ABSTRACT
Gliomatosis cerebri (GC) is a universally fatal extensive and diffuse infiltration of brain parenchyma by a glial tumor. Many aspects of this phenomenon remain unknown. The First International Gliomatosis cerebri Group Meeting had the following goals: refine the clinical and radiologic diagnostic criteria for GC, suggest appropriate diagnostic procedures, standardize tissue manipulation for histologic and molecular characterization, and prioritize relevant preclinical projects. Also, general treatment recommendations were outlined for the pediatric population. Importantly, this meeting was the starting point for meaningful collaborative international research projects. This review is a consensus summary of discussions shared and conclusions derived from this meeting.
Subject(s)
Brain Neoplasms/therapy , Neoplasms, Neuroepithelial/therapy , Brain Neoplasms/diagnosis , Brain Neoplasms/epidemiology , Brain Neoplasms/genetics , Consensus , Epigenesis, Genetic , Humans , Neoplasms, Neuroepithelial/diagnosis , Neoplasms, Neuroepithelial/epidemiology , Neoplasms, Neuroepithelial/genetics , Neuroimaging , PrognosisABSTRACT
The various childhood suprasellar tumors, while pathologically distinct, present similar clinical and surgical challenges as a result of their common anatomic location. These lesions are in close proximity to or may invade the optic nerve and chiasm, pituitary gland and infundibulum, hypothalamus, and third ventricle, leading to presenting features including visual field loss, impairment in visual acuity, endocrine dysfunction, and hydrocephalus. Though many suprasellar lesions are relatively benign in pathology, treatment may be complicated by high surgical morbidity resulting from damage to the hypothalamic-pituitary axis. Here we review the most frequent pediatric lesions occurring in the suprasellar region: craniopharyngioma, chiasmatic glioma, germ cell tumor, Rathke cleft and arachnoid cysts, pituitary adenoma, and histiocytosis. This review outlines both common presenting features and differentiating aspects of these lesions. It also includes classic radiographic presentations and treatment considerations for each lesion.
Subject(s)
Brain Neoplasms/diagnosis , Brain Neoplasms/therapy , Central Nervous System Cysts/diagnosis , Central Nervous System Cysts/therapy , Child , HumansABSTRACT
Gliomatosis cerebri is a rare glial tumor that carries a poor prognosis. Seen in both adults and children, gliomatosis cerebri appears to differ in these populations as with adult versus pediatric glioblastoma. We present 10 children who either presented to the Weill Cornell Medical College or enrolled in the institution's Gliomatosis Cerebri International Registry alongside a cohort of 89 pediatric patients reported in the literature between 2000 and 2014. Age ranged from 4 months to 21 years, with a male to female ratio of 1.71. Median overall survival for patients in the registry cohort was 17 months (n = 10) and for the historic cohort was 13 months (n = 52). Overall survival was analyzed for the combined cohort and was significantly longer when presenting at age ≥ 10 (20 vs 10 months), for boys (18 vs 11 months), and with low-grade pathology (26.5 vs 12 months) but did not vary significantly by treatment approach.
Subject(s)
Brain Neoplasms/therapy , Neoplasms, Neuroepithelial/therapy , Adolescent , Brain Neoplasms/genetics , Brain Neoplasms/metabolism , Brain Neoplasms/pathology , Female , Humans , Male , Neoplasms, Neuroepithelial/genetics , Neoplasms, Neuroepithelial/metabolism , Neoplasms, Neuroepithelial/pathology , Young AdultABSTRACT
BACKGROUND: Plasticity of the developing motor tracts is a contributor to recovery of motor function after pediatric stroke. The mechanism of these plastic changes may be functional and/or structural in nature. The corticospinal tract (CST) represents the major pathway responsible for voluntary movement. Stroke-induced damage to the CST as well as to other motor tracts leads to motor deficits which may show favorable functional recovery particularly in the pediatric population. METHODS: We report the case of a 3-year-old girl demonstrating reorganization of the pyramidal tracts after an extensive left MCA territory stroke secondary to head trauma. Reorganization is characterized using serial diffusion tensor imaging (DTI) of the pyramidal tracts which contain the CST. RESULTS: Imaging shows decreased ipsi-lesional fractional anisotropy (FA) suggestive of Wallerian degeneration and increased contralesional FA. CONCLUSIONS: These results point to plastic reorganization of the pyramidal tract post-stroke and the utility of DTI in recognizing these changes.
