Molecular genetics of neurofibromatosis 2 and related tumors (acoustic neuroma and meningioma).

Meningioma and acoustic neuroma are among the most frequent primary tumors of the central nervous system. They usually arise as sporadic and solitary tumors. They also develop as multiple tumors in the autosomal dominant genetic disorder neurofibromatosis 2 (NF2). Molecular analysis of meningioma and acoustic neuroma revealed that loss of chromosome 22 alleles was the most frequent genetic alteration found in either sporadic or inherited cases. Subsequent studies showed that a marker in the middle of the long arm of chromosome 22 was linked to the disease in NF2 pedigrees. In this paper, the most recent findings concerning the genetics of NF2 and related tumors are reviewed, and strategy to isolate and characterize the NF2 gene is presented.