Outcome, Treatment, and Treatment Failures in Patients Suffering Localized Embryonal Paratesticular Rhabdomyosarcoma: Results From the "Cooperative Weichteilsarkom Studiengruppe" Trials CWS-86, -91, -96, and -2002P.
OBJECTIVE
To evaluate outcome in respect to local treatment strategies in 4 prospective CWS trials in patients with paratesticular rhabdomyosarcoma (PTRMS).


BACKGROUND
PTRMS patients have a high number of surgical treatment failures. Retroperitoneal lymph node (LN) involvement is common in patients more than 10 years, and the optimal treatment is unknown.


METHODS
A total of 173 patients with diagnosis of PTRMS were enrolled. Of these, 26 were excluded and 147 patients were finally analyzed. All patients were treated according to the Cooperative Soft Tissue Sarcoma (CWS) trial protocols.


RESULTS
The 5-year overall survival was 95.5%, and the 5-year event-free survival (EFS) was 89.8%. Positive predictive factors for EFS were age younger than 10 years and tumor size less than 5 cm. Surgical treatment failures were observed in 34 of 135 patients undergoing primary resection. Primary inguinal/iliacal LN sampling was carried out in 15 of 147 patients with no impact on the EFS (87.5%; P = 0.666). Secondary retroperitoneal LN dissection was done in 32 of 147 patients, of which only 8 patients had viable tumor and a worse outcome (EFS: 50%; P = 0.01). Loco-regional, combined, and metastatic relapses were observed in 13 of 147 patients.


CONCLUSIONS
The outcome of PTRMS patients is excellent and is hardly improvable. Positive predictors for outcome are age younger than 10 years and tumor size less than 5 cm. Primary LN sampling seems to have no impact on the EFS and should not be recommended. Inadequate surgery can be avoided by treatment in specialized centers. Secondary retroperitoneal LN dissection revealed a high number of unnecessary procedures, but patients with positive LN had a poor prognosis and require additional local therapy.
*Department of Pediatric Surgery and Urology, University Children's Hospital, Tuebingen, Germany †Department of Clinical Epidemiology and Applied Biometry, University of Tuebingen, Tuebingen, Germany ‡Department of Pediatric Hematology and Oncology, University Children's Hospital, Frankfurt/Main, Germany §Department of Pediatric Pathology, University Hospital of Schleswig-Holstein, Kiel, Germany ¶Department of Radiotherapy, University Hospital, Muenster, Germany ||Department of Pediatric Hematology, Oncology and Immunology, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany.
Guido
G
Seitz
Jörg
J
Fuchs
Peter
P
Martus
Thomas
T
Klingebiel
Ivo
I
Leuschner
Andreas
A
Schuck
Tobias M
TM
Dantonello
Ewa
E
Koscielniak
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols; D002648:Child; D003131:Combined Modality Therapy; D003952:Diagnostic Imaging; D006801:Humans; D008297:Male; D009367:Neoplasm Staging; D011379:Prognosis; D011446:Prospective Studies; D012008:Recurrence; D012208:Rhabdomyosarcoma / Q000473:pathology / Q000601:surgery* / Q000628:therapy; D018570:Risk Assessment; D012307:Risk Factors; D015996:Survival Rate; D013736:Testicular Neoplasms / Q000473:pathology / Q000601:surgery* / Q000628:therapy; D017211:Treatment Failure; D019564:Unnecessary Procedures; D055815:Young Adult