Leiomyosarcoma (LMS) accounts for approximately 7% of all soft tissue
sarcomas and occurs most frequently in the gastrointestinal tract and
uterus. LMS of the nasal cavity and paranasal sinuses are rare and onl
y about 40 cases of LMS of the nose and paranasal sinuses have been re
ported in the literature. A further case of LMS of the right turbinate
is presented, treated twice by surgery. To date, the patient has been
free of disease for more than 48 months. The symptoms, pathology, tre
atment and prognosis, and origin of this tumor are discussed. Initiall
y, LMS should be treated by extensive surgical excision, but long-term
follow-up is essential due to the high rate of local recurrence. Radi
otherapy and chemotherapy are insufficient therapeutic approaches. Fre
quency of recurrence and prognosis depend on the tumor site. The progn
osis of LMS of the nasal cavity is better than LMS of the paranasal si
nuses.