Long-term survival in patients with recurrent glioblastoma treated with bevacizumab: a multicentric retrospective study.

PURPOSE: Recurrence of glioblastoma (GB) occurs in most patients after standard concomitant temozolomide-based radiochemotherapy (CTRC). Bevacizumab (BV), an anti-VEGF antibody, has an effect on progression-free survival (PFS) but not on overall survival (OS). However, a small part of the patients experience a survival, longer than expected. This retrospective study aims to characterize long responder (LR) patients treated with BV for a first or second GBM recurrence. METHODS: Medical records from patients (814) who received BV for a first or second recurrence of primary glioblastoma between September 2010 and September 2015, and initially treated by CTRC were analyzed. Patients, who had at least a stable disease according to RANO criteria at 12 months from the start of BV, were included. Patients who had, a secondary GB, or received BV in neoadjuvant or adjuvant setting were excluded. RESULTS: We focused on 65 LR patients without progression 12 months after the first injection of BV (8%). Median PFS was 21.7 months [95% CI (19.3; 27.2)] and median OS was 31.1 months [95% CI (24.3; 37.5)] from the start of BV. No prognostic factor was associated with OS in multivariate analysis. Karnofsky performance status, neurological status and corticosteroid dose were stable at 12 months. CONCLUSIONS: Our results highlight that among patients receiving bevacizumab in first or second recurrence, one patient out of twelve could be classified as LR. A median OS of 31.1 months from the start of BV could be expected in this subpopulation. These findings reinforce the potential benefit of the use of BV in the situation of recurrence. 256 words.

Input of molecular analysis in medical management of primary brain tumor patients.

Primary brain tumors comprise a large group of malignant and non-malignant tumors including heterogeneous entities with various biological and clinical behaviors. Up till recently, diagnosis of brain cancers, that drives treatment decision-making, was based on integration of clinical, radiological and pathological features of patients and tumors. Over the last years, practical neuro-oncology has entered an era of molecular-based personalized medicine. Indeed, molecular features of tumors provide critical information to physicians for daily clinical management of patients and for design of relevant clinical research. Sporadic gliomas or glial tumors are the most common primary brain tumors in adults. Recently, their medical management has been revolutionized by molecular data. Indeed, optimal therapeutic management of grade III glioma patients now requires assessment of chromosome arms 1p/19q copy number and IDH mutational statuses as predictive and prognostic biomarkers. Indeed, two large phase III clinical trials have demonstrated that early chemotherapy plus radiotherapy, versus radiotherapy alone, doubles median overall survival of patients suffering from 1p/19q co-deleted and/or IDH mutated anaplastic oligodendroglial tumor. Interestingly, both biomarkers have been identified in a large proportion of WHO grade II gliomas. Their clinical value, in this population, is under investigation through multiple phase III clinical trials. In sporadic WHO grade I gliomas, and specifically in pilocytic astrocytomas, MAPK signaling pathway activation is a frequent event, mainly due to genetic alterations involving BRAF gene. This characteristic opens new therapeutic perspectives using MAPK signaling pathway inhibitors. Finally, in the most aggressive gliomas, WHO grade IV gliomas, two critical biomarkers have been identified: (i) MGMT promoter methylation associated with longer survival and better response to chemotherapy and (ii) IDH mutations predicting better prognosis. Although, further studies are needed, MGMT promoter methylation will undoubtedly be transferred soon to clinical practice. Molecular characteristics are beginning to be valuable and indispensable in neuro-oncology for better management of brain tumors patients. The near future will be marked by identification of novel molecular biomarkers and their validation for clinical practice.

Hipertension arterial secundaria a compresion de arteria renal por feocromocitoma extra-adrenal. Presentacion de un caso. / Arterial hypertension secondary to renal artery compression due to extra-adrenal pheocromocytoma. Presentation of a case

Se presenta el caso de un adolescente con hipertension arterial grave, en quien clinicamente y por metodos de gabinete se considero que la hipertension era de origen renovascular. La arteriografia renal mostro compresion de la arteria renal derecha por un tumor parapielico. El estudio histologico del mismo revelo que se trataba de un feocromocitoma extraadrenal. El paciente se curo de la hipertension arterial despues de la nefrectomia y extirpacion del tumor

[Prune bell syndrome]. / Síndrome de abdomen en ciruela pasa.

The prune belly syndrome is a very rare entity, characterized by a marked deficiency of the abdominal muscles, bilateral cryptorchidism and urinary tract abnormalities. The etiology is still unknown; the best accepted theory is the embryologic, due to disturbance in development between the sixth to tenth week. Histologically renal dysplasia is found together with partial or total absence of muscle fibers in the ureter, instead of which there is connective tissue, hypertrophy of the bladder with normal ganglionar cells; the prostatic urethra is dilated. There are three clinical types: neonatal, neonatal urgency and late development. There is not as yet a good management program set out for these children in whom the damage is varied. The best treatment is prophylaxis, control of infection avoiding instrumentation and repeated urological examinations. The prognosis depends on the volumen of functional parenchima and its preservation. Depends also on the degree of dysplasia and of insufficiency and secondary damage due to infection. We are reporting three differents cases and their management; we have obtained good results and we expect a good prognosis according to the special characteristics of their evolution.