Leiomyosarcomas belong to one of the histological subtypes of soft tissue
sarcomas. They most often occur in genital, gastrointestinal tract and
extremities, and the appearance of these tumors in the bones (especially
head and neck) is very rare. Clinically, leiomyosarcomas are aggressive
tumors. Treatment is multidisciplinary and includes surgery, radiotherapy,
and chemotherapy. This case report presents a 61-year-old patient who was
referred to a maxillofacial surgeon due to congestion of the right side of
the nose, pain in the right eye, and occasional epistaxis. After complete
examination, including biopsy with histopathology and immunohistochemistry,
primary intraosseous leiomyosarcoma of the nose and paranasal cavities was
diagnosed. Since the tumor lesion was assessed as inoperable, the treatment
started with radiotherapy. Two and half years after the radiotherapy was
completed, there was good local control of the disease and no dissemination.
The case report illustrates the rarity of localization, challenges and
difficulties in multimodal treatment, and contribution of radiotherapy to
good treatment results.