Nasal chondromesenchyal hamartoma

Nasal chondromesenchyal hamartoma (NCMH) generally presents as a nasal or sinus mass. Symptoms vary by size and location but may include nasal drainage, congestion, nasal obstruction or swelling. Infants may present with respiratory or feeding difficulties.

Nasal endoscopy, computed tomography (CT) or magnetic resonance imaging (MRI) can define tumor location and involvement of surrounding tissues. CT is better for visualizing bony changes. Bony destruction can be seen.

Surgical removal is generally curative although local recurrences have been reported. If complete resection proves difficult, these tumors can be effectively debulked in most cases; however, complete removal is preferred.

Pathology shows a polypoid mass with nodules of immature to mature cartilage are surrounded in part by a spindle cell stroma without rhabdomyoblastic differentiation. Other patterns include aneurysmal bone cyst-like foci, immature mesenchyme and cysts lined by respiratory epithelium.

No imaging studies are indicated in asymptomatic individuals, but individuals, families and care providers should be aware of the risk, albeit low, for NCMH and the need for evaluation with endoscopy and potential imaging evaluation if clinically indicated.