Primary adenocarcinomas of the vulva are rare and constitute sweat gland carcinomas, extramammary Paget disease and primary breast carcinoma of the vulva.1^–3 Here we present a case of primary apocrine carcinoma of the vulva with distant metastasis.

A 71-year-old woman presented with a hard lesion of 1.5 cm on the left labia majora. No breast lumps were noted on palpation and sono-mammographic examination. Wide local excision revealed an apocrine adenocarcinoma of the vulva of primary adnexal origin with extensive perineural and angiolymphatic invasion and involved lateral margins, but no pagetoid component (fig 1A, B). The tumour cells were positive for androgen receptor and GCDFP15 (fig 1C), but were negative for oestrogen receptor (ER) and progesterone …