Longitudinal evaluation of peripheral nerve sheath tumors in neurofibromatosis type 1: growth analysis of plexiform neurofibromas and distinct nodular lesions.

Neuro-oncology
Srivandana AkshintalaBrigitte C Widemann

Abstract

Understanding the natural history of non-malignant peripheral nerve sheath tumors (PNSTs) in neurofibromatosis type 1 (NF1) is critical to optimal clinical care and the development of meaningful clinical trials. We longitudinally analyzed growth of plexiform neurofibromas (PNs) and of PNSTs with distinct nodular appearance (distinct nodular lesions [DNLs]) using volumetric MRI analysis in patients enrolled on a natural history study (NCT00924196). DNLs were observed in 58/122 (45.6%) patients (median 2 DNLs/patient). In DNLs that developed during follow-up, median age of development was 17 years. A moderate negative correlation was observed between the estimated PN growth rate and patients' age at initial MRI (Spearman's r [95% CI]: -0.60 [-0.73, -0.43], n = 70), whereas only a weak correlation was observed for DNLs (Spearman's r [95% CI]: -0.25 [-0.47, 0.004]; n = 61). We observed a moderate negative correlation between tumor growth rate and baseline tumor volume for PNs and DNLs (Spearman's r [95% CI]: -0.52 [-0.67, -0.32] and -0.61 [-0.75, -0.42], respectively). Spontaneous tumor volume reduction was observed in 10 PNs and 7 DNLs (median decrease per year, 3.6% and 7.3%, respectively). We corroborate previously described fin...Continue Reading

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Citations

Jun 14, 2020·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Hildegard Kehrer-SawatzkiVictor-Felix Mautner
Jul 1, 2020·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Eric Legius, Hilde Brems
Jun 22, 2020·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Ben ShoftyShlomi Constantini
Jul 2, 2020·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Andrea M GrossBrigitte C Widemann
Mar 23, 2021·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Helen ToledanoMika Shapira Rootman
Jul 3, 2021·Cancers·Laurence PacotEric Pasmant

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