Rectal and gynecologic amputation for a giant eccrine porocarcinoma of the pelvic floor

Porocarcinoma is a rare skin malignancy that derives from eccrine sweat glands. In most cases described in elderly patients, presenting as a long-standing growth that is predominantly located in the lower extremities, but also described on head, neck, trunk, abdomen and scrotum. 1,2 Porocarcinoma gives the 17-20% local recurrence, and metastatizes the 20% to regional lymph nodes but also to abdominal viscera, lungs, brain and bone.1,2 In these cases prognosis is very poor and mortality rate varies from 75% to 80% according to large case series.3,4 It may appear de novo or arise in a pre-existing eccrine poroma. Clinical appearance is usually non specific and can vary from a nodule to an indurated plaque, from a polypoid growth to an ulcerated and bleeding lesion. 1-4 Herein we describe the first case reported in english literature of a porocarcinoma arising in the female perineum involving vulva and anus.