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

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.