Morphologic and immunohistochemical features of malignant peripheral nerve sheath tumors and cellular schwannomas

Cellular schwannoma is an uncommon, but well-recognized, benign peripheral nerve sheath tumor, which can be misdiagnosed as malignant peripheral nerve sheath tumor. To develop consensus diagnostic criteria for cellular schwannoma, we reviewed 115 malignant peripheral nerve sheath tumor and 26 cellul...

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Bibliographic Details
Published in:Modern pathology 2015-02, Vol.28 (2), p.187-200
Main Authors: Pekmezci, Melike, Reuss, David E, Hirbe, Angela C, Dahiya, Sonika, Gutmann, David H, von Deimling, Andreas, Horvai, Andrew E, Perry, Arie
Format: Article
Language:English
Subjects:
692/53/2421
692/53/2422
Adult
Biomarkers, Tumor - analysis
Diagnosis, Differential
Disease-Free Survival
Female
Humans
Immunohistochemistry
Kaplan-Meier Estimate
Laboratory Medicine
Male
Medicine
Medicine & Public Health
Middle Aged
Nerve Sheath Neoplasms - diagnosis
Nerve Sheath Neoplasms - mortality
Neurilemmoma - diagnosis
Neurilemmoma - mortality
original-article
Pathology
Sensitivity and Specificity
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Summary:Cellular schwannoma is an uncommon, but well-recognized, benign peripheral nerve sheath tumor, which can be misdiagnosed as malignant peripheral nerve sheath tumor. To develop consensus diagnostic criteria for cellular schwannoma, we reviewed 115 malignant peripheral nerve sheath tumor and 26 cellular schwannoma cases from two institutions. Clinical data were retrieved from the electronic medical records, and morphologic features, maximal mitotic counts, Ki67 labeling indices, and immunohistochemical profiles (SOX10, SOX2, p75NTR, p16, p53, EGFR, and neurofibromin) were assessed. Several features distinguish cellular schwannoma from malignant peripheral nerve sheath tumor. First, in contrast to patients with malignant peripheral nerve sheath tumor, no metastases or disease-specific deaths were found in patients with cellular schwannoma. More specifically, 5-year progression-free survival rates were 100 and 18%, and 5-year disease-specific survival rates were 100 and 32% for cellular schwannoma and malignant peripheral nerve sheath tumor, respectively. Second, the presence of Schwannian whorls, a peritumoral capsule, subcapsular lymphocytes, macrophage-rich infiltrates, and the absence of fascicles favored the diagnosis of cellular schwannoma, while the presence of perivascular hypercellularity, tumor herniation into vascular lumens, and necrosis favor malignant peripheral nerve sheath tumor. Third, complete loss of SOX10, neurofibromin or p16 expression, or the presence of EGFR immunoreactivity was specific for malignant peripheral nerve sheath tumor (P
ISSN:0893-3952
1530-0285
DOI:10.1038/modpathol.2014.109