Radical resection and sentinel lymph node evaluation of mammary-like adenocarcinoma of the vulva (MLAV) with somatic BRCA1 mutation

Mammary-like gland adenocarcinoma of the vulva is a rare but aggressive neoplasm that presents diagnostic challenges due to its resemblance to breast carcinoma. Radical resection and sentinel lymph node assessment is feasible. Melanocytic skin types pose a unique set of differences regarding detection, diagnosis and potential genomic variations such as BRCA1 mutation in our patient. Genetic and genomic assessments should be considered to further guide optimal therapeutic options.