Vulva, angiomyxoma [LMP69657]
Keywords
Carney complex
Clinical History
This 15 year old girl had bilateral vulvar angiomyxomas resected at the age of 12. She subsequently developed a myxoma of the soft palate at the age of 13. At the age of 15, she developed a superficial right breast mass and left eyebrow mass, both were resected and were superficial myxomas. With five superficial myxomas at different anatomical locations, there was a very strong clinical suspicion for Carney Complex and the patient was referred to cardiology and genetics.
Clinical Discussion
The slides in this case demonstrate five nearly identical lesions from five different body sites: breast, left eyebrow, soft palate, and two vulvar lesions . This slide shows the right vulvar lesion, see Related Content section for slides showing the other lesions.
The lesions all demonstrate lobular masses with myxoid stroma and banal spindle cells. No mitotic figures, necrosis or atypia is noted. The vulvar lesions have prominent small, thin-walled blood vessels and were classified as angiomyxomas. The other three lesions were classified as superficial myxomas.
The patient is suspected to have Carney Complex, an autosomal dominant condition characterized by lentigines, cardiac myxoma, and endocrine overactivity. Major diagnostic criteria for Carney Complex include: spotty skin pigmentation, cutaneous/mucosal myxomas, breast myxomatosis, cardiac myxomas, endocrine dysfunction, large cell calcifying Sertoli cell tumours, psammomatous melanocytic schwannomas, blue nevi, breast ductal adenoma, and osteochondromyxoma.
The lesions all demonstrate lobular masses with myxoid stroma and banal spindle cells. No mitotic figures, necrosis or atypia is noted. The vulvar lesions have prominent small, thin-walled blood vessels and were classified as angiomyxomas. The other three lesions were classified as superficial myxomas.
The patient is suspected to have Carney Complex, an autosomal dominant condition characterized by lentigines, cardiac myxoma, and endocrine overactivity. Major diagnostic criteria for Carney Complex include: spotty skin pigmentation, cutaneous/mucosal myxomas, breast myxomatosis, cardiac myxomas, endocrine dysfunction, large cell calcifying Sertoli cell tumours, psammomatous melanocytic schwannomas, blue nevi, breast ductal adenoma, and osteochondromyxoma.
Related Content
Wholeslide Image ID:
