Superficial angiomyxoma (SAM) is a rare, benign cutaneous tumor with a predilection for the head, neck, and trunk. SAM is most common in males with a peak incidence during middle age. It was originally described as a component of Carneys syndrome, a condition characterized by the triad of pigmented skin and mucosal lesions, cutaneous and cardiac myxomas, and multiple endocrine tumors. Now, sporadic SAM is also recognized. Grossly, SAM range from polypoid to papulonodular. Histologic features include a multilobular, poorly circumscribed tumor with scattered, bland spindle shaped cells suspended within a myxoid stroma and small, thin-walled blood vessels. A characteristic finding is the presence of neutrophils. Recurrence rates for SAM are reportedly 30 40%, likely due to incomplete resection. Fortunately, recurrences tend to be local, non-destructive, and without distant metastases. Our case describes vulvar SAM in a pediatric patient. Vulvar SAM is rare, with less than 20 total cases reported worldwide.
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