Dysplasia of the orbit and adjacent bone associated with plexiform neurofibroma and ocular disease in 42 NF-1 patients.
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Dysplasia of the orbit and adjacent bone associated with plexiform neurofibroma and ocular disease in 42 NF-1 patients. / Friedrich, Reinhard; Stelljes, Claudia; Hagel, Christian; Giese, Manfred; Scheuer, Hanna.
in: ANTICANCER RES, Jahrgang 30, Nr. 5, 5, 2010, S. 1751-1764.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Dysplasia of the orbit and adjacent bone associated with plexiform neurofibroma and ocular disease in 42 NF-1 patients.
AU - Friedrich, Reinhard
AU - Stelljes, Claudia
AU - Hagel, Christian
AU - Giese, Manfred
AU - Scheuer, Hanna
PY - 2010
Y1 - 2010
N2 - Neurofibromas are the hallmark of neurofibromatosis type 1 (NF1). Interestingly, generalised and localised interference or dysfunction of bone is also a key element of the NF1 phenotype. In the skull, NF1-associated orbital dysplasia often results in a severe disfigurement of affected individuals. However, the underlying pathology of orbital dysplasia is a complex phenomenon and up to now poorly understood. This study was performed to describe the orbit in 42 NF1 patients with large, disfiguring soft-tissue tumour of the orbital/eyelid region (plexiform neurofibroma (PNF)). A dysplastic orbit on the affected side was diagnosed in 80.9%. Orbital PNF extension to adjacent regions revealed a significant correlation of orbit and temporal region (0.33, p0.011), oral cavity and nose (0.35, p
AB - Neurofibromas are the hallmark of neurofibromatosis type 1 (NF1). Interestingly, generalised and localised interference or dysfunction of bone is also a key element of the NF1 phenotype. In the skull, NF1-associated orbital dysplasia often results in a severe disfigurement of affected individuals. However, the underlying pathology of orbital dysplasia is a complex phenomenon and up to now poorly understood. This study was performed to describe the orbit in 42 NF1 patients with large, disfiguring soft-tissue tumour of the orbital/eyelid region (plexiform neurofibroma (PNF)). A dysplastic orbit on the affected side was diagnosed in 80.9%. Orbital PNF extension to adjacent regions revealed a significant correlation of orbit and temporal region (0.33, p0.011), oral cavity and nose (0.35, p
M3 - SCORING: Zeitschriftenaufsatz
VL - 30
SP - 1751
EP - 1764
JO - ANTICANCER RES
JF - ANTICANCER RES
SN - 0250-7005
IS - 5
M1 - 5
ER -
