ABSTRACT
PURPOSE: Pediatric high-grade gliomas (pHGGs) constitute almost 15% of all childhood brain tumors. Recurrent mutations such as H3K27M mutation in H3F3A and HIST1H3B genes encoding histone H3 and its variants were identified in approximately 30% of pediatric glioblastomas. This study aimed to ascertain the morphological and molecular characteristics of pHGGs with H3K27M mutation. METHODS: In total, 61 cases of pHGGs (anaplastic astrocytoma, 12; glioblastomas, 49) from four university hospitals were studied. The histomorphological features were examined and immunohistochemistry was performed to evaluate the mutation status of H3K27M, ATRX, IDH1, BRAF V600E, and p53 genes. RESULTS: The study comprised 25 females and 36 males (age range, 1-18 years) with a clinical follow-up of up to 108 months. From the total, 31 patients were positive for H3K27M mutation located in the midline, mostly in the pons and thalamus. H3K27M mutation was commonly associated with ATRX loss (32.3%) and p53 (74.2%) immunoreactivity with a co-expression rate of 25.8%. While IDH1 mutation was not detected in pHGGs with H3K27M mutation, BRAFV600E mutation was rarely observed. Among the various histomorphological features, increased number of mitosis, increased Ki-67 proliferation index, and palisading and geographical necrosis along with small cell patterns were significantly associated with the H3K27M wild-type tumors. Focal infarct-like necrosis and pilomyxoid morphology was significantly associated with these tumors. CONCLUSION: H3K27M mutation occurs exclusively in pHGGs arising from the midline and presents with varied histomorphological features ranging from low-grade pilomyxoid astrocytoma to highly pleomorphic glioblastoma along with ATRX loss and p53 mutations.
Subject(s)
Astrocytoma/genetics , Brain Neoplasms/genetics , Glioblastoma/genetics , Glioma/genetics , Histones/genetics , Adolescent , Astrocytoma/pathology , Brain Neoplasms/pathology , Child , Child, Preschool , Female , Follow-Up Studies , Genes, p53/genetics , Glioblastoma/pathology , Glioma/pathology , Humans , Infant , Male , Mutation , Necrosis , Retrospective Studies , Survival Analysis , X-linked Nuclear Protein/geneticsABSTRACT
As the use of instrumentation in spinal surgery has become common, the need for revision surgery has increased. During revision surgery one of the most difficult steps is removal of poly-axial pedicle screws, especially if no suitable revision set is available. We describe here an easy method for poly-axial screw removal. Leaving or placing a small piece of rod, attached firmly by a nut, tightens the head of the poly-axial screw. It can no longer move freely from the distal part of the screw and the screw can be removed by turning it counterclockwise with a big clamp or needle driver, which is available in almost every surgery set.
Subject(s)
Bone Screws , Device Removal/methods , Orthopedic Procedures/instrumentation , Spine/surgery , Humans , ReoperationABSTRACT
Diastematomyelia, or split cord malformation, a complete or incomplete sagittal division of the neural axis into halves, is seen in association with many other congenital anomalies. Among these anomalies, intradural spinal teratoma is extremely rare. Diastematomyelia is a well-recognized although unusual clinical syndrome in children, but it is rarely reported in the adult. The authors describe a 42-year-old man who presented with pain and distal left-leg weakness as well as neurogenic claudication for 1 month. The patient underwent radiological examinations, and diastematomyelia and an intradural lumbar teratoma were diagnosed. He underwent surgery and was followed for 1 year. This is the fourth case of an adult who simultaneously presented with diastematomyelia and an intradural teratoma.
