Clinicopathological assay of 15 tumor resections in a family with neurofibromatosis type (CROSBI ID 212780)
Prilog u časopisu | izvorni znanstveni rad | međunarodna recenzija
Podaci o odgovornosti
DiMaio, Salvatore ; Mrak, Goran ; Jurić-Sekhar, Gordana ; Born, Donald ; Mantovani, Alessandra ; Sekhar, Laligam
engleski
Clinicopathological assay of 15 tumor resections in a family with neurofibromatosis type
The objective of this study is the management of multiple family members with multiple neurofibromatosis type 2 (NF2) related tumors of the skull base that can be challenging, on purely technical, decision-making, and ethical levels. These issues are addressed in this manuscript based on an experience treating an unique large family with NF2. A retrospective chart review was performed, reviewing clinical, radiological, surgical, and pathological data. A unique family of 17 siblings, whose father was the proband as a sporadic mutation is reported. Over a 4-month period, five of eight affected siblings underwent 12 procedures for resection of 15 different NF2-related tumors. This single family experience of NF2-related skull base tumors underscores the importance of preservation of function and quality of life as the major determinants of treatment success.
NF2 associated tumors ; ethics ; meningioma ; neurofibromatosis type 2 ; quality of life ; vestibular schwannoma
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Podaci o izdanju
73 (2)
2012.
90-103
objavljeno
2193-6331
2193-634X
10.1055/s-0032-1301394
Povezanost rada
Kliničke medicinske znanosti