Current Treatment Patterns and Outcomes of Sex Cord Stromal Tumor Patients in Japan

TL;DR

This study examines treatment and survival outcomes for patients with sex cord stromal tumors in Japan, highlighting the impact of risk factors and treatment approaches.

Contribution

The study provides new insights into treatment patterns and survival outcomes for sex cord stromal tumors in Japan, emphasizing the role of surgical management in recurrent cases.

Findings

Stage I patients with more than two pathological risk factors had significantly worse recurrence-free survival.

Surgical intervention was the primary treatment for recurrence, with some patients achieving prolonged disease control through repeated surgery.

Patients with metastatic disease at diagnosis had poor survival rates, with only 20.8% five-year overall survival.

Abstract

Sex cord‐stromal tumors (SCSTs) constitute approximately 5% of testicular malignancies. This investigation aimed to elucidate contemporary therapeutic approaches and clinical outcomes for patients with testicular SCSTs in Japan.

Participating institutions included Japan Clinical Oncology Group facilities, affiliated centers, and institutions with published cases. Clinical data and histopathological findings were compiled. Central pathological review involved representative tissue sections when available. Kaplan–Meier methodology assessed recurrence‐free survival (RFS) and overall survival (OS). Stage I patients were stratified by cumulative pathological risk factors (tumor diameter ≥ 5 cm, necrosis, moderate to severe nuclear atypia, lymphovascular invasion, infiltrative growth, and ≥ 3 mitoses per 10 high‐power fields).

Among 116 patients from 66 institutions, 112 met inclusion criteria. Histological distribution included Leydig cell (n = 54), Sertoli cell (n = 36), and other variants (n = 22). Twelve patients presented with metastatic disease; ten received systemic chemotherapy, with 20.8% five‐year OS. Among 91 stage I patients with follow‐up data, eleven developed recurrence. Patients with > 2 risk factors demonstrated significantly inferior RFS compared with those showing ≤ 1 factor (26.4 versus 98.6% at 5 years, p < 0.0001). Surgical intervention predominated in the recurrent cases (81.8%, 9/11), while chemotherapy was administered to 45.5% (5/11), and selected patients achieved prolonged disease control through repeated surgical resection without systemic therapy. OS rates were 90.9% and 50.5% at 5 and 10 years.

Patients with synchronous metastatic presentation demonstrated poor survival. Stage I patients with > 2 pathological risk factors showed reduced RFS, with surgical management potentially efficacious for recurrent disease.

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