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← Childhood Soft Tissue Sarcoma

HEALTH PROFESSIONAL · SOURCE READING

Clinical presentation

Source: Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.

Source updated: April 21, 2025 · Captured 2026-09-09.

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Context: Treatment of Tumors of Uncertain Differentiation / Synovial Sarcoma NOS (Poorly Differentiated, Spindle Cell, and Biphasic Varieties)

The most common primary tumor location is the extremities, followed by trunk and head and neck.[4] Rarely, a synovial sarcoma may arise in the heart or pericardium or appear with a pleuropulmonary presentation.[6-9]

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The most common site of metastasis is the lung.[10,11] The risk of metastases is highly influenced by tumor size. Patients with tumors that are larger than 5 cm have an estimated 32-fold higher risk of developing metastases compared with patients who have tumors smaller than 5 cm.

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The Cooperative Weichteilsarkom Studiengruppe (CWS) reported on 432 patients younger than 21 years diagnosed with synovial sarcoma between 1981 and 2018.[12] The study compared three age groups of patients: children (aged 0–12 years; n = 176), adolescents (aged 13–16 years; n = 178), and young adults (aged 17–21 years; n = 78).

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The proportion of invasive tumors was significantly higher in older patients (children, 33%; adolescents, 39%; and young adults, 54%; P = .009).

The proportion of tumors larger than 10 cm (children, 13%; adolescents, 21%; and young adults, 31%; P = .006) and the presence of metastasis at first diagnosis were also higher in older patients (children, 6%; adolescents, 10%; and young adults, 21%; P = .001).

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