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Adult primary extragonadal germ cell tumors: Treatment results and long-term follow-up.

BACKGROUND
Primary extragonadal germ cell tumors (PEGCT) are rare neoplasms. They have a poor prognosis, different behavior, and natural course compared to their gonadal counterparts. Both primary and salvage treatment of these tumors constitute a challenge. We retrospectively evaluated the clinicopathologic status, therapeutic implications, and outcome of our patients with PEGCT.


PROCEDURE
Between 1991 and 2000, 18 patients with PEGCT (median age 31 years; range 17-63), diagnosed with tru-cut biopsy and treated with cisplatin-based chemotherapy, were evaluated in respect to treatment response and outcome.


RESULTS
Cisplatin-based chemotherapy achieved a complete response rate of 27.8% and a partial response rate of 55.5%. Overall response rate was 83.3%. Only three patients were unresponsive to chemotherapy; an additional six patients with residual mass underwent surgical resection and were rendered disease-free by surgery. The 5-year actuarial event-free and overall survival were 63.4 and 71.3%, respectively.


CONCLUSIONS
The outcomes of our patients with extragonadal primaries including mediastinal localization appear to be slightly better than those previously reported. Multimodality therapy is essential for these patients and given the relatively poor prognosis, prospective trials with large sample sizes, and new treatment approaches to improve outcome are required.