Laryngeal Neuromas in a 5-Year-Old With MEN2B: A Case Report and Literature Review

Multiple endocrine neoplasia type 2B (MEN2B) syndrome is a rare genetic disorder primarily driven by mutations in the RET proto-oncogene. MEN2B exhibits diverse symptoms affecting multiple organ systems, with mucosal neuromas being a prominent feature. These benign tumors contribute to the syndrome's clinical variability, alongside medullary thyroid carcinoma and pheochromocytoma. The article presents a case study of a young girl diagnosed with MEN2B, complicated by laryngeal neuromas leading to respiratory distress post-thyroidectomy. Laryngeal neuromas are exceedingly rare, underscoring the importance of recognizing such manifestations for proper airway management, especially during surgical interventions. Despite its rarity, MEN2B presents significant clinical challenges, requiring early detection, genetic counseling, and multidisciplinary management for improved outcomes. Clinicians must remain vigilant for rare manifestations like laryngeal neuromas to prevent serious complications, particularly during surgical procedures.

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Publication Details

Journal
Ear Nose & Throat Journal
Published
2024-09-01
DOI
https://doi.org/10.1177/01455613241274184
Primary Topic
Neuroendocrine Tumor Research Advances
Type
article
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article

Laryngeal Neuromas in a 5-Year-Old With MEN2B: A Case Report and Literature Review

Marc Mourad, Randa Barazi, Christophe Abi Zeid Daou, Yara Yammine
Ear Nose & Throat Journal
Neuroendocrine Tumor Research Advances
article

Laryngeal Neuromas in a 5-Year-Old With MEN2B: A Case Report and Literature Review

Marc Mourad, Randa Barazi, Christophe Abi Zeid Daou, Yara Yammine
article en

Abstract

Multiple endocrine neoplasia type 2B (MEN2B) syndrome is a rare genetic disorder primarily driven by mutations in the RET proto-oncogene. MEN2B exhibits diverse symptoms affecting multiple organ systems, with mucosal neuromas being a prominent feature. These benign tumors contribute to the syndrome's clinical variability, alongside medullary thyroid carcinoma and pheochromocytoma. The article presents a case study of a young girl diagnosed with MEN2B, complicated by laryngeal neuromas leading to respiratory distress post-thyroidectomy. Laryngeal neuromas are exceedingly rare, underscoring the importance of recognizing such manifestations for proper airway management, especially during surgical interventions. Despite its rarity, MEN2B presents significant clinical challenges, requiring early detection, genetic counseling, and multidisciplinary management for improved outcomes. Clinicians must remain vigilant for rare manifestations like laryngeal neuromas to prevent serious complications, particularly during surgical procedures.

Ear Nose & Throat Journal
American University of Beirut Medical Center (LB)
Good health and well-being
Openalex Percentile: Top 78%
Neuroendocrine Tumor Research Advances
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Laryngeal Neuromas in a 5-Year-Old With MEN2B: A Case Report and Literature Review — Marc Mourad, Randa Barazi, et al. · Ear Nose & Throat Journal (2024) | TGRS Research Map | TGRS