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

**Authors:** Takashige Abe, Kosuke Miyai, Toyonori Tsuzuki, Sachiyo Murai, Katsuyoshi Hashine, Shinichi Yamashita, Takashi Kawahara, Naotaka Nishiyama, Hiroshi Kitamura, Hiroshi Kikuchi, Kazutaka Saito, Hatsuki Hibi, Haruka Miyata, Ryuji Matsumoto, Takahiro Osawa, Hiroyuki Nishiyama, Nobuo Shinohara

PMC · DOI: 10.1111/iju.70399 · 2026-03-10

## 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.

## Key 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.

## Full-text entities

- **Genes:** CALB2 (calbindin 2) [NCBI Gene 794] {aka CAB29, CAL2, CR}, SALL4 (spalt like transcription factor 4) [NCBI Gene 57167] {aka DRRS, HSAL4, IVIC, ZNF797}, TNFRSF8 (TNF receptor superfamily member 8) [NCBI Gene 943] {aka CD30, D1S166E, Ki-1}, SYP (synaptophysin) [NCBI Gene 6855] {aka MRX96, MRXSYP, XLID96}, CTNNB1 (catenin beta 1) [NCBI Gene 1499] {aka CTNNB, EVR7, MRD19, NEDSDV, armadillo}, MLANA (melan-A) [NCBI Gene 2315] {aka MART-1, MART1}
- **Diseases:** Leydig cell tumor (MESH:D007984), testicular (MESH:D013733), Disease (MESH:D004194), inflammatory (MESH:D007249), testicular germ cell tumors (MESH:C563236), Sertoli cell tumors (MESH:D012707), seminoma (MESH:D018239), cancer (MESH:D009369), I (MESH:D006969), testicular neoplasms (MESH:D013736), testicular induration (MESH:D010411), germ cell tumor (MESH:D009373), SCSTs (MESH:D018312), death (MESH:D003643), bone metastasis (MESH:D009362), SCT (MESH:C535780), IIA disease (MESH:D056728), stage 1 disease (MESH:D007676), infertility (MESH:D007246), Sertoli-Leydig cell tumors (MESH:D018310), lymph node metastasis (MESH:D008207), necrosis (MESH:D009336)
- **Chemicals:** vincristine (MESH:D014750), bleomycin (MESH:D001761), CBDCA (MESH:D016190), Nedaplatin (MESH:C053989), actinomycin D. (MESH:D003609), CDDP (MESH:D002945), Hibi (-), etoposide (MESH:D005047), taxane (MESH:C080625)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Figures

3 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12976466/full.md

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Source: https://tomesphere.com/paper/PMC12976466