점액종으로 오인되었던 부비동에 발생한 횡문근 육종 1례

Rhabdomyosarcoma(RMS), an uncommon malignant tumor with skeletal differentiation, is associated generally with a poor prognosis. In adults, the majority of rhabdomyosarcomas are found in the extremities and in the torso. In contrast, the majority of these tumors in children involve the head and neck area and the urogenital tract.

RMS accounts for 8% to 19% of all soft tissue sarcomas and 35% to 45% of these tumors occur in the head and neck region. The nose and paranasal sinuses account for 8%.

Pathologically, the tumors have been divided into four subgroupCpleomorphic, alveolar, embryonal, botryoid). Some parts of these tumors have a myxoma-like-appearance-myxoid stroma, and quite small and round cells, which may be confused histologically with a myxoma. A direct meningeal extension has been reported in 40% of the patients when the primary sites are adjacent to the meninges. With the triple (surgery, radiotherapy, and chemotherapy) method therapy, the 3 year-relapse-free interval of the tumors arising in the nasal cavity and paranasal sinus has been reported 45% of the cases, as opposed to 75% when the tumors are in other areas of the head and neck region (scalp, neck, parotid, oral cavity, larynx).

Recently, we experienced a case of a 27 years-old woman with RMS in the nasal cavity and paranasal sinus extending to the frontal lobe, who had been previously misdiagnosed as myxoma. We managed the patient with a surgical operation combined with postoperative chemotherapy and irradiation.