Treatment of children with nonmetastatic paratesticular rhabdomyosarcoma: results of the Malignant Mesenchymal Tumors studies (MMT 84 and MMT 89) of the International Society of Pediatric Oncology.
OBJECTIVE
To report the results of the Malignant Mesenchymal Tumors studies (MMT 84 and 89) of the International Society of Pediatric Oncology (SIOP) in males with nonmetastatic paratesticular rhabdomyosarcoma.


METHODS
From 1984 to 1994, 96 males were treated in SIOP protocols. Radical inguinal orchidectomy was recommended, but initial retroperitoneal lymph node dissection was not performed. Disease was staged according to the SIOP tumor-node-metastasis staging system. Treatment was stratified by stage. In the MMT 89 study, males with completely resected tumors at diagnosis received less chemotherapy (vincristine and dactinomycin) than patients in the MMT 84 study (ifosfamide, vincristine, and dactinomycin).


RESULTS
Median age at diagnosis was 65 months. Thirty-one tumors were larger than 5 cm, and 13 males were older than 10 years with a tumor larger than 5 cm. At a median follow-up of 7 years, 87 patients were alive; 79 were in first complete remission and eight were in second complete remission. Relapse occurred in 16 patients (17%). At 5 years, the overall survival (OS) rate was 92%, with an event-free survival (EFS) rate of 82%. OS and EFS were significantly worse for males with tumors greater than 5 cm and for males older than 10 years at diagnosis.


CONCLUSIONS
Males with paratesticular RMS have an excellent prognosis except for a selected group of patients older than 10 years or with tumor greater than 5 cm. Intensified chemotherapy incorporating alkylating agents for this subgroup may be preferred to the use of systematic lymphadenectomy to improve survival while minimizing the burden of therapy.
Stewart
Richard J
RJ
Queen's Medical Centre, Nottingham, United Kingdom.
Martelli
Hélène
H
Oberlin
Odile
O
Rey
Annie
A
Bouvet
Nathalie
N
Spicer
Richard D
RD
Godzinski
Jan
J
Stevens
Michael C G
MC
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003609:Dactinomycin / Q000627:therapeutic use*; D018572:Disease-Free Survival; D006801:Humans; D007069:Ifosfamide / Q000627:therapeutic use*; D007223:Infant; D008197:Lymph Node Excision; D008297:Male; D008637:Mesenchymoma / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery; D009364:Neoplasm Recurrence, Local / Q000188:drug therapy / Q000601:surgery; D009367:Neoplasm Staging; D012208:Rhabdomyosarcoma / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery; D016879:Salvage Therapy; D015996:Survival Rate; D013736:Testicular Neoplasms / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery; D016896:Treatment Outcome; D014750:Vincristine / Q000627:therapeutic use*
D003609:Dactinomycin; D014750:Vincristine; D007069:Ifosfamide