Histiocytosis of the temporal bone

Histiocytosis is a rare disease of unknown aetiology which commonly affects the head and neck region. In the ear it can closely mimic acute and chronic ear disease leading to a confusion in diagnosis. It can be easily misdiagnosed unless a high index of clinical suspicion is maintained. We present a case of Letterer Siwe disease of the temporal bone which presented with bilateral mastoid abscesses.     

Haemangiopericytoma of the temporal bone

Haemangiopericytoma is an uncommon vascular tumour with a widespread distribution. Although meningeal involvement is well recognized, only a few sporadic cases of temporal bone lesions have been documented, all with doubtful sites of origin. Late presentation together with the restrictive anatomy of this region often precludes its effective removal and even minimal residual disease may progress rapidly. A series of three such patients are presented in order to discuss the natural history, histological features and treatment of this disease.     

Meningiomas of the temporal bone

Meningiomas should be considered in the differential diagnosis of otologic tumors arising from the middle ear and mastoid. It is generally recognized that meningiomas may invade the temporal bone via direct extension from the intracranial space (secondary). It is not generally recognized that meningiomas can develop initially in the middle ear cleft (primary). Representative cases of primary and secondary meningiomas of the temporal bone are described, and clinical aspects of diagnosis and surgical management are discussed. In order to assess the pathogenesis of meningiomas of the temporal bone, histological studies were done on 200 temporal bone specimens. In certain specimens, ectopic arachnoid granulations, from which primary meningiomas may originate, were identified and described, according to location. One specimen was most unusual in that it contained ectopic glial as well as arachnoidal tissue.     

Chondrosarcoma of the temporal bone

Eight patients with a chondrosarcoma of the temporal bone have been treated at the National Hospital for Neurology and Neurosurgery over a 16-year-period. Patients usually presented with symptoms and signs of lower cranial nerve palsies, though in most cases these resolved after surgery. This result, combined with the fact long-term survival can be achieved, makes surgical treatment of these tumours the best option, as the response to primary radiotherapy is uncertain. Surgical access is difficult, but an infratemporal approach is probably the most satisfactory. The use of post-operative adjuvant radiotherapy may provide some benefit. Long-term follow-up is necessary, and for this magnetic resonance imaging (MRI) is preferable to computed tomography (CT) scanning.     

Chondrosarcoma of the temporal bone

Chondrosarcoma of the temporal bone is a rare disease, with only 36 cases of this tumor having been reported in the English language literature. We report the case of a 52-year-old woman with myxoid chondrosarcoma of the temporal bone whose only symptom was progressive hearing loss. The tumor was removed using aspiration, mastoidectomy and tympanotomy, with retention of the jugular bulb. No recurrence has been noted 45 months after the operation. Diagnosis was established from histological examination of the surgical specimens. The clinical characteristics and management of this rare lesion are discussed.     

Chondroblastoma of the temporal bone

Chondroblastomas are highly destructive tumors that are derived from immature cartilage cells. The occurrence of this tumor in the temporal bone or skull base is uncommon. Approximately 70 cases have previously been reported, several of which have involved the temporomandibular joint (TMJ). We report here the case of a 67-year-old woman who presented with right-sided mixed hearing loss, a right external auditory canal mass, ear fullness, otalgia, blood-stained otorrhea, and pain around the TMJ, associated with difficulty in opening the mouth. CT and MRI revealed a mass involving the TMJ, infratemporal fossa, and pterygopalatine fossa. The patient underwent tumor resection via an infratemporal fossa approach type B. Gross total tumor removal was achieved, with no facial nerve paralysis or other complications observed after surgery. No recurrence or residual tumors were observed on CT and MRI, even after 7.5 years of follow-up. We conclude that temporal bone chondroblastomas are extremely rare and aggressive, but the outcome after appropriate surgical treatment is favorable. From the review, it may be particularly important to deal with tumors that involve the TMJ, which could affect the long-term outcomes, as well as tumor recurrence.