Malignant retroperitoneal and abdominal germ cell tumors: an intergroup study.
OBJECTIVE
This randomized study examined survival (S) and event-free survival (EFS) rates using high-or standard-dose cisplatin-based combination chemotherapy and surgical resection for this subset of germ cell tumors.


METHODS
Twenty-six of 317 patients enrolled on the POG 9049/COG 8882 intergroup study for malignant germ cell tumors had abdomen or retroperitoneum as the primary site. Twenty-five of 26 were eligible for inclusion (n = 25). Patients had biopsy or resection at diagnosis and randomization to chemotherapy including etoposide, bleomycin, and either standard-dose (PEB) or high-dose cisplatin (HDPEB). In patients with initial biopsy, delayed resection was planned.


RESULTS
Median age was 26 months. There were 14 girls and 11 boys. There were 3 stage I to II, 5 stage III, and 17 stage IV patients. Surgical management included primary resection in 5, resection after chemotherapy in 13, and biopsy or partial resection in 7 patients. Overall 6-year EFS rate was 82.8% +/- 10.9%, and 6-year survival rate was 87.6% +/- 9.3%. By group, 6-year survival rate was 90.0% +/- 11.6% for PEB and 85.7 +/- 14.5% for HDPEB. Deaths include one from sepsis, one from malignant tumor progression, and one from bulky disease caused by benign components despite response of the malignant elements to chemotherapy.


CONCLUSIONS
Malignant germ cell tumors arising in the abdomen and retroperitoneum have an excellent prognosis despite advanced stage in most children. Aggressive resection need not be undertaken at diagnosis, but a concerted attempt at complete surgical removal after chemotherapy is important to distinguish viable tumor from necrotic tumor or benign elements that will not benefit from further chemotherapy.
Billmire
D
D
Section of Pediatric Surgery, J.W. Riley Hospital for Children, Indianapolis, Indiana 46202, USA.
Vinocur
C
C
Rescorla
F
F
Colombani
P
P
Cushing
B
B
Hawkins
E
E
Davis
M
M
London
W B
WB
Lauer
S
S
Giller
R
R
D000008:Abdominal Neoplasms / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology / Q000601:surgery; D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001761:Bleomycin / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D002945:Cisplatin / Q000008:administration & dosage; D003131:Combined Modality Therapy; D018450:Disease Progression; D018572:Disease-Free Survival; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D018237:Germinoma / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology / Q000601:surgery; D006801:Humans; D007223:Infant; D015993:Life Tables; D008297:Male; D009367:Neoplasm Staging; D012074:Remission Induction; D012186:Retroperitoneal Neoplasms / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology / Q000601:surgery; D016019:Survival Analysis; D016896:Treatment Outcome
D001761:Bleomycin; D005047:Etoposide; D002945:Cisplatin