A Case of Chondrosarcoma With Maffucci Syndrome Presenting Adult Onset Otitis Media With Effusion

Author: Gil Myeong SON ¹ ; Ki Joon PARK ; Seung Su HA ; Chan Hum PARK
Author Information

1. Department of Otorhinolaryngology-Head and Neck Surgery, Chuncheon Sacred Heart Hospital, Hallym University College of Medicine, Chuncheon, Korea
Publication Type:Case Report
From:Korean Journal of Otolaryngology - Head and Neck Surgery 2022;65(9):538-542
CountryRepublic of Korea
Language:Korean
Abstract: Maffucci syndrome is a non-hereditary disease, in which benign cartilage tumor called enchondroma occurs throughout the body, causing various symptoms. In particular, when chondrosarcoma occurs in the skull base, various neurologic symptoms can appear. Many of these symptoms have been diagnosed and reported by neurosurgeons. This paper presents a rare case of Maffuci syndrome. The patient is a 34-year-old female who first visited the otolaryngology outpatient department for ear fullness without any other neurological symptoms. The initial diagnosis was adult onset otitis media with effusion (OME), but further examination and biopsy revealed skull base chondrosarcoma. Moreover, the mass was extensively invading the skull base, so surgical treatment would have been dangerous; thus, careful follow-up has been conducted for the patient in the outpatient clinic. This report highlights the importance of nasopharyngoscopy as well as other imaging tests to observe nasopharyngeal masses in OME patients with congenital or acquired diseases, which are known to sporadically develop tumor.