Secondary meningioma in a long-term survivor of atypical teratoid/rhabdoid tumour with a germline INI1 mutation

OBJECTIVE: We report on a patient who developed a meningioma more than two decades after removal at a young age of an atypical teratoid/rhabdoid tumour (AT/RT), which was due to a germline INI1 mutation, and radio- and chemotherapy. MATERIALS AND METHODS: We present genetic evidence that the meningioma is not a recurrence or metastasis of the AT/RT and not due to the INI1 mutation, but is a radiation-induced tumour. CONCLUSION: This is the first case illustrating that improved survival of young patients with an AT/RT after aggressive treatment may be gained at the cost of an increased risk for the development of radiation-induced, non-INI1-related tumours

Tuberculum sellae meningiomas: surgical considerations

We report our experience on the treatment of tuberculum sellae meningiomas (TSMs) regarding the involvement of the optic canal and clinical outcomes. We reviewed 23 patients who were operated on between January 1997 and December 2008. The surgical approach was unilateral subfrontal supraorbital osteotomy in one piece. Attempts were made to improve visual function via extra/intradural unroofing of the optic canal, which released the optic nerve. Visual symptoms were present preoperatively in 21 patients, and two patients were asymptomatic. Visual acuity remained intact in 6 patients, improved in 10, was unchanged in 5 patients, and worsened in 2 patients. The postoperative visual field was normal or improved in 17 patients, unchanged in four patients, and worsened in two patients. The optic canal and clinoid were drilled extradurally in eight patients and intradurally in nine patients. Total resection of TSMs was achieved in 19 patients. Incomplete resection occurred in two patients. Decompression of the optic canal seemed to increase the visual outcome.Apresentamos nossa experiência em 23 pacientes operados com meningiomas do tubérculo da sela, com enfoque na descompressão do nervo óptico e nos sintomas visuais do pós-operatório. Vinte e três pacientes com meningiomas do tubérculo da sela foram operados entre janeiro de 1997 e dezembro de 2008, através do acesso subfrontal via ostetomia supraorbital. Remoção do teto do canal óptico por via extra ou intradural foi realizada em 17 pacientes. Sintomas visuais no pré-operatório ocorreram em 21 pacientes, 2 eram assintomáticos. Melhora visual ocorreu em 10, permaneceu inalterada em 5 e piorou em 2 pacientes. No pós-operatório o campo visual normalizou-se em 17 pacientes, permaneceu inalterado em 4 e diminuiu em 2. Descompressão dos nervos ópticos foi realizada em 17 pacientes. Ressecção total dos meningiomas do tubérculo da sela foi possível em 19 pacientes. Abertura do canal óptico permitiu a manipulação do nervo óptico sem novos déficites