Soft tissue sarcoma across the age spectrum: a population-based study from the Surveillance Epidemiology and End Results database

Abstract

Background: Soft tissue sarcomas (STS) are a heterogeneous group of mesenchymal malignancies that occur throughout the lifespan. The impact of age on disease features and outcome is unclear.

Methods: We analyzed the clinical features and outcome of all STS cases registered between 1973 and 2006 in the SEER database.

Results: There were 48,012 cases that met the selection criteria. Individuals less than 20 years of age represented 5.6%, with rhabdomyosarcoma being the most common subtype. In adults, the most common types were Kaposi sarcoma, fibrohistiocytic tumors, and leiomyosarcoma. Rhabdomyosarcoma was the only entity with a median age <20 years. Male predominance (male/female of 1.5:1) was noticed for almost all types of STS, except for alveolar soft part sarcoma and leiomyosarcoma. Tumor stage was similar across different age groups. Younger patients (<50 years) had significantly better survival than older patients (88.8 ± 0.2% vs. 40 ± 0.3%, P < 0.001), but for most histologies the survival decline with advancing age was gradual and did not occur abruptly at the onset of adulthood. The decline in survival with advancing age was particularly significant for rhabdomyosarcoma.

Conclusion: With few exceptions, the clinical features of STS are similar in children and adults. However, individuals over 50 years of age have an inferior survival.

Figure 1

(A) Distribution of histologic subtypes by 10-year age groups, (B)distribution of primary tumor sites by 10-year age groups, (C) distribution of tumor stage by 10-year age groups; abbreviations: RMS: rhabdomyosarcomas; Fibroblastic: fibroblastic and myofibroblastic tumors; Fibrohistiocytic: Fibrohistiocytic tumors; NST: malignant peripheral nerve sheath tumors; Kaposi: Kaposi sarcoma; pPNET: Ewing family tumors; Rhabdoid: extraneral rhabdoid tumor; Liposarcoma: liposarcomas; Synovial: Synovial sarcomas; Blood vessel: blood vessel tumors; ASPS: alveolar soft parts sarcoma; Miscellaneous: Miscellaneous/unspecified soft tissue sarcomas including other fibromatous neoplasms.