Hypoglossal schwannomas are rare skull base tumors. Furthermore, cystic hypoglossal schwannomas are extremely uncommon. We report the first case of a large cystic hypoglossal schwannoma with a fluid-fluid level. A 36-year-old woman presented with increased intracranial pressure and cerebellar signs without hypoglossal nerve palsy. Magnetic resonance imaging showed a predominantly cystic mass with a fluid-fluid level in the foramen magnum region extending into the hypoglossal canal. The intracranial tumor was largely removed via a midline suboccipital subtonsillar approach, leaving only a tiny residue in the hypoglossal canal. Histology confirmed a schwannoma with relative hypervascularity. Twenty months later, the tumor recurred and presented as a multicystic dumbbell-shaped lesion, extending intra-and extracranially through the enlarged hypoglossal canal. A complete resection of the intracranial and intracanalicular parts of the tumor was achieved with a small extracranial remnant treated by radiosurgery. Histology revealed a focal increased K i 67 proliferative index. In this report, we discuss the possible reasons for the absence of hypoglossal nerve palsy and the potential mechanism of the formation of the fluid-fluid level, and we consider the treatment of this lesion.KEYWORDS: Hypoglossal schwannoma, cystic, fluid-fluid level, schwannoma Schwannomas are benign, slow-growing neoplasms of the myelin-producing Schwann cells in the peripheral sensorimotor nervous system. In more than 90% of cases, these present as vestibular schwannomas.
1Schwannomas that arise from the hypoglossal nerve, a pure motor nerve, are very rare, accounting for only 5% of all nonacoustic intracranial schwannomas. 2 To date, around 100 cases of hypoglossal schwannoma have been reported in the English literature. Hypoglossal schwannoma usually originates intracranially but can also extend extracranially through the hypoglossal canal in a so-called ''dumbbell'' fashion or can arise purely from the extracranial portion of the XII nerve. According to Kaye et al's 4 classification of the jugular foramen neurinomas, the hypoglossal schwannomas documented in the literature are divided into three types: type A, intracranial in 31.5%; type B, dumbbellshaped or extra-and intracranial in 50%; type C, extracranial in 18.5%.
5Schwannomas are frequently solid masses, and cystic patterns are uncommon. A fluid-fluid level has been observed in a few intracranial cystic schwannomas on magnetic resonance image (MRI), such as schwannomas that originate from the V, VIII, and IX nerves.
6-8Here we describe a unique case of a monocystic hypoglossal schwannoma with fluid-fluid level. To our