Undifferentiated embryonal sarcoma of the liver in adults.

Lenze F, Birkfellner T, Lenz P, Hussein K, Länger F, Kreipe H, Domschke W

Zusammenfassung

BACKGROUND: Undifferentiated embryonal sarcoma of the liver (UESL), a rare tumor that predominantly affects children, generally has been considered an aggressive neoplasm with an unfavorable prognosis. More recent reports have indicated that modern multimodal treatment and supportive care improve the survival of children with UESL. Data regarding the treatment and survival of adults have not been reviewed comprehensively, and only a few adult patients with UESL have been reported in the literature. METHODS: The authors analyzed demographics, treatment, and actuarial survival of all reported cases of UESL in patients aged >or=15 years (n = 67 patients). In addition, 1 case is presented of a patient with UESL who was treated successfully at the authors' institution. RESULTS: The median survival of all patients with UESL who were analyzed was 29 months. Patients who underwent complete tumor resection followed by adjuvant chemotherapy survived over a median follow-up of 28.5 months and had significantly better survival compared with patients who underwent surgical treatment alone. Patients who underwent an incomplete tumor resection had a tendency toward poorer outcomes. CONCLUSIONS: To the authors' knowledge, this is the first report to demonstrate a significant effect on survival for adjuvant chemotherapy after complete surgical resection of UESL in adults. The role of neoadjuvant chemotherapy was not evaluated in this study. In the case study presented herein, combined therapy with surgery and chemotherapy led to a complete, sustained remission that has lasted for >6 years to date.