Is alveolar histotype a prognostic factor in paratesticular rhabdomyosarcoma? The experience of Italian and German Soft Tissue Sarcoma Cooperative Group.
OBJECTIVE
To ascertain whether alveolar histology retains its adverse prognostic role in the subset of paratesticular rhabdomyosarcoma (RMS) patients, generally characterized by a very good outcome.


METHODS
Twenty pediatric patients were treated over a 25-year period using the protocols of the Italian and German Soft Tissue Sarcoma Cooperative Groups. Clinical characteristics at presentation were much the same as in non-alveolar patients.


RESULTS
The proportion of patients with alveolar histotype (8%) in paratesticular site was lower than in the general RMS population (20-30%). With a median follow-up of 122 months, 5-year EFS and OS were 78 and 89%, respectively.


CONCLUSIONS
Our data suggest a distinctly better clinical behavior of paratesticular alveolar RMS than when the disease occurs at other sites. These patients were more intensively treated than the embryonal cases, however, so-although a treatment intensity reduction may be desiderable-the idea of eliminating the alkylating agents (as in low-risk embryonal paratesticular RMS) must be considered with great caution.
Ferrari
Andrea
A
Pediatric Oncology Unit, Istituto Nazionale Tumori, Milano, Italy. andrea.ferrari@istitutotumori.mi.it
Bisogno
G
G
Casanova
M
M
Brecht
I B
IB
Alaggio
R
R
Cecchetto
G
G
Provenzi
M
M
Koscielniak
E
E
Treuner
J
J
Carli
M
M
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005858:Germany; D006801:Humans; D007558:Italy; D008197:Lymph Node Excision; D008297:Male; D009367:Neoplasm Staging; D011379:Prognosis; D011878:Radiotherapy; D018232:Rhabdomyosarcoma, Alveolar / Q000473:pathology* / Q000628:therapy; D018233:Rhabdomyosarcoma, Embryonal / Q000473:pathology* / Q000628:therapy; D015996:Survival Rate; D013736:Testicular Neoplasms / Q000473:pathology* / Q000628:therapy; D016896:Treatment Outcome