1/2. Differential grading of endolymphatic sac tumor extension by virtue of von hippel-lindau disease status.OBJECTIVE: endolymphatic sac tumors are aggressive papillary tumors of the temporal bone frequently associated with von hippel-lindau disease. The goal of this study was to use a newly devised classification system as a means to analyze differences between endolymphatic sac tumor extension in von hippel-lindau disease and non-von hippel-lindau disease patients. methods: Previously reported cases of endolymphatic sac tumor and two new cases were retrospectively reviewed and assigned to a new classification system consisting of four grades based on tumor extent and location. RESULTS: Mean age of 103 patients without von hippel-lindau disease was 52.5 years, whereas in 46 patients with VHL the mean age was 31.3 years. patients with von hippel-lindau disease were more likely to be female (female-to male ratio of 2:1 for von hippel-lindau disease patients versus 1:1 for non-von hippel-lindau disease patients). Symptoms consisted of hearing loss (100% [mean duration, 10 yr] for VHL patients versus 97% [mean duration, 7.8 yr] for non-von hippel-lindau disease patients), facial weakness (38% versus 49%), and tinnitus or vertigo (41% versus 60%). Bilateral tumors were common in von hippel-lindau disease patients (28% versus 1%). Tumors in von hippel-lindau disease patients were significantly more likely to be lower grade than tumors in non-von hippel-lindau disease patients (Grade I, 40% versus 25%; Grade II, 50% versus 58%; Grade III, 8% versus 14%; and Grade IV, 2% versus 4%; p < 0.05). Before 1988, there were relatively fewer Grade I (15% versus 33%) and relatively more Grade II (69% versus 47%) endolymphatic sac tumors in non-von hippel-lindau disease patients than after 1988. CONCLUSIONS: Increased usefulness of intracranial imaging since 1988 has led to the diagnosis of sporadic endolymphatic sac tumors with lower grades. Surveillance imaging in von hippel-lindau disease may account for the greater proportion of endolymphatic sac tumors diagnosed with lower grades. endolymphatic sac tumors associated with a diagnosis of von hippel-lindau disease appear to affect a younger population of patients than non-von hippel-lindau disease cases and occur in women twice as often as in men when associated with von hippel-lindau disease. In addition, tumors are more frequently bilateral and less advanced in the von hippel-lindau disease patient as opposed to the non-von hippel-lindau disease patient.- - - - - - - - - - ranking = 1keywords = von (Clic here for more details about this article) |
2/2. association of aortic arch anomalies and subclavian artery supply disruption with neurofibromatosis.We report an 8-year-old Japanese girl with von Recklinghausen disease, who presented with aortic arch anomalies and left hemilateral oculo-otolaryngeal abnormalities including strabismus, blepharoptosis, a dysplastic external ear and hearing loss. The aortic arch anomalies including subclavian artery obstruction that appeared to be a consequence of the neurofibromatosis, and the hemilateral oculo-otolaryngeal abnormalities could be explained by disruption of the subclavian artery supply during embryogenesis.- - - - - - - - - - ranking = 0.045454545454545keywords = von (Clic here for more details about this article) |