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A retroperitoneal germ cell tumour is a germ cell tumour found in the back of the abdomen with no obvious tumour in the testis. Many turn out to be spread from a tiny testicular tumour that has scarred over, so the testes are examined closely; treatment and cure rates are close to those of testicular disease.
Retroperitoneal germ cell tumours present as a mass around the aorta and vena cava, the first drainage site of the testis, in a man whose testes feel normal. Whether they are true extragonadal primaries is disputed: in a series of 26 patients treated as primary retroperitoneal germ cell tumours, ultrasound of the testes was abnormal in all 20 examined and testicular histology in 25 showed viable tumour in three, intratubular germ cell neoplasia in four, scar tissue in 12 and sclerosis or fibrosis in six; the authors concluded that such tumours should be regarded as metastases of a viable or burned-out testicular cancer until proven otherwise (Scholz 2002). The NCI PDQ summary keeps the retroperitoneum as one of the two main extragonadal sites.
How it differs from its parent: outcome is better than for the mediastinal site. In the pooled analysis, 63 percent of patients with retroperitoneal non-seminoma were alive after platinum chemotherapy against 49 percent with mediastinal non-seminoma, and no haematological malignancies occurred with retroperitoneal primaries (Bokemeyer 2002; Hartmann 2000). Seminoma survival was 88 percent whatever the site, and seminoma treated with radiotherapy alone had a lower progression-free survival than with chemotherapy (Bokemeyer 2002). In children the retroperitoneum is a site of teratoma: 8 of 11 children in one hospital series had teratomas, average age at diagnosis 18 months (Cancer 1985, PMID 2988749).
How common: no separate incidence figure is published.
Treatment follows the parent page: retroperitoneal non-seminoma is staged by the International Germ Cell Cancer Collaborative Group risk group as testicular disease is, with three or four cycles of BEP and resection of residual masses over one centimetre; seminoma has cisplatin-based chemotherapy. A testicular ultrasound and, where it is abnormal, orchidectomy are part of the work-up because of the burned-out primary (Scholz 2002).
Rare and not counted separately by GLOBOCAN. In the international analysis of 635 extragonadal germ cell tumours, 283 (45 percent) arose in the retroperitoneum (Bokemeyer 2002).
Germ cell tumours drain along the spermatic cord to the para-aortic nodes high in the abdomen, not to the groin, which is why staging scans look at the retroperitoneum.
Same organ: Leydig cell tumour of the testis, Sertoli cell tumour of the testis, Spermatocytic tumour of the testis, Germ cell neoplasia in situ (GCNIS), Embryonal carcinoma of the testis, Yolk sac tumour of the testis, postpubertal type, Choriocarcinoma of the testis, Testicular germ cell tumours, Seminoma, Non-seminomatous germ cell tumour, Germ cell tumours of childhood and adolescence (extracranial and CNS)
Treated as the parent: chemotherapy by risk group, resection of residual masses, and testicular ultrasound with orchidectomy where the testis is abnormal.
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