group with intratumor values of relative cerebral blood volume (rCBV) >3.0 has shown a significant decline in the average survival period with regard to the average survival period of the group with values <3.0 (14.6months vs. 22.8months, P=.046). It has also been proved that variables like Karnofsky's scale and the response time since the intervention significantly influence on the survival period. Conclusions: It has become evident that the tumour perfusion via MRI scan has a prognostic value in the initial analysis of HGG. The average survival period of patients with rCBV less than or equal to 3.0 is significantly higher than those patients whose values are higher, which allows to be more precise with the prognosis of each patient.(AU)

Treatment of pediatric cerebral radiation necrosis using hyperbaric oxygenation.

Introduction: Cerebral radiation necrosis is rarely encountered in pediatric patients. This case report describes a child with cerebral radiation necrosis who was successfully treated using corticosteroids, bevacizumab, and hyperbaric oxygenation. Case report: A 3-year-old boy developed progressive extremity weakness six months after the completion of radiation therapy for the treatment of a neuroepithelial malignancy. Treatment with corticosteroids and bevacizumab was initiated, but his symptoms did not improve, and he was then referred for hyperbaric oxygen therapy. After completing 60 hyperbaric treatments, he experienced significant improvements in mobility, which remained stable over the next year. Discussion: Cerebral radiation necrosis typically presents in children with symptoms of ataxia or headache. Corticosteroids and bevacizumab are common treatments, but hyperbaric oxygen therapy has also been studied as a therapeutic modality for this condition. When considering the use of hyperbaric oxygenation in pediatric patients, careful attention to treatment planning and patient safety can reduce the risks of adverse events such as middle ear barotrauma and confinement anxiety. Conclusion: In addition to other available pharmacologic therapies, hyperbaric oxygenation should be considered for the treatment of pediatric patients with cerebral radiation necrosis.

Rosette Forming Glioneural Tumor Treated with Conformal Radiation.

Rosette forming glioneural tumors (RGNT) are a rare type of low-grade brain tumor included in 2007 in WHO classification. Given the benign nature of the disease, a complete surgical excision has been considered optimum. However, a handful of cases have reported the locally aggressive nature of RGNT. In addition, radiation may also be considered for a tumor located in areas where surgical excision is difficult. We present a similar case, where surgical risk was weighed against resection and we treated the patient with conformal radiation.

[Recurrent High-grade Astroblastoma Treated with Stereotactic Radiotherapy:A Case Report].

INTRODUCTION: Astroblastoma is a rare, supratentorial glial tumor, occurring predominantly in children and young adults. However, treatment strategies have not yet been established for this rare disease. CASE PRESENTATION: A 6-year-old boy presented with headache and nausea. CT and MRI revealed a left frontal mass lesion with slight edema and macrocalcifications. Gross tumor resection was performed. Histological examination found neoplastic cells with astroblastic characteristics, and a striking perivascular array of pseudorosettes. The final diagnosis was high-grade astroblastoma. MRI 13 months after surgery suggested local recurrence, and an enlargement was found 3 months later. Stereotactic radiotherapy(SRT)was performed. MRI after SRT showed enhanced cyst formation around the tumor bed, suggesting tumor recurrence. However, 11C-methionine positron emission tomography(PET)revealed radiation necrosis. The last follow-up MRI 15 months after SRT showed no further recurrence. CONCLUSION: Astroblastoma is rare, therefore, no optimal management is known. SRT may be effective to treat recurrent astroblastomas. 11C-methionine PET/CT was useful to differentiate metastatic disease from radiation necrosis.

Posterior fossa astroblastoma in a child: a case report and a review of the literature.

A 4-year-old girl presented to the hospital with a progressive headache, difficulty walking, and persistent daily vomiting for 3 weeks. Papilledema was observed on fundoscopic examination. A large left cerebellovermian tumor with "bubbly" appearance was discovered. Total removal of the tumor mass was performed, and a diagnosis of low-grade astroblastoma was made. Adjuvant radiotherapy was performed due to the risk of recurrence. The patient is disease-free and has been kept on close follow-up for 6 months. The occurrence of posterior fossa astroblastoma has been rarely reported in the literature. Thus, when a "bubby" appearance enhancing cystic solid tumor is located on the cerebellar hemisphere in a child, an astroblastoma should also be included in the differential diagnosis.

Radiologic response to radiation therapy concurrent with temozolomide for progressive simple dysembryoplastic neuroepithelial tumor.

Dysembryoplastic neuroepithelial tumors (DNETs) are low-grade neuroglial tumors that are traditionally considered to be benign hamartoma-like mass lesions. Malignant transformation and disease progression have been reported in complex DNETs. We report a case of a simple DNET with disease progression following subtotal resection. A 34-year-old woman underwent craniotomy with subtotal resection of a large nonenhancing right temporal lobe and insular mass. Histopathological analysis revealed a simple DNET. Magnetic resonance imaging obtained 6 months after surgery demonstrated disease progression with no enhancement or change in signal characteristics. Following concurrent therapy with temozolomide and external beam radiation therapy, a significant radiologic response was observed. Progressive DNET with malignant transformation exhibits predominantly glial transformation and occurs predominantly in complex DNETs. The histological classification of DNETs into simple, complex, and nonspecific are reviewed. Contrast-enhancing regions are more frequently seen in complex tumors, with nonenhancing regions having fewer complex histologic features. Close clinical and radiographic follow-up is important in all cases of DNET. Following tumor progression, radiation therapy with concurrent and adjuvant temozolomide chemotherapy may be an effective treatment.

Gliomatosis cerebri having a poor performance status without recurrence after radiotherapy: a single institutional experience.

OBJECTIVE: Radiotherapy (RT) has been advocated for gliomatosis cerebri (GC) to delay tumor progression. However, patients with GC may experience poor performance status without recurrence after RT. Herein, we reviewed the responses of patients with GC treated with RT. MATERIALS AND METHODS: Seventeen patients with diffuse radiologic infiltration of more than two cerebral lobes and diagnosed as having grade II (N=12) or III (N=5) glioma were treated with a mean radiation dose of 51.8 Gy (range, 42-59.4 Gy). The mean radiation volume to a mean marginal dose of 42.8 Gy (range, 36-45 Gy) was 693.4 cc (range, 316.5-1279.6 cc), which was 45.3% of the mean whole brain volume. We reviewed the clinical prognostic factors related to progression-free survival (PFS) and overall survival (OS) in all patients and described characteristics of patients with poor performance (Eastern Cooperative Oncology Group performance scale 3-4) without recurrence. RESULTS: In all patients, the median PFS was 12.3 months, and oligodendroglial pathology (p=0.002) and non-enhanced tumor (p=0.002) were associated with an improved PFS. The median OS was 39.3 months, and young age and adjuvant chemotherapy were associated with improved OS (p=0.022 and 0.002, respectively). Based on multivariate analysis, adjuvant chemotherapy was significantly associated with a longer OS (p=0.012; hazard ratio=0.099; 95% CI, 0.016-0.596). Five patients (29.4%) showed poor performance without recurrence. The median age of these 5 patients was 65 years (range, 60-75 years). The mean radiation volume to a mean marginal dose of 41 Gy (range, 36-45 Gy) was 539.9 cc (range, 255.5-983.1 cc). The pathologic diagnosis was grade II glioma in 2 patients and grade III in 3 patients. The median OS was 10.0 months (range, 8.2-45.9 months). CONCLUSIONS: Some GC patients, especially the elderly, might have a poor performance status without recurrence after RT of a larger radiation field.

Astroblastoma: report of two cases with unexpected clinical behavior and review of the literature.

Astroblastoma is a rare, mostly supratentorial glial tumor, occurring predominantly in children and young adults with female preponderance. Due to the rarity, with only about 230 reported cases, treatment strategies are still to be discussed. We describe two more cases to add to the clinical experiences with this tumor entity. In both of these cases, the clinical behavior did not follow the expectations based on histopathological classification. Case 1: A sixteen-year old female presented with a six month history of intermittent headaches, nausea and dizziness. MRI scans revealed a left parietooccipital mass lesion with bubbly contrast enhancement and marked peritumoral edema. After gross total tumor resection the histology gave the diagnosis of low grade astroblastoma. Fifteen months later, the patient was operated on a local recurrence and received postoperative radiotherapy, with the histology still being that of low grade astroblastoma. Two years later, a meningeally based tumor nodule frontal of the former tumor bed was removed, now diagnosed as high grade astroblastoma. Eighteen months later, a fourth operation with excision of two more meningeally based tumor nodules of high-grade astroblastoma followed. Chemotherapy was suggested, but the patient decided against it. The last MRI follow-up 14 months after last operation showed no further recurrence so far. Case 2: A 24-year old female presented with a four week history of vomiting and headaches with focal seizures affecting her left arm. CT and MRI scans revealed a superficial partly cystic right temporal mass lesion with few edema and macrocalcifications and adjacent bone atrophy. Despite dural invasion, total tumor resection could be performed. MRI scans six months later showed no recurrence. The patient refused further MRI controls but did not show any clinical signs or symptoms suggesting tumor recurrence four years after the operation. In order to find more predictive tools that might help to determine the individual clinical course and treatment, we performed a review of the literature, analyzing 29 cases with detailed data on clinical history, MRI/CT characteristics, histopathological subtyping, treatment details and a follow-up of at least 12 months. We found, that low-grade astroblastoma with marked peritumoral edema has a tendency to early recurrence and suggest that it should be treated by combined surgery and radiotherapy. In high-grade astroblastoma with well defined tumor borders and few peritumoral edema, the prognosis may be better than expected for a high-grade glioma, if GTR is possible.

Patterns of presentation and failure in patients with gliomatosis cerebri treated with partial-brain radiation therapy.

BACKGROUND: The patterns of lobar involvement, optimal treatment, and disease course among patients with gliomatosis cerebri (GC) have not been fully characterized. The current study evaluates the clinical presentations and outcomes for patients with GC treated with radiation therapy (RT) at our institution. METHODS: A total of 26 patients (25 with follow-up) with GC were diagnosed and treated between January 2004 and June 2012. Inclusion criteria consisted of brain magnetic resonance imaging and neuroradiology confirmation of contiguous involvement of ≥ 3 lobes/lobar equivalents with preservation of neural architecture. Patients were treated with either partial-brain RT to involved tumor (25 patients) or whole-brain RT (1 patient). The median RT dose was 54.0 Gray. The median follow-up was 17.3 months. RESULTS: The median age of the patients at the time of diagnosis was 57 years. Twenty-one patients (81%) and 5 patients (19%) had 3 to 6 and ≥ 7 involved lobes/lobar equivalents, respectively. The median progression-free survival and overall survival were 7.4 months and 14.9 months, respectively. Fifteen patients experienced radiographic disease progression after partial-brain RT, 14 of whom (93%) developed infield disease recurrence. On univariate analysis, higher tumor grade and type II GC (with focal mass) were associated with a poorer progression-free survival. The extent of lobar involvement and chemotherapy were not associated with overall survival. CONCLUSIONS: Even with partial-brain RT, nearly all disease recurrences were infield and clinical outcomes were similar to previous GC series, thereby suggesting that whole-brain RT is not necessary for this patient population. A greater number of involved lobes did not correlate with inferior outcomes. Further studies are necessary to establish more uniform and optimal treatments for this rare disease.

Exclusive radiotherapy for gliomatosis cerebri: long-term follow-up at a single institution.

PURPOSE: To evaluate the efficacy of radiotherapy and factors affecting the prognosis of gliomatosis cerebri. METHODS: Twenty-eight patients with pathologically confirmed gliomatosis cerebri underwent radiotherapy between August 1988 and September 2003. The median age of the patients was 39 years (range 18-67). Performance status was good (ECOG score ≤2) in 23 patients (82 %). The extent of radiotherapy was partial brain in 17 patients, whole brain in 2 patients, and whole brain followed by partial brain in 9 patients. The median radiation dose was 55.8 Gy (range 46.8-70.4). The median duration of follow-up was 136 months for survivors (range 39-191). RESULTS: The median overall and progression-free survival times of all patients were 20 and 11 months, respectively. When initial response to radiotherapy was grouped as improved, stationary, and aggravated, the median overall survival times in patients with improved, stationary, and aggravated responses were 76, 20, and 7 months, respectively (p = 0.0129). However, radiation parameters such as dose and irradiation volume had no impact on overall survival. On multivariate analysis, both performance status and initial response to radiotherapy were significant prognostic factors affecting overall survival (p = 0.0249 and 0.0065, respectively). CONCLUSIONS: This study showed that gliomatosis cerebri could be effectively treated with radiotherapy and that initial response to radiotherapy was a significant prognostic factor affecting the survival.

Astroblastomas: a Surveillance, Epidemiology, and End Results (SEER)-based patterns of care analysis.

OBJECTIVE: This study sought to report patient characteristics, risk factors, and trends in management for astroblastoma patients. METHODS: A retrospective analysis was conducted utilizing the Surveillance, Epidemiology and End Results Program of the National Cancer Institute. RESULTS: Two hundred and thirty-nine patients were identified, with 206 patients receiving treatment. The median age at diagnosis was 35 years (range 0 to 84 years). Tumor location was available for 177 patients, and the majority were supratentorial (n = 144, 81.3%). The median overall survival and cause-specific survival for the cohort receiving treatment was 55 and 65 months, respectively. On univariate analysis, patients receiving surgery alone compared to only radiotherapy displayed improved overall survival (OS) and cause-specific survival (CSS) with a 5-year OS of 62.2% vs. 27.3%, P < .001, and CSS of 67.3% vs. 31.9%, P = .003. Supratentorial tumor location was associated with worse survival, with an estimated 5-year OS of 44.9% for supratentorial tumors compared to 75% for infratentorial tumors (hazard ratio 3.41 [95% CI, 1.76 to 6.62]; P < .001) and CSS of 47.5% (supratentorial) to 82% (infratentorial) (hazard ratio 3.95 [95% CI, 1.81 to 8.62]; P = .001). Age >60 years at diagnosis and treatment before 1990 were correlated with decreased survival on both the univariate and the multivariate analyses. CONCLUSIONS: To our knowledge, this is the largest report of astroblastoma patients described in the literature. Supratentorial tumor location, older age, and treatment prior to 1990 were poor prognostic factors.

Phantosmia during radiation therapy: a report of 2 cases.

Phantosmia is an infrequently reported and poorly understood qualitative olfactory disorder characterized by the perception of a frequently unpleasant odor in the absence of an odorant stimulus. Peripheral phantosmia is hypothesized to involve abnormally active olfactory receptor neurons while central phantosmia is theorized to be the result of hyperactive neurons in the cortex. The authors present a case report that describes 2 patients with incomparable tumors and radiation fields who both experienced phantosmia featuring a halitosis-like odor during their courses of radiation therapy. Both the 6-year-old with diffuse intrinsic pontine glioma and the 15-year-old with World Health Organization grade II-III astrocytoma in the bifrontal lobes experienced significant distress and decreased quality of life during treatment because of the phantosmia, which resolved after completion of radiation therapy. To the best of the authors' knowledge, these are the first descriptions of phantosmia during focal or whole-brain radiation therapy.

Pediatric astroblastoma: a rare case with a review of the literature.

Astroblastoma is a rare glial tumor with uncertain histopathological origin and unpredictable clinical behavior. We present a case of an 11-year-old girl who presented with headache and blurring of vision for 2 months. A well-demarcated mass was found in the right frontoparietal lobe on a brain MRI. The patient was treated with total tumor resection followed by postoperative radiotherapy. Histologically, the features were suggestive of high-grade astroblastoma. The patient is alive and disease free 23 months after surgery. The characteristic radiological and histopathological features and treatment of this case are described with a literature review.

Marked response of gliomatosis cerebri to temozolomide and whole brain radiotherapy.

Gliomatosis cerebri (GC) represents an unfortunate, rare variant of glioma with a very poor prognosis. Given this lesion's rarity, little information exists on appropriate treatment options. The diffuse, infiltrative nature of GC precludes any surgical resection and limits therapy. Because of the improved survival seen with the use of temozolomide (TMZ) in malignant glioma, a rigorous systematic review of the published literature was performed to ascertain the benefit of TMZ in GC. We identified all GC cases in the literature where there was enough information to ascertain a clear response to a specific chemoradiotherapeutic treatment. In addition to our experience with a recent case, we have identified 61 patients with GC in the published literature who demonstrated a positive radiographic or clinic response after treatment. Statistical analysis of survival was performed by Kaplan-Meier analysis. A positive radiographic and clinical response was seen in patients ranging in age from 4 to 84 years. Overall median survival in patients diagnosed with GC who demonstrated a response after treatment was 25 months, with 1- and 2-year survival rates of 89% and 55%, respectively. The most common treatment regimens for responders included TMZ alone (26.2%), external whole-brain radiotherapy (WBRT) (26.2%), and concomitant TMZ and WBRT (20%). Our patient was treated with concomitant TMZ (150 mg/m(2)/day over 5 days) and WBRT (50 Gy) and has remained with a complete radiographic response after 36 months. In conclusion, patients with GC confirmed by surgical biopsy should be aggressively treated with concomitant TMZ and WBRT, as marked responses have been seen, and this appears to offer overall survival benefit.

Efectos cognitivos de la radioterapia en gliomas de bajo grado / No disponible

El aumento en las tasas de supervivencia de los pacientes con gliomas de bajo grado está suscitando un elevado interés en los efectos neurotóxicos asociados a los tratamientos, en especial a la radioterapia. En la actualidad, se señala la presencia de alteraciones principalmente en memoria visual asociada a dicha técnica de tratamiento, aunque existen datos contrarios que postulan que la radioterapia, al igual que otros tratamientos, favorece el control de la enfermedad y preserva el rendimiento neuropsicológico de esta población. No obstante, las limitaciones metodológicas de estos estudios y la dificultad en el seguimiento de estos pacientes podrían estar provocando esta divergencia en los resultados hallados en este campo de investigación. Por ello, se reitera la necesidad de realizar estudios que utilicen las técnicas de tratamiento de radioterapia actuales, que aumentan la seguridad y disminuyen los efectos secundarios neurológicos, así como que apliquen cuestionarios con una adecuada sensibilidad a los efectos neurotóxicos de los tratamientos adyuvantes (AU)

The increased survival of patients with low grade gliomas generated increased interest in the neurotoxic effects associated with treatment, especially radiotherapy. Currently, the radiotherapy is associated with visual memory failures in this population. On the contrary, some authors postulate that radiotherapy, as well as other treatments, helps control the disease and preserves the neuropsychological performance. However, methodological limitations of these studies and the difficulty in monitoring these patients could be causing this divergence in the results found in this research field. Therefore, we note the need for studies that use radiation therapy treatment techniques current, which increase safety and reduce neurological side effects, and apply appropriate questionnaires with a sensitivity to the neurotoxic effects of adjuvant treatments (AU)

Deterioro cognitivo asociado a radioterapia en tumores cerebrales / No disponible

Introducción: Existe un limitado conocimiento acerca de la influencia sobre las funciones cognitivas de los tratamientos para los tumores cerebrales basados en la radioterapia (RT). Objetivo: Realizar una revisión actualizada de los trabajos desarrollados en los últimos años acerca de los efectos de la RT sobre la cognición en los tumores cerebrales. Método: Mediante el software EndNote X5 se extrajeron los artículos que contenían “radiotherapy” y “cognition” tanto en su título, como en palabras claves, abstracts o contenidos en los años 2009-2011. Asimismo, se realizó una búsqueda ampliada a través de las referencias de los artículos extraídos. Se descartaron aquellos artículos en los que no se reflejase la relación entre RT y el desarrollo de alteraciones cognitivas o no se centraran en tumores cerebrales. Resultados: 14 artículos fueron seleccionados: 7 originales, 6 revisiones y 1 artículo de opinión. Los hallazgos se centraron en 3 aspectos: a) alteraciones cognitivas debidas a la RT; b) factores de riesgo para el desarrollo de deterioro cognitivo inducido por la RT; c) tiempo transcurrido desde la aplicación de la RT hasta la aparición de las alteraciones cognitivas. Conclusiones: Los nuevos estudios confirman hallazgos de las últimas décadas, principalmente que las funciones más comúnmente afectadas son la atención, la memoria y el aprendizaje, la psicomotricidad y las funciones ejecutivas; que existen algunos factores de riesgo identificables para el desarrollo de deterioro cognitivo asociado a la RT; así como que las alteraciones en la función cognitiva tras la RT suelen aparecer de manera evidente después un largo período de tiempo (AU)

Introduction: There is limited knowledge about the influence on cognitive functions of the treatments for brain tumors based on radiotherapy (RT). Objective: To review the works conducted in recent years about the effects of RT on cognition in brain tumors. Method: Using EndNote software X5, articles containing “radiotherapy” and “cognition” in its title, keywords, abstracts or main text in the years 2009-2011 were extracted. Also, an extensive search through the references of articles taken was done. Those works that did not reflect the relationship between RT and the development of cognitive impairment or did not focus on brain tumors were disregarded. Results: 14 articles were selected: 7 originals, 6 reviews, and 1 opinion article. The findings focused on three aspects: a) cognitive impairment due to the RT, b) risk factors for cognitive impairment induced by RT c) time from the application of RT to the onset of cognitive impairment. Conclusions: The new studies confirm findings of recent decades, namely that the functions most commonly affected are attention, memory and learning, psychomotor and executive functions; that there are identifiable risk factors for cognitive impairment associated to RT; and that alterations in cognitive function after RT usually occur after a long period of time (AU)

Long survival of primary diffuse leptomeningeal gliomatosis following radiotherapy and temozolomide: case report and literature review.

OBJECTIVE: Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare neoplasm with a short survival time of a few months. There is currently no standardized therapeutic approach for PDLG. - MATERIAL AND METHODS: We report on a 53-year-old male patient who presented with epileptic seizures, gait disturbance, paraparesis and sensory deficits in the dermatomes T8-10. - RESULTS: Magnetic resonance imaging (MRI) revealing numerous spinal and cranial gadolinium-enhancing nodules in the meninges and histopathology led us to diagnose primary diffuse leptomeningeal gliomatosis with WHO grade III astrocytic cells. Consecutively, the patient underwent craniospinal radiotherapy (30Gy) and 11 sequential cycles of temozolomide. This regimen led to partial tumor regression. Thirteen months later, spinal MRI revealed tumor progression. Second-line chemotherapy with 5 cycles of irinotecan and bevacizumab did not prevent further clinical deterioration. The patient died twenty-two months after diagnosis, being the longest survival time described thus far with respect to PDLG consisting of astrocytic tumor cells. - CONCLUSIONS: Radiochemotherapy including temozolomide, as established standard therapy for brain malignant astrocytomas, might be valid as a basic therapeutic strategy for this PDLG subtype.

Gliomatosis cerebri.

Gliomatosis cerebri is a rare diffusely infiltrating glial tumor involving two or more lobes and is frequently is bilateral. Infiltrative extent of tumor is out of proportion to histological and clinical features. We present a case in which finally the diagnosis of gliomatosis cerebri was made. In this case, computed tomography showed that midline structures were insignificantly shifted to the left, there was a mild dilatation of lateral ventricles more expressed on the right, and no pathologic changes of brain tissue density were found. On magnetic resonance tomography, T2W/SE and T2W/FLAIR images revealed zones of hyperintense signal, spreading with time, through several lobes of the brain with no enhancement on T1W images. Diagnosis of gliomatosis cerebri was suspected, stereotaxic biopsy was performed, and pathological examination revealed changes typical of diffuse glial tumor. In this article, changes typical of gliomatosis cerebri seen in other radiological methods such as computed tomography, magnetic resonance spectroscopy, dynamic contrast-enhanced T2*-weighted magnetic resonance, and positron emission tomography also are discussed.

Two cases of pediatric high-grade astroblastoma with different clinical behavior.

Astroblastoma is a rare glial tumor occurring in older children and defined by histological criteria as low or high-grade. We describe two children with high-grade astroblastoma. The first patient, with multiple recurrences of a frontoparietal tumor, died 10 years from diagnosis after progression of the disease despite surgery, radiotherapy and chemotherapy. The second patient underwent subtotal resection of a temporal mass; the residual tumor progressed five months after radiotherapy, but after a subsequent gross total resection the patient is now in complete remission 54 months from diagnosis. Although both patients had high-grade astroblastomas, there were histological differences between the two tumors, in particular regarding the proliferative index, which was 30% and 5-10%, respectively. High-grade astroblastoma is usually treated with surgery and radiotherapy, but may have an unpredictable behavior even when tumor excision is deemed complete. The two cases reported here illustrate the variable clinical course of this rare tumor. The proliferative index may be a useful tool to better define prognosis.