Journal of Clinical Otolaryngology Head and Neck Surgery

The Busan, Ulsan, Gyeoungnam Branch of Korean Society of Otolaryngology-Head and Neck Surgery

J Clin Otolaryngol Head Neck Surg 1998; 9(1):148-155

pISSN: 1225-0244, eISSN: 2713-833X

DOI: https://doi.org/10.35420/jcohns.1998.9.1.148

종설

점액종으로 오인되었던 부비동에 발생한 횡문근 육종 1례

문인희¹, 최익수¹, 강윤경², 김혜성²

A Case of Rhabdomyosarcoma, which was misdiagnosed as myxoma, in the Nasal Cavity and Paranasal Sinus

In Hee Moon¹, Ick Soo Choi¹, Yun Kyung Kang², Hye Sung Kim²

¹인제대학교 의과대학 이비인후과학교실, 해부학교실

²서울백병원

¹Department of Otolaryngology, Anatomical Phathology, Inje University, Seoul Paik Hospital

²College of Medicine, Inje University, Seoul Paik Hospital

© Copyright 1998 The Busan, Ulsan, Gyeoungnam Branch of Korean Society of Otolaryngology-Head and Neck Surgery. This is an Open-Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Published Online: May 31, 2020

ABSTRACT

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.

Keywords: Rhabdomyosarcoma; Paranasal sinus; Myxoma