Extracranial, Extragonadal Germ Cell Tumors: A Multicenter Australian Case Series

To describe the treatment pattern and outcomes for patients with extracranial, extragonadal germ cell tumors (EGCTs).

We conducted a retrospective analysis of male patients with EGCT prospectively registered in the Australian GCT clinical registry (iTestis) since its inception in 2018. Demographics, clinicopathologic features, treatment characteristics, and outcomes were recorded. Data were analyzed using descriptive statistics and the Kaplan-Meier method estimates of overall survival (OS).

Of 1256 patients in the iTestis registry, 33 (3%) had EGCT including 18 (55%) with mediastinal, 14 (42%) with retroperitoneal, and 1 (3%) with a lung primary. The median age was 31 years (range: 24-38), ECOG performance status was 0-1 in 26 (79%), and 21 (64%) were non-seminoma. Chemotherapy was administered upfront in 27 (82%) patients, upfront surgery in four patients (12%), and treatment was unknown in two (6%) patients. The most common chemotherapy regimen was bleomycin/etoposide/cisplatin (BEP) (n = 20, 74%), followed by etoposide/ifosfamide/cisplatin (VIP) (n = 5, 19%) and EP (n = 2, 7%). Post-chemotherapy surgery was carried out in 16 (59%) patients, and 4 (15%) patients received second-line chemotherapy. At a median follow-up of 22.7 months (range: 0-126), no deaths were observed in individuals with retroperitoneal primary (n = 14), seminoma (n = 12), or International Germ Cell Cancer Collaborative Group (IGCCCG) good (n = 15) and intermediate risk (n = 3) disease. Three deaths occurred in patients with mediastinal non-seminoma. The estimated 24-month OS for mediastinal non-seminoma and IGCCCG poor risk disease was 71% (95% confidence interval [CI]: 27%-94%) and 79% (95% CI: 25%-95%), respectively.

Incidence of EGCT in Australia is consistent with published literature. Treatment patterns reflect international practices. Primary mediastinal non-seminoma has the poorest outcomes. Primary retroperitoneal and primary mediastinal seminomas have excellent outcomes.