Primary myxoid temporal bone tumor: A rare neurosurgical manifestation of Carney complex?

BACKGROUND: Carney complex (CNC) is a rare autosomal dominant syndrome, manifesting mainly with cardiac, cutaneous, and mucosal myxomas. Osteochondromyxoma is known as an extremely rare bone lesion of CNC which usually appears early in life; however, there were no reports of primary bone myxoma of the skull in the patients with CNC. We present the first case of primary myxoid skull tumor in the patient with CNC. CASE DESCRIPTION: We report the left temporal bone tumor with significant intracranial mass effect in a 58-year- old woman already diagnosed with CNC. Complete resection of the tumor with skull bone reconstruction was carried out. Pathological diagnosis was labeled the lesion as an atypical myxoid spindle cell neoplasm. The features were different from atrial myxoma and osteochondromyxoma which has been described in CNC. There have been no signs of recurrence in 9 years follow-up. CONCLUSION: To the best of our knowledge, there have been no reports of the primary myxoid tumors in the skull in the patients with CNC. This paper highlighted a possible important association between CNC and primary intracranial myxoid tumors.