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Relationship between whole‐body tumor burden, clinical phenotype, and quality of life in patients with neurofibromatosis
Author(s) -
Merker Vanessa L.,
Bredella Miriam A.,
Cai Wenli,
Kassarjian Ara,
Harris Gordon J.,
Muzikansky Alona,
Nguyen Rosa,
Mautner Victor F.,
Plotkin Scott R.
Publication year - 2014
Publication title -
american journal of medical genetics part a
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.064
H-Index - 112
eISSN - 1552-4833
pISSN - 1552-4825
DOI - 10.1002/ajmg.a.36466
Subject(s) - medicine , neurofibromatosis , quality of life (healthcare) , visual analogue scale , population , cohort , physical therapy , oncology , pathology , nursing , environmental health
Patients with neurofibromatosis 1 (NF1), NF2, and schwannomatosis share a predisposition to develop multiple nerve sheath tumors. Previous studies have demonstrated that patients with NF1 and NF2 have reduced quality of life (QOL), but no studies have examined the relationship between whole‐body tumor burden and QOL in these patients. We administered a QOL questionnaire (the SF‐36) and a visual analog pain scale (VAS) to a previously described cohort of adult neurofibromatosis patients undergoing whole‐body MRI. One‐sample t ‐tests were used to compare norm‐based SF‐36 scores to weighted population means. Spearman correlation coefficients and multiple linear regression analyses controlling for demographic and disease‐specific clinical variable were used to relate whole‐body tumor volume to QOL scales. Two hundred forty‐five patients (142 NF1, 53 NF2, 50 schwannomatosis) completed the study. Subjects showed deficits in selected subscales of the SF‐36 compared to adjusted general population means. In bivariate analysis, increased tumor volume was significantly associated with pain in schwannomatosis patients, as measured by the SF‐36 bodily pain subscale (rho = −0.287, P = 0.04) and VAS (rho = 0.34, P = 0.02). Regression models for NF2 patients showed a positive relationship between tumor burden and increased pain, as measured by the SF‐36 ( P = 0.008). Patients with NF1, NF2, and schwannomatosis suffer from reduced QOL, although only pain shows a clear relationship to patient's overall tumor burden. These findings suggest that internal tumor volume is not a primary contributor to QOL and emphasize the need for comprehensive treatment approaches that go beyond tumor‐focused therapies such as surgery by including psychosocial interventions. © 2014 Wiley Periodicals, Inc.