Extragonadal germ cell tumors in Japan

Abstract

Extragonadal germ cell tumors (EGCT) represent only 2-5% of adult germ cell malignancies. Some publications from Asia have reported inferior treatment outcomes compared to data from an international study group. To ascertain whether this is generally the case, here we analyze treatment outcomes for 30 Japanese patients with EGCT. The medical records of 30 patients (25 nonseminomas and 4 pure seminomas) treated from 1992 to 2002 were reviewed retrospectively. All patients with seminoma achieved long survival except one who died of chemotherapy-related sepsis. Ten and 11 patients with EGCT presented with mediastinal and retroperitoneal primary sites, respectively. The 5-year overall survival (OS) and progression-free survival (PFS) for nonseminoma was 71% and 42%, respectively. The 5-year OS and PFS was 60% and 44%, respectively, for 10 patients with mediastinal nonseminoma, and 91% and 48%, respectively, for patients with retroperitoneal nonseminoma. Tumor marker values on day 7 were available for 19 patients. Among the 19 patients in whom AFP or β β β β-HCG were measured on day 7, the values had declined in 12 patients and were transiently elevated in 7 patients compared to pretreatment values. The transient elevations of tumor markers were significantly associated with poor OS (P = = = =0.02) and PFS (P = = = =0.008). The treatment outcome of Japanese patients with EGCT seemed to be comparable to that reported from international studies, suggesting no difference between ethnic groups. Transient tumor marker elevations on day 7 predict poor survival in EGCT patients and may be a useful parameter for identifying patients requiring more aggressive treatment. (Cancer Sci 2003; 94: 1107-1111) erm cell tumors (GCT) predominantly arise in the testes, but a small subset of 2 to 5% is of extragonadal origin. 1)Extragonadal germ cell tumors (EGCT) usually present in midline structures, such as the anterior mediastinum, the retroperitoneum, and the pineal gland, but occasionally arise in the lung or upper gastrointestinal tract. 2, 3)Extragonadal germ cell tumors differ from GCTs of testicular origin in several ways. First, the distribution of histological subtypes presented in adult EGCTs is different from that of testicular EGCTs. Second, patients with EGCTs usually present with far more advanced disease than those with primary testicular tumors. Furthermore, mediastinal nonseminomatous tumors are associated with Klinefelter's syndrome and hematological malignancy. 4,5) Cisplatin-containing chemotherapy dramatically improved the outlook for patients with GCT in the 1970s. The International Germ Cell Cancer Collaborative Group (IGCCCG) has proposed a staging classification based on prognostic factors for patients with metastatic GCT who were treated with cisplatin-containing chemotherapy.6) Because of the rarity of this disease, only limited data had been available regarding the clinical course of EGCTs, [7][8][9][10] prior to a recent report from an international study group on the characteristics and prognostic variabl...

Influence of Suboptimal Treatment in Patients with Mediastinal Primary Nonseminomatous Germ Cell Tumors

“…The other reasons for suboptimal treatment were poor patient compliance, the concomitant presence of other medical conditions and delayed diagnosis/therapy. Recent studies have also reported suboptimal treatment of a significant number of patients in a similar way to the present study [14,15,16,17]. However, these studies did not assess the quality of the chemotherapeutic management in detail.…”

Primary retroperitoneal dysgerminoma presenting as an adrenal tumor: A case report and literature review

“…In the largest international multicenter analysis conducted to date, all 635 patients with malignant EGCT were male 3 . Smaller national or institutional studies from Canada, Japan, Taiwan, Turkey, and India have found that 3 of 40, 8 1 of 30, 9 5 of 59, 4 1 of 18, 10 and 11 of 37 11 patients, respectively, were female, with most having intracranial germinomas or nonseminomatous GCT in the mediastinum or retroperitoneum. Studies in Belgium and Korea included 4 and 41 female patients, respectively, who had nonseminomatous GCT or mature teratomas of the retroperitoneum or other sites 12,13…”

Gonadal and extragonadal germ cell tumours in the United States, 1973–2007

“…Germ cell tumours (GCTs) most frequently arise in the gonads, but some develop extragonadally (Schmoll, 2002) (Ebi et al. , 2003) (Bokemeyer et al.…”

“…Germ cell tumours (GCTs) most frequently arise in the gonads, but some develop extragonadally (Schmoll, 2002) (Ebi et al, 2003) (Bokemeyer et al, 2002) (Bokemeyer et al, 2003). Extragonadal germ cell tumours (EGCTs) have similar morphology as gonadal germ cell tumours (GGCT), and most often occur in the midline of the body, e.g.…”