Outcome of 11 children with ependymoblastoma treated within the prospective HIT-trials between 1991 and 2006

Ependymoblastoma is a rare malignant brain tumor of early childhood. Data on clinical behavior and optimal treatment strategies are scarce. We report on 11 consecutively treated children with centrally confirmed diagnosis of CNS ependymoblastoma, registered between February 1994 and October 2006 to the prospective GPOH-HIT multicenter brain tumor trials, and treated by multimodal regimens. Median age at diagnosis was 3.5 years (range, 1.8-5.6 years), and the median follow-up of survivors was 5.9 years (range, 2.2-12.7 years). Initial stage was M0 in 9, and M0/1 (no cerebrospinal fluid examination done) in 2 patients. Gross-total tumor resection was achieved in 7 patients, incomplete resection in 4 patients. Further primary therapy included chemotherapy in all patients, craniospinal radiotherapy in 5 patients and high-dose chemotherapy in 2 patients. Tumor response to chemotherapy was observed in 1 of 4 evaluable patients. Tumor progression occurred in 7 patients after a median time of 5.0 months (range, 2.5-19.2 months). Five-year progression-free survival was 36.4% (±14.5%), 5-year overall survival 30.3% (±15.9%). Of 4 survivors, 3 had gross-total tumor resection, and all were treated by either craniospinal radiotherapy and/or high-dose chemotherapy with autologous blood stem cell rescue. Prognosis of children with ependymoblastoma is poor, but sustained remissions have been achieved after multimodal treatment. Considerable diagnostic discrepancies between local and central pathologists underscore the importance of central review. Further studies are needed to improve survival of children with this rare malignant central nervous system tumor.     

Clinical and molecular characterization of isolated M1 disease in pediatric medulloblastoma: experience from the German HIT-MED studies

Journal of Neuro-Oncology - To evaluate the clinical impact of isolated spread of medulloblastoma cells into cerebrospinal fluid without additional macroscopic metastases (M1-only). The HIT-MED...     

CT and MR imaging in atypical teratoid/rhabdoid tumors of the central nervous system

Introduction Atypical teratoid/rhabdoid tumors (ATRT) are rare aggressive neoplasms of the CNS affecting predominantly very young children. Methods We retrospectively reviewed the imaging findings of 9 CT and 32 MR examinations of the brain and spine of 33 children. Results Of the 33 tumors, 11 were located in the infratentorial compartment, 16 in the supratentorial compartment, 5 in both cranial compartments, and 1 in the lower thoracic spinal cord. The mean age of the children with infratentorial or infra- and supratentorial tumors was significantly lower than the mean age of the children with purely supratentorial tumors. Heterogeneity on imaging, large size and high tumor stages are striking features reflecting the aggressive nature of this histopathological entity. Although not present in the majority of children, a distinct and unusual pattern of a wavy band-like enhancement surrounding a central hypointensity was present in 12 of 32 children (38%) in whom contrast medium was used. Conclusion To the best of our knowledge this is the largest number of imaging examinations of ATRTs so far reported. A rather unusual pattern of contrast enhancement may be typical of ATRTs. This work was funded by the German Children’s Cancer Foundation (Deutsche Kinderkrebsstiftung).     

Wegener-Granulomatose

Wegener's granulomatosis is a necrotizing, granulomatous vasculitis that involves the upper and lower respiratory tracts and the kidney. It may also affect the skin, the central and peripheral nervous system and the bowel. We present a case of a 70 year-old woman who underwent bilateral carotid repair. She was readmitted with late rejection of the Dacron patch and necrotizing ulcerative lesions of the neck. The final diagnosis of Wegener's granulomatosis was made with reference to the leukocytoclastic skin lesions and an elevated cANCA titer. We review the symptoms and histopathologic features of Wegener's granulomatosis to explain this unusual postoperative course of carotid repair.