We report a male patient with carcinoma arising on the basis of neglected s
acrococcygeal pilonidal sinus disease. Following initial operation, perform
ed without suspicion of malignancy, histology demonstrated cellular atypia
and an increased mitotic rate. A second, wider tissue excision was recommen
ded but the patient declined further surgery. Two years later, he presented
with fungating carcinoma involving the rectum but again declined surgery.
This rare case demonstrates that the presence of carcinoma should be suspec
ted in long-standing, although innocent-looking, pilonidal sinus disease. I
n the circumstance of uncertain histologic diagnosis, more generous surgica
l sampling is required. Every effort must be made to overcome patient's rel
uctance to accept a second, possibly life-saving procedure.