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A Rare Tumor in the Neck of a Child: Plexiform Neurofibroma

Plexiform neurofibroma represents an uncommon variant of neurofibromatosis type 1, constituting only 5%–30% of all cases. Plexiform neurofibroma is usually diagnosed during childhood and arises from multiple nerves, manifesting as bulging and deforming masses that can also involve connective tissue...

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Main Authors: Siti Sarah Mohd Ramli, Goh, Bee See, Faizah Mohd Zaki, Suria Hayati Md Pauzi, Mohd Fariz Amri
Format: Article
Language:English
English
Published: Galenos Publishing House 2021
Subjects:
RC254-282 Neoplasms. Tumors. Oncology Including cancer and carcinogens
RJ370-550 Diseases of children and adolescents
Online Access:https://eprints.ums.edu.my/id/eprint/35234/1/Abstract.pdf
https://eprints.ums.edu.my/id/eprint/35234/2/Full%20text.pdf
https://eprints.ums.edu.my/id/eprint/35234/
https://cms.galenos.com.tr/Uploads/Article_50940/tao-59-297-En.pdf
https://doi.org/10.4274/tao.2021.2021-3-10
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spelling my.ums.eprints.352342023-03-23T02:13:17Z https://eprints.ums.edu.my/id/eprint/35234/ A Rare Tumor in the Neck of a Child: Plexiform Neurofibroma Siti Sarah Mohd Ramli Goh, Bee See Faizah Mohd Zaki Suria Hayati Md Pauzi Mohd Fariz Amri RC254-282 Neoplasms. Tumors. Oncology Including cancer and carcinogens RJ370-550 Diseases of children and adolescents Plexiform neurofibroma represents an uncommon variant of neurofibromatosis type 1, constituting only 5%–30% of all cases. Plexiform neurofibroma is usually diagnosed during childhood and arises from multiple nerves, manifesting as bulging and deforming masses that can also involve connective tissue and skin folds. We report a case of a two-year-old girl who presented with worsening stridor since birth and later exhibited progressively increasing left neck swelling at the age of 10 months old. Ultrasound and magnetic resonance imaging (MRI) showed a lobulated solid mass in the left deep neck space extending to the midline and having a mass effect on the airway with involvement of the supraglottic region. Tracheostomy was done, and a biopsy of the supraglottic lesion revealed a plexiform neurofibroma. The patient was conservatively managed after a discussion with her parents concerning the associated potential of operative morbidity. The patient’s parents had learned about tracheostomy care, and the patient was scheduled for yearly MRI surveillance. MRI was performed again three months after the initial diagnosis and showed stable lesion. Plexiform neurofibroma is a slow-growing tumor. A treatment decision must consider the benefits of surgery and the morbidity of the progressing disease. Hence, airway management is crucial prior to the final decision of such cases Galenos Publishing House 2021 Article PeerReviewed text en https://eprints.ums.edu.my/id/eprint/35234/1/Abstract.pdf text en https://eprints.ums.edu.my/id/eprint/35234/2/Full%20text.pdf Siti Sarah Mohd Ramli and Goh, Bee See and Faizah Mohd Zaki and Suria Hayati Md Pauzi and Mohd Fariz Amri (2021) A Rare Tumor in the Neck of a Child: Plexiform Neurofibroma. Turkish Archives of Otorhinolaryngology, 59. pp. 297-301. ISSN 2667-7474 https://cms.galenos.com.tr/Uploads/Article_50940/tao-59-297-En.pdf https://doi.org/10.4274/tao.2021.2021-3-10
institution Universiti Malaysia Sabah
building UMS Library
collection Institutional Repository
continent Asia
country Malaysia
content_provider Universiti Malaysia Sabah
content_source UMS Institutional Repository
url_provider http://eprints.ums.edu.my/
language English
English
topic RC254-282 Neoplasms. Tumors. Oncology Including cancer and carcinogens
RJ370-550 Diseases of children and adolescents
spellingShingle RC254-282 Neoplasms. Tumors. Oncology Including cancer and carcinogens
RJ370-550 Diseases of children and adolescents
Siti Sarah Mohd Ramli
Goh, Bee See
Faizah Mohd Zaki
Suria Hayati Md Pauzi
Mohd Fariz Amri
A Rare Tumor in the Neck of a Child: Plexiform Neurofibroma
description Plexiform neurofibroma represents an uncommon variant of neurofibromatosis type 1, constituting only 5%–30% of all cases. Plexiform neurofibroma is usually diagnosed during childhood and arises from multiple nerves, manifesting as bulging and deforming masses that can also involve connective tissue and skin folds. We report a case of a two-year-old girl who presented with worsening stridor since birth and later exhibited progressively increasing left neck swelling at the age of 10 months old. Ultrasound and magnetic resonance imaging (MRI) showed a lobulated solid mass in the left deep neck space extending to the midline and having a mass effect on the airway with involvement of the supraglottic region. Tracheostomy was done, and a biopsy of the supraglottic lesion revealed a plexiform neurofibroma. The patient was conservatively managed after a discussion with her parents concerning the associated potential of operative morbidity. The patient’s parents had learned about tracheostomy care, and the patient was scheduled for yearly MRI surveillance. MRI was performed again three months after the initial diagnosis and showed stable lesion. Plexiform neurofibroma is a slow-growing tumor. A treatment decision must consider the benefits of surgery and the morbidity of the progressing disease. Hence, airway management is crucial prior to the final decision of such cases
format Article
author Siti Sarah Mohd Ramli
Goh, Bee See
Faizah Mohd Zaki
Suria Hayati Md Pauzi
Mohd Fariz Amri
author_facet Siti Sarah Mohd Ramli
Goh, Bee See
Faizah Mohd Zaki
Suria Hayati Md Pauzi
Mohd Fariz Amri
author_sort Siti Sarah Mohd Ramli
title A Rare Tumor in the Neck of a Child: Plexiform Neurofibroma
title_short A Rare Tumor in the Neck of a Child: Plexiform Neurofibroma
title_full A Rare Tumor in the Neck of a Child: Plexiform Neurofibroma
title_fullStr A Rare Tumor in the Neck of a Child: Plexiform Neurofibroma
title_full_unstemmed A Rare Tumor in the Neck of a Child: Plexiform Neurofibroma
title_sort rare tumor in the neck of a child: plexiform neurofibroma
publisher Galenos Publishing House
publishDate 2021
url https://eprints.ums.edu.my/id/eprint/35234/1/Abstract.pdf
https://eprints.ums.edu.my/id/eprint/35234/2/Full%20text.pdf
https://eprints.ums.edu.my/id/eprint/35234/
https://cms.galenos.com.tr/Uploads/Article_50940/tao-59-297-En.pdf
https://doi.org/10.4274/tao.2021.2021-3-10
_version_ 1761617770261250048
score 13.211869

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