A Rare Case of Vulvar Superficial Angiomyxoma
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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 Carney Complex (CC), a rare 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. Due to its benign and cyst-like appearance, vulvar SAM is often confused with skin tags, labial cysts, or cysts of the Bartholin or Gartner’s ducts. Grossly, SAM range from polypoid to papulonodular. Distinctive histologic features of SAM include a poorly circumscribed tumor with a multilobular pattern of growth. The component cells include scattered, bland, spindle shaped cells suspended within a myxoid stroma. When present, the presence of neutrophils in the cellular milieu is an important diagnostic feature that distinguishes SAM from other myxoid tumors. The blood vessels within the tumor are usually small and thin-walled. Nuclear atypia and hyperchromasia are rare. Recurrence rates for SAM are reportedly 30 to 40%. Recurrences are thought most likely to be due to incomplete resection. Fortunately, recurrences tend to be local, non-destructive, and without distant metastases.

A 7-year-old female with no prior medical history was referred by her primary care provider for evaluation and management by pediatric gynecology for a mass arising from the right labia majora. The lesion had been present since birth but had grown slowly over the years. The decision was made for surgical excision of the mass. Exam under anesthesia revealed a 2.5 cm x 1.5 cm x 1.2 cm, pink-tan, pedunculated, cauliflower-like mass with a 0.5 cm stalk. It was excised at the base of the pedunculated stalk using Bovie cautery. On gross pathologic examination, the skin-covered lesion had underlying yellow-tan to tan-pink, glistening, gelatinous cut surfaces. Microscopically, it was a vaguely multinodular dermal mass with lobules of bland spindle cells in abundant myxoid matrix. Occasional entrapped epithelial basaloid nests and thin-walled blood vessels were noted. Prominent neutrophils were also a notable finding. Immunohistochemistry staining of the cells was positive for CD34 and negative for desmin, progesterone receptor (PR), and estrogen receptor (ER) (Figure 2A-B). The pathologic diagnosis was superficial angiomyxoma.

Source: https://www.jpagonline.org/article/S1083-3188(20)30291-6/fulltext?rss=yes