Evidence map›Paper›PMID 36332985›Full record

ArticleNeurology2023

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

K Ina Ly, Vanessa L Merker, Wenli Cai, Miriam A Bredella, Alona Muzikansky, Raquel D Thalheimer, Jennifer Liwei Da, Christina C Orr, Hamilton P Herr, Mary E Morris and 4 more

Open access · greenAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Neurology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.

0numbers the graph read from it
0cells of the map it votes in
15citing papers in PubMed
1.9field-weighted citation impact, top 13% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

15 citing papers in PubMed, 17 citations in OpenAlex.

  1. Trial
  2. Article
  3. Management of plexiform neurofibromas in neurofibromatosis type 1: An Italian Delphi consensus.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2026
    Article
  4. Article
  5. Review
  6. Article
  7. Article
  8. Review
  9. Article
  10. Article
  11. Review
  12. Article
  13. Contemporary Approach to Neurofibromatosis Type 1-Associated Malignant Peripheral Nerve Sheath Tumors.American Society of Clinical Oncology educational book. American Society of Clinical Oncology. Annual Meeting · 2024
    Review
  14. Review
  15. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

14 authors at 1 institution in 1 country.

K Ina LyFrom 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.
Vanessa L MerkerFrom 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.ORCID 0000-0002-4542-5227
Wenli CaiFrom 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.ORCID 0000-0001-5883-5383
Miriam A BredellaFrom 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.ORCID 0000-0001-5482-0440
Alona MuzikanskyFrom 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.ORCID 0000-0001-7585-8181
Raquel D ThalheimerFrom 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.
Jennifer Liwei DaFrom 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.
Christina C OrrFrom 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.
Hamilton P HerrFrom 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.
Mary E MorrisFrom 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.
Connie Y ChangFrom 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.
Gordon J HarrisFrom 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.
Scott R PlotkinFrom 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.ORCID 0000-0002-6109-6419
Justin T JordanFrom 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.
Massachusetts General Hospital · US

Funding

Cloud quantitative imaging for whole-body tumor burden in neurofibromatosesR42CA192600 · NCI · IQ MEDICAL IMAGING, LLC · PI CAI, WENLI, WU, YIN · 2018 to 2019
$1.5M
NCI NIH HHS R42 CA192600
6 · The paper itself

Abstract

BACKGROUND AND

objectivesInternal 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.

methodsIn 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.

resultsOf 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.

Indexed as

NeurofibromaNeurofibroma, PlexiformNeurofibromatosis 1AdolescentAdultAgedChildFollow-Up StudiesHumansMagnetic Resonance ImagingMiddle AgedProspective StudiesQuality of LifeYoung Adult

Identifiers

PMID36332985
PMCPMC9969927
OpenAlexW4308256826

What OpenQuestion holds

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.