Aim: We report a case of squamous carcinoma arising from a pilonidal sinus.

Case Report: Patient of 83 years old, that after 30 years had a recurrence of pilonidal sinus revealed by a sacral abscess studied with pelvic CT scan and MRI. After clinical investigation, traditional open surgical technique was performed and pathologic studies revealed a squamous carcinoma. The patient performed a new CT scan with persistence of disease, and a second surgical look with mass excision until the presacral fascia and V-Y flap was performed. Histological exami-nation was found to be positive for squamous carcinoma on the margin and the patient underwent adjuvant radiotherapy cycles with a close follow-up with evidence of free disease survival. He died after 5 years for old age.

Discussion: The incidence of carcinoma arising from a pilonidalis sinus is about 0.1% and the most important risk factor is represented by a chronic abscess from 20-30 years. In literature there are about 100 cases. Gold standard treatment is surgery with complete excision of the presacral fascia, while radiotherapy decrease the risk of recurrence.

Conclusion: All cases reported in literature are submitted as case report. Sacro-coccyxal fistula should be treated early because chronic inflammation can determine neoplastic degeneration. Histological examination should be performed routinely. Gold standard is surgery with wide excision. There is no evidence about the gold standard for the reconstructive time.

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