Growth behavior of plexiform neurofibromas after surgery
Standard
Growth behavior of plexiform neurofibromas after surgery. / Nguyen, Rosa; Ibrahim, Chadi; Friedrich, Reinhard E; Westphal, Manfred; Schuhmann, Martin; Mautner, Victor-Felix.
in: GENET MED, Jahrgang 15, Nr. 9, 01.09.2013, S. 691-7.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
Harvard
APA
Vancouver
Bibtex
}
RIS
TY - JOUR
T1 - Growth behavior of plexiform neurofibromas after surgery
AU - Nguyen, Rosa
AU - Ibrahim, Chadi
AU - Friedrich, Reinhard E
AU - Westphal, Manfred
AU - Schuhmann, Martin
AU - Mautner, Victor-Felix
PY - 2013/9/1
Y1 - 2013/9/1
N2 - PURPOSE: The aim of this study was to analyze growth rate and identify prognostic factors for progression of postoperative plexiform neurofibromas in patients with neurofibromatosis type 1.METHODS: We measured postoperative tumor volume change per year on magnetic resonance imaging. Linear regression models were applied to identify risk factors for tumor progression.RESULTS: Fifty-two patients (mean age: 25.4 years (3.2-64.2 years)) with 56 plexiform neurofibromas were analyzed. Initial median tumor volume was 40.3 ml (SD: 1,552 (0-10,800)). Surgical indications included disfigurement (n = 21), pain (n = 20), and functional deficits (n = 16). Sixteen percent of all cases experienced acute surgical complication, and 13% showed late complication. Eight patients (19%; 6 children and 2 adults) with residual tumor had repeat surgery for tumor progression. Median tumor progression was 0.6% change per year (SD ± 27.4; range: -59.2 to 88.1) and 2.9% from baseline (SD ± 163.9; range -1,001.3 to 81.8). Patients aged 21 years and younger had the highest progression rate (P < 0.01). For every year of age, the mean growth rate decreased by -0.463 mean percent (P = 0.03). With age as a continuous variable, age, the site of the tumor, and depth were the only factors associated with tumor progression. Fourteen plexiform neurofibromas (10 nodular and 4 diffuse) in 13 patients (5 children and 8 adults) were completely resected (by visualization) and did not relapse during observation (mean: 2.9 years; range: 1.1-5.8 years).CONCLUSION: Age, tumor type, location, and depth are helpful to estimate the progression of plexiform neurofibromas after surgery. Patients benefit from elective surgery of small and completely removable plexiform neurofibromas.
AB - PURPOSE: The aim of this study was to analyze growth rate and identify prognostic factors for progression of postoperative plexiform neurofibromas in patients with neurofibromatosis type 1.METHODS: We measured postoperative tumor volume change per year on magnetic resonance imaging. Linear regression models were applied to identify risk factors for tumor progression.RESULTS: Fifty-two patients (mean age: 25.4 years (3.2-64.2 years)) with 56 plexiform neurofibromas were analyzed. Initial median tumor volume was 40.3 ml (SD: 1,552 (0-10,800)). Surgical indications included disfigurement (n = 21), pain (n = 20), and functional deficits (n = 16). Sixteen percent of all cases experienced acute surgical complication, and 13% showed late complication. Eight patients (19%; 6 children and 2 adults) with residual tumor had repeat surgery for tumor progression. Median tumor progression was 0.6% change per year (SD ± 27.4; range: -59.2 to 88.1) and 2.9% from baseline (SD ± 163.9; range -1,001.3 to 81.8). Patients aged 21 years and younger had the highest progression rate (P < 0.01). For every year of age, the mean growth rate decreased by -0.463 mean percent (P = 0.03). With age as a continuous variable, age, the site of the tumor, and depth were the only factors associated with tumor progression. Fourteen plexiform neurofibromas (10 nodular and 4 diffuse) in 13 patients (5 children and 8 adults) were completely resected (by visualization) and did not relapse during observation (mean: 2.9 years; range: 1.1-5.8 years).CONCLUSION: Age, tumor type, location, and depth are helpful to estimate the progression of plexiform neurofibromas after surgery. Patients benefit from elective surgery of small and completely removable plexiform neurofibromas.
KW - Adolescent
KW - Adult
KW - Aged
KW - Aged, 80 and over
KW - Child
KW - Child, Preschool
KW - Female
KW - Head and Neck Neoplasms
KW - Humans
KW - Linear Models
KW - Magnetic Resonance Imaging
KW - Male
KW - Middle Aged
KW - Neoplasm Recurrence, Local
KW - Neurofibroma, Plexiform
KW - Neurofibromatosis 1
KW - Retrospective Studies
KW - Young Adult
U2 - 10.1038/gim.2013.30
DO - 10.1038/gim.2013.30
M3 - SCORING: Journal article
C2 - 23598713
VL - 15
SP - 691
EP - 697
JO - GENET MED
JF - GENET MED
SN - 1098-3600
IS - 9
ER -