Metastatic Soft Tissue Sarcomas in Adolescents and Young Adults: A Specialist Center Experience

Eugenie Younger,Bernice Asare,Olga Husson,Charlotte Benson,Winette T.A Van Der Graaf,Ian Judson,Shane Zaidi,Alison Dunlop,Omar Al-Muderis,Robin L Jones,Aisha Miah,Winan J Van Houdt

doi:10.1089/jayao.2020.0010

Abstract

Purpose: Soft tissue sarcomas (STS) account for 8% of all cancers in adolescents and young adults (AYAs). Metastatic STS contribute significantly to disease-related mortality in this age group; however, data are limited due to under-representation in clinical trials.Methods: AYAs aged 18–39 years, diagnosed with metastatic STS between 1990 and 2012, were identified from The Royal Marsden Hospital database. Outcomes of interest were clinical characteristics, treatment patterns, overall survival (OS), and prognostic factors.Results: Overall, 455 patients were included. Median age at diagnosis of metastatic STS was 33 years (interquartile range [IQR] 27–37 years). The most common histological subtypes were leiomyosarcoma (n = 68, 15%), synovial sarcoma (n = 68, 15%), Ewing sarcoma (n = 44, 10%), and rhabdomyosarcoma (n = 35, 8%). Treatments included systemic therapy (n = 395, 87%; median 2 lines [IQR 1–3]; clinical trial n = 93, 22%), radiotherapy (n = 297, 66%), and metastasectomy (n = 191, 43%). Median duration between last chemotherapy regimen and death was 4.6 months (IQR 2–10). Median OS was 19.2 months (95% confidence interval [CI] 15.8–22.2); 5-year OS was 16%. Of common subtypes, patients with rhabdomyosarcoma had the worst OS (8.8 months; 95% CI 7.9–11.4). Adverse prognostic factors included male gender, synchronous metastases, bone or liver metastases, first-line polychemotherapy, and no metastasectomy.Conclusions: Outcomes were variable; patients with supposed chemosensitive subtypes had particularly poor survival. The diverse behavior of STS in AYAs highlights the need for subtype-specific clinical trials.

Highlights

Soft tissue sarcomas (STS) are rare heterogeneous tumors that can arise in patients of any age.[1]
Four hundred fifty-five AYAs diagnosed with STS aged 18–39 years were included in the analysis (Table 1)
This study describes treatment patterns and clinical outcomes of a large group of AYAs with metastatic STS treated at a specialist sarcoma unit

Summary

Introduction

Soft tissue sarcomas (STS) are rare heterogeneous tumors that can arise in patients of any age.[1]. Rhabdomyosarcoma predominately occurs in children (typically embryonal subtype), can arise in AYAs (more commonly alveolar subtype).[5] Ewing sarcoma of the bone has a peak incidence in adolescence, whereas extraosseous Ewing sarcoma is more commonly seen in young adults.[6] Synovial sarcoma, epithelioid sarcoma, and alveolar soft part sarcoma typically occur in young adults.[5] STS classically affecting older adults, such as undifferentiated pleomorphic sarcoma (UPS), are seen in patients in the upper age range of the AYA group.[5,7]

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