ABSTRACT
BACKGROUND: Superficial angiomyxoma (SAM) is a rare, benign cutaneous tumor. Originally described as a component of Carney complex, it is now recognized as a sporadic condition. CASE: A 7-year-old girl was referred for management of a 2.5-cm mass arising from the right labia majora. Key pathologic features included lobules of spindle-shaped cells in a myxoid matrix and prominent neutrophilic infiltrate. The cells were positive for CD34 and negative for desmin, progesterone receptor, and estrogen receptor staining. SUMMARY AND CONCLUSION: This case is that of the youngest described patient with vulvar SAM. Patients should be carefully examined for manifestations of Carney complex to avoid potentially life-threatening complications. It is critical to distinguish SAM from aggressive angiomyxoma. The patient was referred to genetics and will be followed for local recurrence.
