4 Histopathology of Vestibular Schwannoma



10.1055/b-0039-169158

4 Histopathology of Vestibular Schwannoma

Aditya Raghunathan and Caterina Giannini

4.1 Introduction


The eighth cranial nerve (vestibulocochlear nerve), which transmits auditory and vestibular input from the inner ear to the brainstem, represents the most common site of origin of intracranial schwannoma, followed at a distance by the fifth (trigeminal) nerve and other cranial nerves. Although the antiquated term “acoustic neurinoma/neuroma” is commonly used interchangeably with “vestibular schwannoma” (VS), the former is technically incorrect as the tumor is not a neuroma, nor does it typically arise from the cochlear nerve. Because these tumors arise from Schwann cells of the vestibular divisions of the eighth cranial nerve, the term VS is highly preferred. VSs are typically conventional schwannomas, benign nerve sheath tumors composed of well-differentiated Schwann cells at the histologic, immunophenotypic, and ultrastructural level. Uncommonly, cellular and epithelioid variants may occur.



4.2 Pathologic Features



4.2.1 Macroscopic Features


Vestibular schwannoma usually presents as a globular or lobulated mass on the surface of which the parent nerve is splayed. The cut surfaces are firm and often smooth, and sometimes may appear slightly lobulated. The color is usually homogenous light tan-pink to grey. Areas showing cystic change, dark red foci of hemorrhage, bright yellow foci corresponding to macrophage aggregates, and white areas corresponding to fibrosis may also be seen. The cystic and hemorrhagic changes may be extensive in fast growing or larger tumors.



Conventional Schwannoma

Most VSs have histopathological features of conventional schwannoma. Although frequently removed in a piecemeal fashion in multiple fragments, a well-formed fibrous pseudocapsule may be identified toward the periphery, and fascicles of the associated parent nerve may also be seen. The VS “capsule” has been shown to hold neoplastic cells, which carries implications with regard to tumor recurrence when “subcapsular dissection” is employed.s. Literatur VSs characteristically show two intermingled, histologically distinct regions, designated Antoni A and Antoni B (Fig. 4‑1 ). Antoni A regions are composed of compact arrangements of elongated cells, arranged as sheets, intersecting fascicles, and, occasionally, palisades. The cells have eosinophilic cytoplasmic processes and indistinct cell membranes.

Fig. 4.1 Schwannomas are composed of intermingled regions of compact (Antoni A, “A”) and loose (Antoni B, “B”) cellularity. Hyalinized vessels are frequently seen (arrow).


The nuclei are spindle shaped, tapering and wavy, with uniformly distributed chromatin and inconspicuous nucleoli. The most distinctive palisaded arrangements, termed “Verocay bodies,” are composed of two parallel rows of nuclei separated by a relatively anuclear zone of eosinophilic fibrillary cytoplasmic processes (Fig. 4‑2 ). In contrast, Antoni B regions are relatively hypocellular, with a loose-textured pale-blue, myxoid stroma. The tumor cells in these regions have somewhat more ovoid to rounded nuclei and loosely knit cytoplasmic processes. These Antoni A and B regions can be present in variable relative proportions. Mitotic figures are rarely seen in conventional schwannomas.

Fig. 4.2 Verocay bodies are a distinctive palisaded arrangement characteristic of schwannoma, composed of two parallel rows of nuclei separated by a relatively anuclear zone of eosinophilic fibrillary cytoplasmic processes.


Schwannomas are typically well vascularized, with blood vessels ranging from thick walled and hyalinized (Fig. 4‑1 , arrow) to thin walled and dilated. Episodes of prior intratumoral bleeding may result in perivascular hemosiderin deposition. Aggregates of foamy macrophages may be seen, particularly adjacent to Antoni B regions. In long-standing tumors, scattered cells may show degenerative nuclear changes (the so-called ancient change/schwannoma), with markedly enlarged and hyperchromatic nuclei that have lost chromatin texture and may occasionally show intranuclear pseudoinclusions (Fig. 4‑3 ). Foci of bland, infarct-like necrosis, hyalinization, and calcification may also be present in long-standing cases. These findings are of no prognostic significance.

Fig. 4.3 Scattered cells may show degenerative nuclear changes (arrows), including enlargement, hyperchromasia, loss of chromatin texture, and, occasionally, intranuclear pseudoinclusions. However, these are of no prognostic significance.



Epithelioid Schwannoma

In some schwannomas, the tumor cells may show epithelioid features, with more abundant cytoplasm, identifiable cell borders, and some degree of nuclear atypia.s. Literatur These may be diagnosed as epithelioid schwannomas when such features are extensive. A recent series of 58 epithelioid schwannomas arising in various systemic sites did not identify any association with adverse outcomes, even in the presence of identifiable mitotic activity and moderate nuclear atypia.s. Literatur



Cellular Schwannoma

Another variant that may rarely be seen in this region is the cellular schwannoma.s. Literatur ,​ s. Literatur This tumor has high cellularity, is predominantly composed of Antoni A areas, and often has readily identifiable mitotic figures. However, even with elevated mitotic activity (generally no more than 4 mitoses per 10 high-power fields), these tumors have been well established to behave akin to conventional schwannoma. The significance of recognizing this variant lies in averting misdiagnosis as a malignancy and potential overtreatment.

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May 13, 2020 | Posted by in NEUROSURGERY | Comments Off on 4 Histopathology of Vestibular Schwannoma

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