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Dermatologic Manifestations of Neurofibromatosis Type 1 and Emerging Treatments

by Dina Poplausky, Jade N. Young, Hansen Tai, Ryan Rivera-Oyola, Nicholas Gulati and Rebecca M. Brown

Cancers 2023, 15(10), 2770; https://doi.org/10.3390/cancers15102770 - 16 May 2023

Cited by 14 | Viewed by 18380

Abstract

Neurofibromatosis type 1 (NF1) is an autosomal dominant tumor predisposition syndrome that increases one’s risk for both benign and malignant tumors. NF1 affects every organ in the body, but the most distinctive symptoms that are often the most bothersome to patients are the cutaneous manifestations, which can be unsightly, cause pain or pruritus, and have limited therapeutic options. In an effort to increase awareness of lesser-known dermatologic associations and to promote multidisciplinary care, we conducted a narrative review to shed light on dermatologic associations of NF1 as well as emerging treatment options. Topics covered include cutaneous neurofibromas, plexiform neurofibromas, diffuse neurofibromas, distinct nodular lesions, malignant peripheral nerve sheath tumors, glomus tumors, juvenile xanthogranulomas, skin cancer, and cutaneous T-cell lymphoma. Full article

(This article belongs to the Special Issue Frontiers in Neurofibromatosis)

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10 pages, 958 KB

Open AccessArticle

Surgical Treatment and Complications of Deep-Seated Nodular Plexiform Neurofibromas Associated with Neurofibromatosis Type 1

by Kunihiro Ikuta, Yoshihiro Nishida, Tomohisa Sakai, Hiroshi Koike, Kan Ito, Hiroshi Urakawa and Shiro Imagama

J. Clin. Med. 2022, 11(19), 5695; https://doi.org/10.3390/jcm11195695 - 26 Sep 2022

Cited by 8 | Viewed by 6213

Abstract

Background: Nodular plexiform neurofibromas in individuals with neurofibromatosis type 1 often cause significant symptoms and are treated with surgical excision despite the potential risk of complications. This study aimed to clarify the surgical outcomes of deep-seated nodular plexiform neurofibromas and identify the factors associated with postoperative complications. Methods: We retrospectively reviewed patients with neurofibromatosis type 1 who underwent surgical excision for deep-seated nodular plexiform neurofibromas in our hospital from 2015 to 2021. Enucleation while preserving the nerve fascicles was attempted first, and en bloc resection, ligating the nerve origin in cases in which the parent nerve was entrapped by the tumor, making the tumor difficult to dissect, was performed. Results: In 15 patients, 24 nodular plexiform neurofibromas received surgical excision. Sixteen tumors were enucleated, and eight were en bloc resected. The symptoms of all 10 patients with preoperative symptoms resolved after surgery. Four patients developed new neurological deficits immediately after surgery, two of whom had retained neurological symptoms at the last visit, but these symptoms were mild. Conclusions: The present study demonstrates that surgical treatment of nodular plexiform neurofibromas, even deep-seated neurofibromas, is safe with a low risk of severe complications and improvement in preoperative symptoms. Full article

(This article belongs to the Special Issue Neurocutaneous Disorders – Advances in Diagnosis and Treatment)

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