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

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 neurofibrom...

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Autores principales: Ikuta, Kunihiro, Nishida, Yoshihiro, Sakai, Tomohisa, Koike, Hiroshi, Ito, Kan, Urakawa, Hiroshi, Imagama, Shiro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9571780/
https://www.ncbi.nlm.nih.gov/pubmed/36233563
http://dx.doi.org/10.3390/jcm11195695
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author Ikuta, Kunihiro
Nishida, Yoshihiro
Sakai, Tomohisa
Koike, Hiroshi
Ito, Kan
Urakawa, Hiroshi
Imagama, Shiro
author_facet Ikuta, Kunihiro
Nishida, Yoshihiro
Sakai, Tomohisa
Koike, Hiroshi
Ito, Kan
Urakawa, Hiroshi
Imagama, Shiro
author_sort Ikuta, Kunihiro
collection PubMed
description 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.
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spelling pubmed-95717802022-10-17 Surgical Treatment and Complications of Deep-Seated Nodular Plexiform Neurofibromas Associated with Neurofibromatosis Type 1 Ikuta, Kunihiro Nishida, Yoshihiro Sakai, Tomohisa Koike, Hiroshi Ito, Kan Urakawa, Hiroshi Imagama, Shiro J Clin Med Article 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. MDPI 2022-09-26 /pmc/articles/PMC9571780/ /pubmed/36233563 http://dx.doi.org/10.3390/jcm11195695 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Ikuta, Kunihiro
Nishida, Yoshihiro
Sakai, Tomohisa
Koike, Hiroshi
Ito, Kan
Urakawa, Hiroshi
Imagama, Shiro
Surgical Treatment and Complications of Deep-Seated Nodular Plexiform Neurofibromas Associated with Neurofibromatosis Type 1
title Surgical Treatment and Complications of Deep-Seated Nodular Plexiform Neurofibromas Associated with Neurofibromatosis Type 1
title_full Surgical Treatment and Complications of Deep-Seated Nodular Plexiform Neurofibromas Associated with Neurofibromatosis Type 1
title_fullStr Surgical Treatment and Complications of Deep-Seated Nodular Plexiform Neurofibromas Associated with Neurofibromatosis Type 1
title_full_unstemmed Surgical Treatment and Complications of Deep-Seated Nodular Plexiform Neurofibromas Associated with Neurofibromatosis Type 1
title_short Surgical Treatment and Complications of Deep-Seated Nodular Plexiform Neurofibromas Associated with Neurofibromatosis Type 1
title_sort surgical treatment and complications of deep-seated nodular plexiform neurofibromas associated with neurofibromatosis type 1
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9571780/
https://www.ncbi.nlm.nih.gov/pubmed/36233563
http://dx.doi.org/10.3390/jcm11195695
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