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A rare case report of neurofibromatosis type 1 in a 12-year-old child: A 15-month follow-up
Neurofibromatosis type 1 (NF-1) or von Recklinghausen's disease is a rare genetic disorder characterized by the development of multiple noncancerous (benign) tumors of nerves and skin (neurofibromas). Head-and-neck neurofibroma is generally located in the soft tissue. Here, we present a case of...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Wolters Kluwer - Medknow
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7069136/ https://www.ncbi.nlm.nih.gov/pubmed/32189916 http://dx.doi.org/10.4103/jomfp.JOMFP_35_20 |
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author | Nallanchakrava, Srinivas Mallela, Manoj Kumar Jeenepalli, V Shiva Kumar Niharika, H M |
author_facet | Nallanchakrava, Srinivas Mallela, Manoj Kumar Jeenepalli, V Shiva Kumar Niharika, H M |
author_sort | Nallanchakrava, Srinivas |
collection | PubMed |
description | Neurofibromatosis type 1 (NF-1) or von Recklinghausen's disease is a rare genetic disorder characterized by the development of multiple noncancerous (benign) tumors of nerves and skin (neurofibromas). Head-and-neck neurofibroma is generally located in the soft tissue. Here, we present a case of a 12-year-old girl with NF-1. The disease started in childhood with the appearance of multiple hyperpigmented skin macules. The girl presents generalized freckling and café au lait spots throughout the body and a diffused swelling measuring about 4 cm × 3 cm, extending from the right maxillary hard palate region to the midpalate. The diagnosis of NF-1 was made according to the presence of two or more diagnostic criteria of the National Institute of Health Consensus Development Conference. No recurrence was observed in a 15-month follow-up after extensive surgical ablation. |
format | Online Article Text |
id | pubmed-7069136 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Wolters Kluwer - Medknow |
record_format | MEDLINE/PubMed |
spelling | pubmed-70691362020-03-18 A rare case report of neurofibromatosis type 1 in a 12-year-old child: A 15-month follow-up Nallanchakrava, Srinivas Mallela, Manoj Kumar Jeenepalli, V Shiva Kumar Niharika, H M J Oral Maxillofac Pathol Case Report Neurofibromatosis type 1 (NF-1) or von Recklinghausen's disease is a rare genetic disorder characterized by the development of multiple noncancerous (benign) tumors of nerves and skin (neurofibromas). Head-and-neck neurofibroma is generally located in the soft tissue. Here, we present a case of a 12-year-old girl with NF-1. The disease started in childhood with the appearance of multiple hyperpigmented skin macules. The girl presents generalized freckling and café au lait spots throughout the body and a diffused swelling measuring about 4 cm × 3 cm, extending from the right maxillary hard palate region to the midpalate. The diagnosis of NF-1 was made according to the presence of two or more diagnostic criteria of the National Institute of Health Consensus Development Conference. No recurrence was observed in a 15-month follow-up after extensive surgical ablation. Wolters Kluwer - Medknow 2020-02 2020-02-28 /pmc/articles/PMC7069136/ /pubmed/32189916 http://dx.doi.org/10.4103/jomfp.JOMFP_35_20 Text en Copyright: © 2020 Journal of Oral and Maxillofacial Pathology http://creativecommons.org/licenses/by-nc-sa/4.0 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. |
spellingShingle | Case Report Nallanchakrava, Srinivas Mallela, Manoj Kumar Jeenepalli, V Shiva Kumar Niharika, H M A rare case report of neurofibromatosis type 1 in a 12-year-old child: A 15-month follow-up |
title | A rare case report of neurofibromatosis type 1 in a 12-year-old child: A 15-month follow-up |
title_full | A rare case report of neurofibromatosis type 1 in a 12-year-old child: A 15-month follow-up |
title_fullStr | A rare case report of neurofibromatosis type 1 in a 12-year-old child: A 15-month follow-up |
title_full_unstemmed | A rare case report of neurofibromatosis type 1 in a 12-year-old child: A 15-month follow-up |
title_short | A rare case report of neurofibromatosis type 1 in a 12-year-old child: A 15-month follow-up |
title_sort | rare case report of neurofibromatosis type 1 in a 12-year-old child: a 15-month follow-up |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7069136/ https://www.ncbi.nlm.nih.gov/pubmed/32189916 http://dx.doi.org/10.4103/jomfp.JOMFP_35_20 |
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