Robust surgical approach for cutaneous neurofibroma in neurofibromatosis type 1

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Abstract

BACKGROUND Cutaneous neurofibromas (cNFs) are physically disfiguring, are painful, and cause extensive psychologic harm in patients with neurofibromatosis type 1 (NF1). There is currently no effective medical treatment, and surgical procedures are inaccessible to most NF1 patients globally. Although research is underway to find an effective medical treatment for cNF, there is an urgent need to develop a surgical approach that is accessible to all NF1 patients worldwide with the skill set and equipment found in most general medical office settings. Here, we present a robust surgical approach to remove cNFs that does not require a sterile surgical field, uses accessible clinical equipment, and can be performed by any health care provider, including family practitioners and physician assistants.METHODS In a prospective case series, patients with NF1 underwent this surgical procedure, which removes multiple cNFs. The Dermatology Life Quality Index was given to subjects before and after the procedure as a surrogate measurement of patient satisfaction.RESULTS Eighty-three tumors were removed throughout the body from 12 individuals. Examination at follow-up visits revealed well-healed scars without infection or adverse events, including aberrant scarring. Patient satisfaction with the procedure was high, with significant improvements in symptoms, daily activities, leisure, personal relationships, and treatment experience (P = 0.00062).CONCLUSION This study demonstrates a robust surgical approach to manage cNFs, which can be accessed worldwide by individuals with NF1 and performed by a wide variety of medical specialists with high clinical efficacy and patient satisfaction.FUNDING The Burroughs Wellcome Fund, the National Cancer Institute of the NIH, the Neurofibromatosis Therapeutic Acceleration Program, the NF1 Research Consortium Fund, and the Giorgio Foundation.

Authors

Bahir H. Chamseddin, La’Nette Hernandez, Dezehree Solorzano, Juan Vega, Lu Q. Le