Surgical Burden of Cutaneous Neurofibromas in Neurofibromatosis Type 1, Patterns of Head and Neck Involvement, and Factors Associated With Multiple Surgeries.

Authors

Juan I Isaac, Cameron Ward, Aidin A Gharavi, Waleed Gibreel, Jose A Castro Garcia

Published in

The Journal of craniofacial surgery. Aug 31, 2026. Epub Aug 31, 2026.

Abstract

Cutaneous neurofibromas (cNF) are common in neurofibromatosis type 1 (NF1) and may require repeated procedural management, yet practical data on cNF-specific surgical burden remain limited. We characterized surgical event burden, anatomic distribution, recurrence patterns, and clinical correlates among NF1 patients undergoing surgery specifically for cNF.
We performed a retrospective chart review of patients with NF1 evaluated at a tertiary academic center from 1990 to 2024. Patients were included if they underwent at least one institutionally documented surgical procedure primarily for cNF. Procedures for plexiform neurofibromas or other non-cutaneous NF1 manifestations were excluded. Surgical events, anatomic sites, indications, recurrence, and clinical features were abstracted and analyzed using descriptive statistics, Wilcoxon rank-sum tests, Fisher exact tests, Spearman correlation, and exploratory negative binomial regression.
Thirty-five patients underwent 88 cNF surgical events. Median events per patient were 2.0, and 9 patients were classified as having a high surgical burden. Pain was the most common documented indication. The trunk, arm, scalp, forehead, and cheeks contributed most to cumulative site-specific procedures. Greater proportional head and neck involvement was associated with lower total event burden, suggesting higher-volume disease was driven primarily by broader non-head and neck involvement. Site-specific recurrence was frequent and strongly correlated with total event burden. Chart-documented recurrence was associated with both higher event counts and high-burden status, whereas skeletal involvement, surgical follow-up duration, and age at first surgery were not robust predictors.
In this cNF-specific NF1 surgical cohort, cumulative operative burden was driven primarily by recurrent procedures across multiple anatomic sites rather than by skeletal phenotype, age at first surgery, or duration of follow-up. These findings suggest that documented recurrence and multisite involvement may help identify patients at risk for repeated surgical intervention. Distinguishing high-volume disease affecting the trunk and extremities from high-impact head and neck disease may improve patient counseling, longitudinal operative planning, and future efforts to reduce cumulative surgical burden in NF1.

PMID:
42673529
Bibliographic data and abstract were imported from PubMed on 01 Sep 2026.

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