EXTRACRANIAL NON-TESTICULAR TERATOMA IN CHILDHOOD AND ADOLESCENCE - INTRODUCTION OF A RISK SCORE FOR STRATIFICATION OF THERAPY

Patients and methods According to previous literature incomplete turne r resection, coccygeal or ovarian primary site and immaturity are know n risk factors for relapse in teratoma. To establish a risk score poin ts are allocated for resection, primary site and histology in the foll owing manner and added: incomplete resection 4 points, primary site co ccyx 3 points, ovary 2 points, other site 1 point, histological gradin g 0-3 points, This score system is evaluated on 270 extracranial non-t esticular teratoma cases collected between 1982 and 1995 in the MAKEI cooperative treatment protocols of the German Society of Pediatric Onc ology and Hematology. Treatment was resection alone (230 patients) or resection followed by postoperative adjuvant chemotherapy (40 patients ). Results Patients treated with surgery alone: 28/230 (12%) patients relapsed. 14/230 (6%) patients showed highly malignant histology (most ly yolk sac tumor) in relapse. Mortality in case of relapse was 6/28 ( 21%). Patients scoring greater than or equal to 6 points (n=45) had a relapse rate of 21/45 (47%) resulting in a 23%-mortality (5/21), Patie nts scoring <6 points (n=185) had a 4%-relapse risk (8/185) resulting in 13%-mortality (1/8) (p<0.01), Patients treated with surgery and adj uvant chemotherapy: 7/40 patients (18%) suffered a relapse, none of th em showing malignant histology. Mortality rate in case of relapse was 3/7 (43%). Patients scoring greater than or equal to 6 points initiall y treated with adjuvant chemotherapy (n=18) had a relapse rate of 7/18 (39%), compared to patients scoring < 6 points (n=22), in whom no rel apses occured (p<0.01). There were no highly malignant relapses in the group treated with adjuvant chemotherapy. Regardless of the scored po ints the difference in highly malignant relapse histology comparing th e group treated with surgery and adjuvant chemotherapy to the group tr eated with surgery was statistically significant (p=0.02). Conclusion The risk score system marks a high risk group including 63/270 (23%) o f all evaluated extracranial al non-testicular teratoma cases (scoring greater than or equal to 6 points), In this group 28/35 (80%) of rela pses and 8/9 (89%) of tumor deaths occurred. For this high risk group a randomized trial will be suggested to evaluate the effect of adjuvan t chemotherapy on the rate of malignant relapses. It should also be in vestigated, if adjuvant chemotherapy will influence relapse rate and m ortality.