دورية أكاديمية
أعرض في EDS

Ten-Year Follow-up of Internal Neurofibroma Growth Behavior in Adult Patients With Neurofibromatosis Type 1 Using Whole-Body MRI.

التفاصيل البيبلوغرافية
العنوان: Ten-Year Follow-up of Internal Neurofibroma Growth Behavior in Adult Patients With Neurofibromatosis Type 1 Using Whole-Body MRI.
المؤلفون: Ly KI; From the Stephen E. and Catherine Pappas Center for Neuro-Oncology (K.I.L., V.L.M., R.D.T., J.L.D., C.C.O., H.P.H., S.R.P., J.T.J.), Massachusetts General Hospital; Department of Radiology (W.C., M.A.B., C.Y.C., G.J.H.), Massachusetts General Hospital; Biostatistics Center (A.M.), Massachusetts General Hospital; and Department of Obstetrics and Gynecology (M.E.M.), Massachusetts General Hospital, Boston. ily@partners.org., Merker VL; From the Stephen E. and Catherine Pappas Center for Neuro-Oncology (K.I.L., V.L.M., R.D.T., J.L.D., C.C.O., H.P.H., S.R.P., J.T.J.), Massachusetts General Hospital; Department of Radiology (W.C., M.A.B., C.Y.C., G.J.H.), Massachusetts General Hospital; Biostatistics Center (A.M.), Massachusetts General Hospital; and Department of Obstetrics and Gynecology (M.E.M.), Massachusetts General Hospital, Boston., Cai W; From the Stephen E. and Catherine Pappas Center for Neuro-Oncology (K.I.L., V.L.M., R.D.T., J.L.D., C.C.O., H.P.H., S.R.P., J.T.J.), Massachusetts General Hospital; Department of Radiology (W.C., M.A.B., C.Y.C., G.J.H.), Massachusetts General Hospital; Biostatistics Center (A.M.), Massachusetts General Hospital; and Department of Obstetrics and Gynecology (M.E.M.), Massachusetts General Hospital, Boston., Bredella MA; From the Stephen E. and Catherine Pappas Center for Neuro-Oncology (K.I.L., V.L.M., R.D.T., J.L.D., C.C.O., H.P.H., S.R.P., J.T.J.), Massachusetts General Hospital; Department of Radiology (W.C., M.A.B., C.Y.C., G.J.H.), Massachusetts General Hospital; Biostatistics Center (A.M.), Massachusetts General Hospital; and Department of Obstetrics and Gynecology (M.E.M.), Massachusetts General Hospital, Boston., Muzikansky A; From the Stephen E. and Catherine Pappas Center for Neuro-Oncology (K.I.L., V.L.M., R.D.T., J.L.D., C.C.O., H.P.H., S.R.P., J.T.J.), Massachusetts General Hospital; Department of Radiology (W.C., M.A.B., C.Y.C., G.J.H.), Massachusetts General Hospital; Biostatistics Center (A.M.), Massachusetts General Hospital; and Department of Obstetrics and Gynecology (M.E.M.), Massachusetts General Hospital, Boston., Thalheimer RD; From the Stephen E. and Catherine Pappas Center for Neuro-Oncology (K.I.L., V.L.M., R.D.T., J.L.D., C.C.O., H.P.H., S.R.P., J.T.J.), Massachusetts General Hospital; Department of Radiology (W.C., M.A.B., C.Y.C., G.J.H.), Massachusetts General Hospital; Biostatistics Center (A.M.), Massachusetts General Hospital; and Department of Obstetrics and Gynecology (M.E.M.), Massachusetts General Hospital, Boston., Da JL; From the Stephen E. and Catherine Pappas Center for Neuro-Oncology (K.I.L., V.L.M., R.D.T., J.L.D., C.C.O., H.P.H., S.R.P., J.T.J.), Massachusetts General Hospital; Department of Radiology (W.C., M.A.B., C.Y.C., G.J.H.), Massachusetts General Hospital; Biostatistics Center (A.M.), Massachusetts General Hospital; and Department of Obstetrics and Gynecology (M.E.M.), Massachusetts General Hospital, Boston., Orr CC; From the Stephen E. and Catherine Pappas Center for Neuro-Oncology (K.I.L., V.L.M., R.D.T., J.L.D., C.C.O., H.P.H., S.R.P., J.T.J.), Massachusetts General Hospital; Department of Radiology (W.C., M.A.B., C.Y.C., G.J.H.), Massachusetts General Hospital; Biostatistics Center (A.M.), Massachusetts General Hospital; and Department of Obstetrics and Gynecology (M.E.M.), Massachusetts General Hospital, Boston., Herr HP; From the Stephen E. and Catherine Pappas Center for Neuro-Oncology (K.I.L., V.L.M., R.D.T., J.L.D., C.C.O., H.P.H., S.R.P., J.T.J.), Massachusetts General Hospital; Department of Radiology (W.C., M.A.B., C.Y.C., G.J.H.), Massachusetts General Hospital; Biostatistics Center (A.M.), Massachusetts General Hospital; and Department of Obstetrics and Gynecology (M.E.M.), Massachusetts General Hospital, Boston., Morris ME; From the Stephen E. and Catherine Pappas Center for Neuro-Oncology (K.I.L., V.L.M., R.D.T., J.L.D., C.C.O., H.P.H., S.R.P., J.T.J.), Massachusetts General Hospital; Department of Radiology (W.C., M.A.B., C.Y.C., G.J.H.), Massachusetts General Hospital; Biostatistics Center (A.M.), Massachusetts General Hospital; and Department of Obstetrics and Gynecology (M.E.M.), Massachusetts General Hospital, Boston., Chang CY; From the Stephen E. and Catherine Pappas Center for Neuro-Oncology (K.I.L., V.L.M., R.D.T., J.L.D., C.C.O., H.P.H., S.R.P., J.T.J.), Massachusetts General Hospital; Department of Radiology (W.C., M.A.B., C.Y.C., G.J.H.), Massachusetts General Hospital; Biostatistics Center (A.M.), Massachusetts General Hospital; and Department of Obstetrics and Gynecology (M.E.M.), Massachusetts General Hospital, Boston., Harris GJ; From the Stephen E. and Catherine Pappas Center for Neuro-Oncology (K.I.L., V.L.M., R.D.T., J.L.D., C.C.O., H.P.H., S.R.P., J.T.J.), Massachusetts General Hospital; Department of Radiology (W.C., M.A.B., C.Y.C., G.J.H.), Massachusetts General Hospital; Biostatistics Center (A.M.), Massachusetts General Hospital; and Department of Obstetrics and Gynecology (M.E.M.), Massachusetts General Hospital, Boston., Plotkin SR; From the Stephen E. and Catherine Pappas Center for Neuro-Oncology (K.I.L., V.L.M., R.D.T., J.L.D., C.C.O., H.P.H., S.R.P., J.T.J.), Massachusetts General Hospital; Department of Radiology (W.C., M.A.B., C.Y.C., G.J.H.), Massachusetts General Hospital; Biostatistics Center (A.M.), Massachusetts General Hospital; and Department of Obstetrics and Gynecology (M.E.M.), Massachusetts General Hospital, Boston., Jordan JT; From the Stephen E. and Catherine Pappas Center for Neuro-Oncology (K.I.L., V.L.M., R.D.T., J.L.D., C.C.O., H.P.H., S.R.P., J.T.J.), Massachusetts General Hospital; Department of Radiology (W.C., M.A.B., C.Y.C., G.J.H.), Massachusetts General Hospital; Biostatistics Center (A.M.), Massachusetts General Hospital; and Department of Obstetrics and Gynecology (M.E.M.), Massachusetts General Hospital, Boston.
المصدر: Neurology [Neurology] 2023 Feb 14; Vol. 100 (7), pp. e661-e670. Date of Electronic Publication: 2022 Nov 04.
نوع المنشور: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.
اللغة: English
بيانات الدورية: Publisher: Lippincott Williams & Wilkins Country of Publication: United States NLM ID: 0401060 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1526-632X (Electronic) Linking ISSN: 00283878 NLM ISO Abbreviation: Neurology Subsets: MEDLINE
أسماء مطبوعة: Publication: Hagerstown, MD : Lippincott Williams & Wilkins
Original Publication: Minneapolis.
مواضيع طبية MeSH: Neurofibromatosis 1*/complications , Neurofibromatosis 1*/diagnostic imaging , Neurofibromatosis 1*/genetics , Neurofibroma, Plexiform*/diagnostic imaging , Neurofibroma, Plexiform*/pathology , Neurofibroma*/diagnostic imaging, Child ; Young Adult ; Humans ; Aged ; Adolescent ; Adult ; Middle Aged ; Follow-Up Studies ; Prospective Studies ; Quality of Life ; Magnetic Resonance Imaging
مستخلص: Background and Objectives: Internal neurofibromas, including plexiform neurofibromas (PNF), can cause significant morbidity in patients with neurofibromatosis type 1 (NF1). PNF growth is most pronounced in children and young adults, with more rapid growth thought to occur in a subset of PNF termed distinct nodular lesions (DNL). Growth behavior of internal neurofibromas and DNL in older adults is not well documented; yet knowledge thereof is important for patient risk stratification and clinical trial design. The primary objective of this study was to evaluate the long-term growth behavior of internal neurofibromas in adults with NF1. Secondary objectives were to correlate tumor growth behavior with patient-specific, tumor-specific, and patient-reported variables.
Methods: In this prospective cohort study, internal neurofibromas were identified on coronal short TI inversion recovery sequences on baseline and follow-up whole-body MRIs (WBMRIs). Tumor growth and shrinkage were defined as a volume change ≥20%. The association between tumor growth and patient-specific (baseline age, sex, and genotype), tumor-specific (morphology, location, DNL presence on baseline WBMRI, and maximum standardized uptake value on baseline PET imaging), and patient-reported variables (endogenous and exogenous hormone exposure, pain intensity, and quality of life) was assessed using the Spearman correlation coefficient and Kruskal-Wallis test.
Results: Of 106 patients with a baseline WBMRI obtained as part of a previous research study, 44 had a follow-up WBMRI. Three additional patients with WBMRIs acquired for clinical care were included, generating 47 adults for this study. The median age during baseline WBMRI was 42 years (range 18-70). The median time between WBMRIs was 10.4 years. Among 324 internal neurofibromas, 62.8% (56% of PNF and 62.1% of DNL) shrank spontaneously without treatment and 17.1% (17.9% of PNF and 13.8% of DNL) grew. Growth patterns were heterogeneous within participants. Patient-specific, tumor-specific, and patient-reported variables (including endogenous and exogenous hormone exposure) were not strong predictors of tumor growth.
Discussion: Internal neurofibroma growth behavior in older adults differs fundamentally from that in children and young adults, with most tumors, including DNL, demonstrating spontaneous shrinkage. Better growth models are needed to understand factors that influence tumor growth. These results will inform clinical trial design for internal neurofibromas.
(© 2022 American Academy of Neurology.)
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معلومات مُعتمدة: R42 CA192600 United States CA NCI NIH HHS
تواريخ الأحداث: Date Created: 20221104 Date Completed: 20230215 Latest Revision: 20230301
رمز التحديث: 20231215
مُعرف محوري في PubMed: PMC9969927
DOI: 10.1212/WNL.0000000000201535
PMID: 36332985
قاعدة البيانات: MEDLINE
الوصف
تدمد:1526-632X
DOI:10.1212/WNL.0000000000201535