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

Christophe Abi Zeid Daou1, Yara Yammine1, Marc Mourad1

  • 1Department of Otolaryngology and Head and Neck Surgery, American University of Beirut Medical Center, Beirut, Lebanon.

Ear, Nose, & Throat Journal
|September 2, 2024
PubMed

Insights

Multiple endocrine neoplasia type 2B (MEN2B) is a rare genetic disorder. This case highlights rare laryngeal neuromas causing respiratory distress, emphasizing vigilant management of MEN2B complications.

Area of Science:

  • Endocrinology
  • Genetics
  • Oncology

Background:

  • Multiple endocrine neoplasia type 2B (MEN2B) is a rare genetic disorder caused by RET proto-oncogene mutations.
  • MEN2B presents with diverse symptoms, including mucosal neuromas, medullary thyroid carcinoma, and pheochromocytoma.
  • Mucosal neuromas contribute significantly to the clinical variability observed in MEN2B patients.

Purpose of the Study:

  • To present a case study of a young girl diagnosed with MEN2B.
  • To highlight the rare complication of laryngeal neuromas in MEN2B.
  • To emphasize the importance of recognizing rare manifestations for effective airway management during surgery.

Main Methods:

  • Case report presentation.
  • Review of clinical presentation and management.
  • Discussion of genetic basis and rare manifestations.

Main Results:

  • A young girl with MEN2B developed laryngeal neuromas.
  • These neuromas caused significant respiratory distress post-thyroidectomy.
  • The case underscores the rarity and clinical impact of laryngeal neuromas in MEN2B.

Conclusions:

  • Early detection and genetic counseling are crucial for managing MEN2B.
  • Multidisciplinary care is essential for improved patient outcomes.
  • Clinicians must be aware of rare manifestations like laryngeal neuromas to prevent surgical complications.
Keywords:
larynxpediatricssurgerythyroidectomy

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