Prognostic Factors and Survival in Pediatric and Adolescent Liposarcoma

الملخص EN

Purpose.

Liposarcoma is extremely rare in the pediatric population.

To identify prognostic factors and determine treatment outcomes, we reviewed our institutional experience with pediatric liposarcoma.

Methods.

We retrospectively reviewed all pediatric patients (age <22 years) with confirmed liposarcoma treated at Memorial Sloan-Kettering Cancer Center.

Histologic subtype, tumor location, margin status, recurrence, and adjuvant therapy were analyzed and correlated with overall survival.

Results.

Thirty-four patients (56% male) with a median age of 18.1 years were identified.

Twenty-two (65%) had peripheral tumors and 12 (35%) had centrally located tumors.

Histologically, 29 (85%) tumors were low grade, and 5 (15%) were high grade pleomorphic.

Eleven (32%) had recurrent disease, 9 patients with central tumors and 2 patients with peripheral lesions.

Eight deaths occurred, all in patients with central disease.

Five-year overall survival was 78%, with a median follow-up time of 5.4 years (range, 0.3–30.3 years).

Tumor grade (P=.003), histologic subtype (P=.01), and primary location (P<.001) all correlated with survival, as did stage (P<.001) and margin status (P=.001).

Conclusions.

Central location of the primary tumor, high tumor grade, and positive surgical margins are strongly correlated with poor survival in pediatric patients with liposarcoma.