Abstract
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.
Highlights
The neurofibromatoses, including neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis, are a group of related tumor suppressor syndromes that share a predisposition to develop multiple nerve sheath tumors
In this study, we used whole-body MRI, the Short Form-36 (SF-36), and a visual analog pain scale to examine the effect of internal tumor burden on quality of life in three closely related tumorsuppressor syndromes
Patients with NF2 had deficits primarily in the physical domain, as shown by their significant decrease in multiple physical subdomains of the SF-36 as well as the physical component summary score compared to the general population
Summary
The neurofibromatoses, including neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis, are a group of related tumor suppressor syndromes that share a predisposition to develop multiple nerve sheath tumors. Using WBMRI, along with the SF-36 and a visual analog pain scale (VAS), we assessed the association between tumor burden and quality of life in adults with NF1, NF2, or schwannomatosis. Conclusions: Patients with NF1, NF2, and schwannomatosis suffer from reduced QOL, 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
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