PRACTICA OTO-RHINO-LARYNGOLOGICA
Vol. 99 No. 4 April 2006
Skull Base Surgery of a Rhabdomyosarcoma in the Ethmoid Sinus
Hidetaka Shirouzu, Akiteru Maeda, Hirohito Umeno and Tadashi Nakashima
(Kurume University School of Medicine)
Rhabdomyosarcoma occurs predominantly in infants and children, but less frequently in adolescents and young adults. We experienced a 33-year-old female with a rhabdomyosarcoma which originated in the ethmoid sinus and extended into the brain. After systemic neoadjuvant chemotherapy (ACT-D, VCR, CPA), an extended anterior skull base surgery with reconstruction was successfully performed. Additional radiotherapy of 41.2 Gy and chemotherapy (VP-16, IFM, CDDP, VCR, CPA, THP) were also carried out. The postoperative course of the patient was uneventful for one year. However, there has been recurrence in the oropharynx, cervical lymph node, anterior mediastinum and the brain. For each recurrence, operation, chemotherapy, radiotherapy, and cyberknife were performed, and the patient is presently doing well without any obvious recurrent tumor. Additional immunochemotherapy is now underway.
Key words : skull base surgery, rhabdomyosarcoma, ethmoid sinus