Laryngeal Multiple Mucosal Neuromas: A Diagnostic Challenge

Area of Science:

• Neuropathology
• Head and Neck Surgery
• Oncology

Background:

• Palisaded encapsulated neuromas (PENs), also termed solitary circumscribed neuromas, are benign peripheral nerve sheath tumors.
• Typically presenting as solitary lesions on mucocutaneous surfaces, PENs are generally considered to have no association with systemic syndromes.
• Multiple endocrine neoplasia 2B (MEN2B) is a rare genetic disorder associated with mucosal neuromas, medullary thyroid carcinoma, and pheochromocytoma.

Observation:

• A rare case of multiple, widespread mucosal neuromas involving the larynx is presented.
• The patient did not exhibit the characteristic clinical or genetic findings of multiple endocrine neoplasia 2B.
• The lesions were identified during diagnostic workup for unrelated laryngeal symptoms.

Findings:

• Histopathologic examination confirmed the diagnosis of palisaded encapsulated neuromas.
• The distribution and multiplicity of the neuromas in the larynx were atypical for sporadic lesions.
• Absence of MEN2B markers suggests an alternative etiology or a novel presentation.

Implications:

• This case broadens the differential diagnosis for multiple laryngeal lesions.
• It underscores the importance of thorough histopathologic evaluation for accurate diagnosis of laryngeal neuromas.
• Further research may elucidate the specific factors contributing to non-MEN2B-associated multiple mucosal neuromas.