Primary pulmonary rhabdomyosarcoma in an adult with neurofibromatosis-1.

Rhabdomyosarcomas arising in various tissues associated with neurofibromatosis type 1 have been sporadically described in children and young adults. We report a unique case of primary pulmonary rhabdomyosarcoma in an adult with neurofibromatosis type 1. A right lower lobectomy was performed. The Intergroup Rhabdomyosarcoma Study IV postsurgical grouping classification was 1A. The patient is scheduled for chemotherapy without adjuvant radiation therapy as per standard pediatric protocol.