Growth behavior of plexiform neurofibromas after surgery

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Growth behavior of plexiform neurofibromas after surgery. / Nguyen, Rosa; Ibrahim, Chadi; Friedrich, Reinhard E; Westphal, Manfred; Schuhmann, Martin; Mautner, Victor-Felix.

In: GENET MED, Vol. 15, No. 9, 01.09.2013, p. 691-7.

Research output: SCORING: Contribution to journalSCORING: Journal articleResearchpeer-review

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Nguyen, R, Ibrahim, C, Friedrich, RE, Westphal, M, Schuhmann, M & Mautner, V-F 2013, 'Growth behavior of plexiform neurofibromas after surgery', GENET MED, vol. 15, no. 9, pp. 691-7. https://doi.org/10.1038/gim.2013.30

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@article{9af1676499e54b2b982e72103c1c21eb,
title = "Growth behavior of plexiform neurofibromas after surgery",
abstract = "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.",
keywords = "Adolescent, Adult, Aged, Aged, 80 and over, Child, Child, Preschool, Female, Head and Neck Neoplasms, Humans, Linear Models, Magnetic Resonance Imaging, Male, Middle Aged, Neoplasm Recurrence, Local, Neurofibroma, Plexiform, Neurofibromatosis 1, Retrospective Studies, Young Adult",
author = "Rosa Nguyen and Chadi Ibrahim and Friedrich, {Reinhard E} and Manfred Westphal and Martin Schuhmann and Victor-Felix Mautner",
year = "2013",
month = sep,
day = "1",
doi = "10.1038/gim.2013.30",
language = "English",
volume = "15",
pages = "691--7",
journal = "GENET MED",
issn = "1098-3600",
publisher = "Lippincott Williams and Wilkins",
number = "9",

}

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 -