A case of pediatric Osteochondromyxoma in the nasal sinuses

Osteochondromyxoma is a rare neoplasm associated with Carney Complex, an inherited neoplastic syndrome associated with PRKAR1A mutations. There are few case reports of this tumor developing in the nasal sinuses. An 11-year-old male presented to the ED for epistaxis secondary to minor trauma. The patient denied any history of pain, prior epistaxis, anosmia, or double vision, but endorsed a several year history of nasal obstruction. CT imaging was notable for a nasal mass. He was scheduled for surgical resection of the mass and underwent pre-operative embolization due to concern for intra-operative blood loss. He underwent endoscopic resection and did well post-operatively. Pathology of the tumor showed hypercellular cartilaginous matrix with myxoid stroma. Genetic testing showed loss of expression of the PRKAR1A in the tumor, suggesting the diagnosis of Osteochondromyxoma. He was subsequently referred for genetic evaluation for Carney Complex. By presenting this case report, we add to the limited body of literature on this rare disease entity. Osteochondromyxoma should be kept on the differential for a cartilaginous nasal mass. Treatment is endoscopic resection, and surveillance is necessary to reduce morbidity. It is important to recognize the association between Osteochondromyxoma and Carney Complex.