Forty-nine patients with histologically proven
germ cell tumors arising in extragonadal sites were retrospectively reviewed. Included in the review were an additional seven patients with undifferentiated
tumors with a pathologic appearance compatible with that of a
germ cell tumor and elevated levels of serum
biomarkers (beta subunit of
human chorionic gonadotropin [beta-
HCG] +/- alpha-
fetoprotein [AFP]. Nineteen patients had a pure
seminoma arising in an extragonadal site, whereas 30 patients had nonseminomatous
germ cell tumors. Seven patients had primary undifferentiated
tumors with elevated levels of serum
biomarkers. Sixteen (84%) of the 19 patients with pure extragonadal
seminomas with normal levels of serum AFP are alive and free of disease. Eighteen of these 19 patients received
platinum-containing regimens and four had received prior
chemotherapy that failed. Of the patients with nonseminomatous
germ cell tumors, 12 (40%) of the 30 are alive and free of disease with
vinblastine/
bleomycin +/-
cisplatin (13 patients) or CISCAII (
cisplatin,
cyclophosphamide, and
doxorubicin) (nine patients) alternating CISCAII/VBIV (eight patients)
chemotherapy. None of the seven patients with undifferentiated
germ cell tumors are alive and free of disease. Three of the five patients with pure anterior mediastinal
endodermal sinus tumors treated with
chemotherapy remain alive and free of disease. Of the seven patients with
choriocarcinomas arising in extragonadal sites, three are alive and free of disease. A classification for patients with extragonadal
germ cell tumors incorporating site of origin, histology, and likelihood of being truly extragonadal is proposed. The implications of this classification are discussed.