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&...

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Veröffentlicht in:Ear, nose, & throat journal nose, & throat journal, 2024-09, p.1455613241274184
Hauptverfasser: Abi Zeid Daou, Christophe, Yammine, Yara, Mourad, Marc, Al Barazi, Randa
Format: Artikel
Sprache:eng
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Zusammenfassung: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.
ISSN:0145-5613
1942-7522
1942-7522
DOI:10.1177/01455613241274184