In a review of 182 pediatric patients with adult-type sarcomas, only 14 had a diagnosis of liposarcoma. In another review, the characteristics of 82 cases of pediatric liposarcoma were reported. The median age was 15.5 years and females were more commonly affected. Myxoid liposarcoma was the predominant histologic subtype, and 94% of these patients were alive following surgical resection. In contrast, seven of ten patients with pleomorphic myxoid liposarcoma in this series died of their disease.
Malignant fibrous histiocytoma
At one time, MFH was the single most common histiotype among adults with soft tissue sarcomas. Since it was first recognized in the early 1960s, however, MFH has been plagued by controversy in terms of both its histogenesis and its validity as a clinicopathologic entity. The latest World Health Organization classification no longer includes MFH as a distinct diagnostic category but rather as a subtype of an undifferentiated pleomorphic sarcoma. (Refer to the Osteosarcoma and Malignant Fibrous Histiocytoma of Bone summary for information on MFH of bone.)
Malignant peripheral nerve sheath tumor
MPNST arises in children with type 1 neurofibromatosis (NF1), and it arises sporadically. Features with favorable prognosis have been reported to include localized disease, absence of NF1, smaller tumor size, lower stage, and an extremity as the primary site.[48,49,50] Chemotherapy has achieved objective responses in childhood MPNST. The role of adjuvant chemotherapy following resection of MPNST has not been prospectively evaluated. A retrospective survey of cancer centers in Japan identified 56 patients with MPNST, mostly adults, but including children and adolescents. This survey identified large tumor size, metastasis at presentation, and high histologic grade as unfavorable prognostic features. In this report, documentation of NF1 did not confer an inferior prognosis. A retrospective review of 140 patients with MPNST from the MD Anderson Cancer Center included children and adolescents. The disease-specific survival at 10 years was 32%. In this series, presence of metastatic disease was associated with a much worse prognosis. For patients with localized disease, there was no significant difference in outcome between patients with and without NF1. In a multivariate analysis, only tumor size and nuclear p53 expression were found to be independent predictors of disease-specific survival.
Mesenchymal chondrosarcoma is a highly malignant tumor with a propensity to spread to the lungs. A review of 15 patients aged younger than 26 years from the German Cooperative Soft Tissue Sarcoma (11 with soft-tissue lesions) and German-Austrian-Swiss Cooperative Osteosarcoma Study Group (four with primary bone lesions) protocols suggests that complete surgical removal, or incomplete resection followed by radiation therapy, is necessary for local control.[Level of evidence: 3iiA] Multiagent chemotherapy may decrease the likelihood of lung metastases: OS at 10 years was 67%, compared with approximately 20% in an earlier series of young patients.