Plexiform Neurofibroma of the Larynx in Children: About an Unusual Case Report

<p><strong>Introduction: </strong>The plexiform neurofibroma is a rare benign tumor which is often associated to type 1 neurofibromatosis or Von Recklinghausen's disease. The laryngeal involvement in neurofibromatosis is extremely rare. </p><p>The objective of our...

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Main Authors: R Mahdoufi (Author), I Barhmi (Author), N Tazi (Author), R Sami (Author), R Abada (Author), M Roubal (Author), M Mahtar (Author)
Format: Book
Published: Archives of Otolaryngology and Rhinology - Peertechz Publications, 2016-07-04.
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Summary:<p><strong>Introduction: </strong>The plexiform neurofibroma is a rare benign tumor which is often associated to type 1 neurofibromatosis or Von Recklinghausen's disease. The laryngeal involvement in neurofibromatosis is extremely rare. </p><p>The objective of our report is the consideration of laryngeal neurofibromatosis in the differential diagnosis of dyspnea in infants and children. </p><p><strong>Summary of the clinical case:</strong> We report the case of a 4 year-old child who presented with inspiratory dyspnea. Laryngoscopy revealed a mass obstructing completely the laryngeal vestibule. Pathological examination of the resected tissue revealed a plexiform neurofibroma after immunohistochemical examination. The patient had regular follow up and she remained symptom free.  </p><p><strong>Discussion: </strong>There is an association between neurofibromatosis type 1 and plexiform neurofibromas of the larynx. Analysis of the margins of neurofibromas of the larynx associated with neurofibromatosis type 1 may  elp predict clinical behavior.</p>
DOI:10.17352/2455-1759.000020