Vascular tumors (vascular malformations and hemangiomas) of the temporal bone are uncommon, and guidelines for their management have not been published. In an effort to develop an approach to their management, the present study examined the pathological, clinical, and surgical experience with ten of these lesions treated at the Otologic Medical Group, Inc., and St. Vincent Medical Center in Los Angeles from 1960 to 1980. Intratemporal vascular tumors occurred most frequently at two sites, the internal auditory canal and the geniculate ganglion. Histological features and clinical behavior did not correlate. Both vascular malformations and hemangiomata invaded the facial nerve. Surgical excision in these cases required severence and repair of the involved facial nerve. Complete surgical excision is the treatment of choice of vascular lesions of the temporal bone.
The emerging concept that aggressive adenomatous tumors of the temporal bone arise from the endolymphatic sac and constitute a distinct clinicopathologic entity merits wider recognition. These tumors share a common clinical pattern and exhibit consistent imaging and histopathologic features. Endolymphatic sac tumors (ELSTs) have been mistaken for other neoplasms such as paragangliomas, adenomatous tumors of mixed histology, ceruminomas, and choroid plexus papillomas. A review of the literature shows similarities among case studies of these aggressive adenomatous lesions. An analysis of the data supports the endolymphatic sac as an origin for these tumors. This report also presents an additional case of a less differentiated variant of this rare but important clinicopathologic entity.
Facial nerve hemangiomas are benign vascular tumors that arise within the temporal bone and have a histologic appearance similar to both cavernous hemangiomas and vascular malformations. In contrast to facial nerve schwannomas, these are extraneural tumors that cause symptoms by compression and tend to produce deficits when very small in size. We report our experience at the House Ear Clinic with 34 patients having these nonglomus intratemporal vascular tumors. Hemangiomas arising in the internal auditory canal tend to produce a progressive sensorineural hearing loss and are demonstrated with magnetic resonance imaging (MRI), whereas those at the geniculate ganglion are usually first seen with facial nerve symptoms and may require high-resolution computerized tomography (CT) for detection. Facial electromyography is helpful in establishing the diagnosis. Because of their extraneural nature, early diagnosis can permit removal of the tumor with preservation of facial nerves in some patients.
Eleven patients each with a benign intratemporal vascular tumor (hemangioma or vascular malformation) were assessed with computed tomography (CT). Clinical, surgical, and histologic correlations were also available. On CT scans, most of the 11 tumors were smaller than 10 mm. Four occurred in or around the internal acoustic canal, six at the geniculate ganglion, and one at the posterior genu. The involved bone margins were often unsharp, and "honeycomb" bone or intratumoral bone spicules were sometimes present. Intratemporal vascular tumors cause profound nerve deficits despite their small size and must be resected early to salvage nerve function.
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