Atypical lipomatous tumors/well-differentiated liposarcomas are low-grade malignant mesenchymal neoplasms with high propensity to local recurrence and potential to dedifferentiate to higher grades over time. However, the published risks of local recurrence and dedifferentiation vary, and no unified treatment and follow-up plan has been accepted. We performed a study to evaluate the long-term clinical behavior and proper treatment and follow-up strategy for these tumors. We retrospectively reviewed the files of 101 patients treated between 1990 and 2008 with the diagnosis of atypical lipomatous tumors/well-differentiated liposarcomas. For 67 of these patients, complete data and 2-year minimum follow-up were available and were included in the study; 47 patients (group A) had primary surgical treatment at our institution and 20 patients (group B) were referred after ≥1 local recurrences. Mean follow-up was 81 months (range, 24-229 months). The local recurrence rate of primary atypical lipomatous tumors/well-differentiated liposarcomas was 10.6% (5/47 group A patients). The local re-recurrence rate of the recurrent atypical lipomatous tumors/well-differentiated liposarcomas was 52% (13/67 group A and B patients). Recurrences developed as late as 140 months after diagnosis and treatment. The rate of dedifferentiation at recurrences was 4% (1/25 group A and B patients with recurrent tumors). No patients developed metastases. Atypical lipomatous tumors/well-differentiated liposarcomas are associated with an increased rate of local re-recurrence and low risk of dedifferentiation at recurrences. Long-term follow-up is recommended for early diagnosis and treatment of local recurrences.
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