PRIMARY PULMONARY RHABDOMYOSARCOMA IN CHILDHOOD - CLINICO-BIOLOGIC FEATURES IN 2 CASES WITH REVIEW OF THE LITERATURE

The cases of two children under three years of age with primary pulmon ary rhabdomyosarcoma and no associated lung malformations are reported and a review of the literature is presented. In both, complete surgic al removal of the tumor was performed and histologic examination revea led embryonal subtype. Flow cytometric assessment showed a tumor-cell diploid DNA content. Postoperative radio- and chemotherapy were carrie d out, but in spite of treatment both girls died because of disease pr ogression, fourteen and nine months after diagnosis. The importance of associated cystic lung malformations and DNA content in predicting cl inical outcome of primary pulmonary rhabdomyosarcoma is evaluated. (C) 1996 Wiley-Liss, Inc.