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In this group of tumors, symptoms and signs of general cerebral impairment and increased pressure occur late or not at all Instead, special syndromes referable to particular intracranial loci arise and progress slowly One arrives at the correct diagnosis by localizing the lesion accurately from the neurologic ndings and by reasoning that the etiology must be neoplastic because of an afebrile and steadily progressive nature Investigation by CT, MRI, and other special studies will usually con rm the clinical impression The tumors that most often produce these unique intracranial syndromes are acoustic neuroma and other tumors of the cerebellopontine angle, craniopharyngioma, pituitary adenomas and various nonneoplastic enlargements of the sella, meningiomas of the sphenoid ridge and olfactory groove, glioma of the optic nerve and chiasm, pontine glioma, chordoma and chondrosarcoma, glomus

Figure 31-15 Colloid cyst of the third ventricle MRI in the sagittal and axial planes Most cases have associated hydrocephalus, which is not apparent here

Figure 31-16 Bilateral acoustic schwannomas in neuro bromatosis type 2 MRI in the axial plane, without (left) and with (right) enhancement

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jugulare and carotid body tumors, as well as a number of other erosive tumors at the base of the skull Acoustic Neuroma (Vestibular Schwannoma) This tumor was rst described as a pathologic entity by Sandifort in 1777, rst diagnosed clinically by Oppenheim in 1890, and rst recognized as a surgically treatable disease in the early 1900s Cushing s monograph (1917) was a milestone, and the papers of House and Hitselberger and of Ojemann and colleagues provide excellent descriptions of the modern diagnostic tests and surgical treatment as well as comprehensive bibliographies Approximately 3000 new cases of acoustic neuroma are diagnosed each year in the United States (incidence rate of 1 per 100,000 per year) The tumor occurs occasionally as part of von Recklinghausen neuro bromatosis, in which case it takes one of two forms In classic von Recklinghausen disease (peripheral or type 1 neuro bromatosis, see page 869), a schwannoma may sporadically involve the eighth nerve, usually in adult life, but it may involve any other cranial nerve (particularly trigeminal) or spinal nerve root Rarely if ever do bilateral acoustic neuromas occur in this form of the disease However, bilateral acoustic neuromas are the hallmark of the genetically distinct neuro bromatosis type 2, in which they practically always occur before the age of 21 and show a strong (autosomal dominant) heredity (Fig 31-16) Schwannomas are distinguished from neuro bromas (composed of both Schwann cells and broblasts) found in peripheral nerves of type 1 von Recklinghausen disease A small percentage of neuro bromas become malignant, a phenomenon that is highly unusual in schwannomas In this context, it should be stated that there is a form of familial schwannomatosis, characterized by multiple schwannomas without vestibular tumors, that maps genetically to chromosome 22 but is distinct from NF2 The primary gene defect in this familial schwannomatosis has not been de ned (MacCollin et al) The usual acoustic neuroma in adults presents as a solitary tumor Being a schwannoma, it originates in nerve The examina-.

tion of small tumors reveals that they practically always arise from the vestibular division of the eighth nerve, just within the internal auditory canal (Fig 31-17) As the eighth nerve schwannoma grows, it extends into the posterior fossa to occupy the angle between the cerebellum and pons (cerebellopontine angle) In this lateral position it is so situated as to compress the seventh, fth, and less often the ninth and tenth cranial nerves, which are implicated in various combinations Later it displaces and compresses the pons and lateral medulla and obstructs the CSF circulation; very rarely, it is a source of subarachnoid hemorrhage

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