Abstract

Superficialangiomyxoma (SMA) is a rare benign skin tumorwith a peak incidence in middle age . The diseaseiscaused by mutations in the PRKAR1A gene. Which, codes for the type of I alpha regulatorysubunit of the enzyme protein kinase A. We report a rare case of vulvarangiomyxomain 19-year-old patient beneficied of large exicion of mass in the leftlabia majora. the diagnostic of SMA retained, afterhistologicalstudy of surgicalspecimen , The evolution has been good withoutrecurrence.

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