ABSTRACT
Idiopathic Spinal Cord Herniation (ISCH) is considered to be a rare cause of Thoracic Myelopathy. It is secondary to the gliding of the Spinal Cord through an anterior dural defect, without a completely defined cause. We present a case of ISCH which, even though was in its usual location, developed in a woman at a younger age than expected. The patient was 20 years old when diagnosed with Brown-Séquard Syndrome. MRI showed herniation at T4-T5 level, which was corrected using a posterior approach to expose the dural defect, reduce the herniation and place a heterologous graft. Postoperatively, neurological function improved, and adequate reduction was seen on imaging. Given the reports of recurrence and deterioration that have been seen after 18 months, follow-up was prolonged for a total of 2 years. We consider postoperative MRI performance important to establish the degree of reduction and alignment of the Spinal Cord.
Subject(s)
Brown-Sequard Syndrome/diagnosis , Hernia/diagnostic imaging , Spinal Cord Diseases/diagnosis , Spinal Cord/pathology , Female , Herniorrhaphy/methods , Humans , Laminectomy/methods , Magnetic Resonance Imaging , Rare Diseases , Spinal Cord Diseases/physiopathology , Spine/diagnostic imaging , Spine/surgery , Thoracic Vertebrae/diagnostic imaging , Thoracic Vertebrae/surgery , Treatment Outcome , Young AdultABSTRACT
PURPOSE: Patients with recurrent glioblastoma (rGBM) have a poor prognosis, with survival ranging from 25 to 40 weeks. Antiangiogenic agents are widely used, showing a variable response. In this study, we explored the efficacy of carmustine plus bevacizumab (BCNU/Bev) for treating rGBM. METHODS/PATIENTS: In this study, we assessed 59 adult patients with histologically confirmed rGBM who were treated with BCNU/Bev as second-line regimen. The response rate (RR), progression-free survival (PFS) and overall survival (OS) were evaluated according to their molecular expression profile, including CD133 mRNA expression, MGMT methylation (pMGMT), PDGFR amplification, YKL40 mRNA expression, IDH1/2 condition, p53 and EGFRvIII mutation status. RESULTS: Median follow-up was 18.6 months, overall RR to the combination was 56.3%, and median PFS was 9.0 months (95% CI 8.0-9.9). OS from time of diagnosis was 21.0 months (95% CI 13.2-28.7) and from starting BCNU/Bev it was 10.7 months (95% CI 9.5-11.8). IDH1/2 mutations were found in 30.5% of the patients, pMGMT in 55.9% and high CD133 mRNA expression in 57.6%. Factors which positively affected PFS included performance status (p = 0.015), IDH+ (p = 0.05), CD133 mRNA expression (p = 0.009) and pMGMT+ (p = 0.007). OS was positively affected by pMGMT+ (p = 0.05). Meanwhile, YKL40 negatively affected PFS (p = 0.01) and OS (p = 0.0001). Grade ≥ 3 toxicities included hypertension (22%) and fatigue (12%). CONCLUSIONS: BCNU/Bev is a safe and tolerable treatment for rGBM. Patients with MGMT+/IDH+ derive the greatest benefit from the treatment combination in the second-line setting. Nonetheless, high YKL40 expression discourages the use of antiangiogenic therapy.
Subject(s)
Angiogenesis Inhibitors/therapeutic use , Bevacizumab/therapeutic use , Brain Neoplasms/drug therapy , Carmustine/therapeutic use , Glioblastoma/drug therapy , Neoplasm Recurrence, Local/drug therapy , AC133 Antigen/genetics , AC133 Antigen/metabolism , Adult , Aged , Angiogenesis Inhibitors/adverse effects , Antineoplastic Agents/adverse effects , Antineoplastic Agents/therapeutic use , Bevacizumab/adverse effects , Brain Neoplasms/blood supply , Brain Neoplasms/genetics , Brain Neoplasms/mortality , Carmustine/adverse effects , Chitinase-3-Like Protein 1/genetics , Colombia , DNA Modification Methylases/metabolism , DNA Repair Enzymes/metabolism , Drug Administration Schedule , Female , Genes, erbB-1 , Genes, p53 , Glioblastoma/blood supply , Glioblastoma/genetics , Glioblastoma/mortality , Humans , Isocitrate Dehydrogenase/genetics , Male , Methylation , Middle Aged , Mutation , Neoplasm Recurrence, Local/blood supply , Neoplasm Recurrence, Local/genetics , Neoplasm Recurrence, Local/mortality , Progression-Free Survival , RNA, Messenger/metabolism , Receptors, Platelet-Derived Growth Factor/genetics , Survival Analysis , Tumor Suppressor Proteins/metabolism , Young AdultABSTRACT
El tumor fibroso solitario se localiza principalmente en la pleura. Su localización extrapleural es rara, en cabeza y cuello se han encontrado pocos casos, principalmente en los senos paranasales y rinofaringe. De igual manera es de presentación infrecuente la localización en los tejidos blandos de la órbita, la cual ha sido descrita recientemente. El objetivo de este informe es la presentación clínica patológica y por imágenes de un caso tratado en la Fundación Santa Fe de Bogotá, en conjunto con la Clínica Barraquer