Childhood liposarcoma. Report of a case and review of the literature.

A case of liposarcoma presenting in an adolescent black male is described. Features unique to this case include a mediastinal primary site (the fourth to be documented in a pediatric patient), a demonstrable response to radiotherapy and chemotherapy allowing complete surgical excision of an initially inoperable tumor, and the apparent histologic maturation of the tumor following multimodal therapy. A review of previously published accounts of pediatric liposarcoma revealed the following: (1) peak incidences during infancy and in early adolescence; (2) the extremities to be the most common site of origin (51%); (3) a predominance of myxoid histology (76%); and (4) a lower overall recurrence rate when compared with adult cases (37% versus 72%, respectively). The influence of histology, location of the tumor, and completeness of surgical excision on the prognosis of adult liposarcoma was confirmed in this limited pediatric experience. While the use of radiotherapy and chemotherapy cannot be advocated in all instances of liposarcoma, our experience and that from other reports would support the incorporation of these modalities of treatment in selected patients where the tumor is surgically inaccessible due to size.