Acoustic Neuroma and Other Skull Base Tumors



Acoustic Neuroma and Other Skull Base Tumors


Randy S. D’Amico

Michael B. Sisti



VESTIBULAR SCHWANNOMA (ACOUSTIC NEUROMA)


EPIDEMIOLOGY

Vestibular schwannomas (e.g., acoustic neuroma, acoustic neurofibroma) are slow-growing, benign extra-axial tumors that arise commonly from the superior vestibular portion of the eighth cranial nerve (CN VIII). Vestibular schwannomas comprise 8% to 10% of intracranial tumors. Tumors may arise either sporadically (95%) or in association with neurofibromatosis type 2 (NF2). The incidence of sporadic cases is believed to be 1 per 100,000 personyears, with a median age of 50 years. As a result of the prevalence of magnetic resonance imaging (MRI), the incidence of vestibular schwannomas has increased recently, whereas the typical size at diagnosis has decreased.


PATHOBIOLOGY

Vestibular schwannomas are histopathologically benign tumors that grow slowly from the Schwann cell sheath surrounding the vestibular branch of CN VIII. Tumors are composed of Antoni A (narrow elongated bipolar cells) and Antoni B fibers (loose reticulated pattern of cells). Verocay bodies are frequently seen and consist of acellular eosinophilic areas surrounded by parallel arrangements of spindle-shaped Schwann cells.






FIGURE 102.1 Vestibular Schwannoma. A: T1-weighted axial MRI before contrast shows large hypo intense mass within the CPA angle exerting mass effect on the brainstem. B: T1-weighted axial MRI post-contrast shows a vestibular schwannoma with expansion of the internal auditory meatus.

Vestibular schwannomas are typically unilateral lesions. Bilateral vestibular schwannomas occur in less than 5% of patients and are a defining characteristic of NF2, an autosomal dominant disorder involving the NF2 gene located on chromosome 22 band q11-13.1. This gene normally encodes the protein merlin (schwannomin), which is thought to play a role in membrane stability through interactions with cytoskeletal and integral membrane proteins. Loss of function of merlin in Schwann cells has been associated with both sporadic and NF2-related vestibular schwannomas, and the gene is often considered a classic tumor suppressor. Cytologically, vestibular schwannomas that arise in NF2 are identical to sporadic cases but with a greater tendency to infiltrate the nerve rather than displace it. In addition to patients with bilateral lesions, any patient younger than 40 years of age with a unilateral vestibular schwannoma should also undergo evaluation for NF2.

Tumors often originate within the internal acoustic meatus (intracanalicular; Fig. 102.1) and may extend into the cerebellopontine angle (extracanalicular). Mass effect from extracanalicular tumor may compromise function of cranial nerves, brain stem nuclei, and the cerebellum. Tumors typically follow three growth patterns: (1) no or
very slow growth; (2) slow growth (2 mm/yr linear growth on imaging studies); or (3) fast growth (> 8 mm/yr) as a result of the enlargement of cystic components or rarely, intratumoral hemorrhage.








TABLE 102.1 Symptoms in Vestibular Schwannoma (1,000 Patients)



































Symptoms


%


Hearing loss


95


Tinnitus


63


Dysequilibrium/vertigo


61


Headache


12


Trigeminal nerve disturbance


9


Facial paresis


5.2


Caudal cranial nerve disturbances


2.7


Change of taste


2


Diplopia


1.8


From Matthies C, Samii M. Management of 1000 vestibular schwannomas (acoustic neuromas): clinical presentation. Neurosurgery. 1997;40(1):1-9; discussion 9-10.



CLINICAL FEATURES

Vestibular schwannomas most commonly present with progressive unilateral hearing loss, characterized by difficulty with speech discrimination, especially when talking on the telephone. The next most common symptoms include tinnitus, followed by balance difficulties (Table 102.1). This triad of symptoms is related to pressure on the eighth nerve complex in the internal auditory canal. Other presenting symptoms include facial paralysis, trigeminal neuralgia, and rarely, hydrocephalus and brain stem compression in the setting of larger tumors. Although this constellation of symptoms may occur with any mass in the cerebellopontine angle, including meningioma, cholesteatoma, or trigeminal neuroma, only rarely will these other lesions cause tinnitus or hearing dysfunction.


Jul 27, 2016 | Posted by in NEUROLOGY | Comments Off on Acoustic Neuroma and Other Skull Base Tumors

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