Background: Malignant degeneration is a rare complication of pilonidal disease and is associated with a high recurrence rate and poor prognosis compared with regular nonmelanoma skin cancer. Treatment in our departments and in the international literature was evaluated.
Methods: We analyzed the data from three patients with malignant degeneration who were treated in our departments and an additional 56 patients who were found after an extensive literature search.
Results: A total of 47 males and 12 females, with a mean age of 52 years, were most frequently primarily treated with surgery. After a mean follow-up time of 28 months, 20% of all patients died with evidence of disease and an additional 10% died of unrelated causes. The overall recurrence rate was 39%, with a median time to recurrence of only 9 months. The local recurrence rate was lower when radiotherapy was added to surgical treatment alone (30% vs. 44%). Re-excision of local recurrence resulted in some long-term survivals.
Conclusions: Early diagnosis and treatment may lead to improvement of the relative poor prognosis. Surgical treatment should be tailored according to the locoregional extent. The high recurrence rate after surgical treatment can be reduced by the addition of radiotherapy. Although repeat surgery for recurrent disease may involve extensive resection and morbidity, this may result in prolonged survival.