Solitary mastocytoma of the orbit: A case report and review of the literature

Abstract

Purpose: The authors present the first known case report of an histologically confirmed orbital mastocytoma. This report is unique for two reasons: its orbital location and its presentation in an adult.

Methods: A 24-year-old white woman presented with a ten-year history of a mass in the right, superior orbit. She had been previously followed with a diagnosis of urticaria pigmentosa (UP) since two years of age. Her skin lesions were typical for UP in that rubbing them produced urtication. She had no other signs of systemic involvement. Radiologic examination confirmed the presence of an homogeneous oval mass in the right orbit with no signs of bone erosion.

Results: The patient underwent an anterior orbitotomy for biopsy and excision of the mass. H & E and Giemsa stains, immunohistochemical studies and ultrastructural confirmation by electron microscopy revealed features consistent with a diagnosis of mastocytoma. No complications occurred and the patient required no further therapy.

Conclusion: Mastocytoma of the orbit has not been reported prior to this case. The unique aspects of this case include its site of presentation, possible familial inheritance pattern of UP, and the persistence of this lesion in an adult.

Key Words:

• Proptosis
• orbit
• orbital tumor
• mastocytosis
• mastocytoma