초록 열기/닫기 버튼
Maffucci syndrome is a non-hereditary disease, in which benign cartilage tumor called enchondromaoccurs throughout the body, causing various symptoms. In particular, when chondrosarcomaoccurs in the skull base, various neurologic symptoms can appear. Many of thesesymptoms have been diagnosed and reported by neurosurgeons. This paper presents a rarecase of Maffuci syndrome. The patient is a 34-year-old female who first visited the otolaryngologyoutpatient department for ear fullness without any other neurological symptoms. Theinitial diagnosis was adult onset otitis media with effusion (OME), but further examinationand biopsy revealed skull base chondrosarcoma. Moreover, the mass was extensively invadingthe skull base, so surgical treatment would have been dangerous; thus, careful follow-up hasbeen conducted for the patient in the outpatient clinic. This report highlights the importanceof nasopharyngoscopy as well as other imaging tests to observe nasopharyngeal masses inOME patients with congenital or acquired diseases, which are known to sporadically developtumor.
