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Is alveolar histotype a prognostic factor in paratesticular rhabdomyosarcoma? The experience of Italian and German Soft Tissue Sarcoma Cooperative Group
Author(s) -
Ferrari Andrea,
Bisogno G.,
Casanova M.,
Brecht I.B.,
Alaggio R.,
Cecchetto G.,
Provenzi M.,
Koscielniak E.,
Treuner J.,
Carli M.
Publication year - 2004
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.10460
Subject(s) - medicine , rhabdomyosarcoma , alveolar rhabdomyosarcoma , soft tissue , sarcoma , soft tissue sarcoma , alveolar soft part sarcoma , embryonal rhabdomyosarcoma , presentation (obstetrics) , population , histology , pathology , surgery , radiology , oncology , environmental health
Abstract Purpose 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. Patients and 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. Conclusion 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. © 2003 Wiley‐Liss, Inc.