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Tumors displaying hybrid schwannoma and neurofibroma features in patients with neurofibromatosis type 2
- Source :
- Clinical Neuropathology
- Publication Year :
- 2016
-
Abstract
- Although schwannoma and neurofibroma tumors are generally reported as distinct pathologic diagnoses, sporadic schwannoma/neurofibroma hybrid nerve sheath tumors have been reported in the general population with components of both entities. We report the clinicopathological features of these hybrid nerve sheath tumors in patients with neurofibromatosis type 2 (NF2). A retrospective review of nerve sheath tumor surgical specimens from patients with NF2 enrolled at the National Institutes of Health was performed. Those specimens reported to have schwannoma-like and neurofibromalike features were selected for further characterization by morphology, immunohistochemical panel (CD34, S100, neurofilament triplet protein (immunostain) (NFTP), epithelial membrane antigen (EMA)), and confirmation as hybrid tumors. Of 43 total NF2 patients undergoing resection of nerve sheath tumors, 11 specimens from 11 (26%) patients were found to be benign nerve sheath tumors exhibiting hybrid features of both neurofibroma and schwannoma. Immunohistochemical studies showed the schwannoma component to be S100+, CD 34- while the neurofibroma component was CD34+, variable S100+. Our experience emphasizes the importance of including this distinct tumor subtype, the schwannoma/neurofibroma hybrid tumor, in the differential diagnosis of nerve sheath tumors in NF2 patients and suggests that the relationship between neurofibroma and schwannoma tumors is closer than previously suspected..
- Subjects :
- Male
Pathology
medicine.medical_specialty
Neurofibromatosis 2
neurofibromatosis type 2
Adolescent
peripheral nerve sheath tumor
Population
CD34
hybrid tumors
Schwannoma
Pathology and Forensic Medicine
03 medical and health sciences
0302 clinical medicine
medicine
otorhinolaryngologic diseases
Biomarkers, Tumor
Neurofibroma
Humans
Neurofibromatosis type 2
education
Child
schwannoma
Neuropathology
Retrospective Studies
education.field_of_study
integumentary system
business.industry
General Medicine
Middle Aged
medicine.disease
Immunohistochemistry
Nerve sheath tumor
Neurology
030220 oncology & carcinogenesis
Female
Neurology (clinical)
Differential diagnosis
business
030217 neurology & neurosurgery
Neurilemmoma
Research Article
Subjects
Details
- ISSN :
- 07225091
- Volume :
- 35
- Issue :
- 2
- Database :
- OpenAIRE
- Journal :
- Clinical neuropathology
- Accession number :
- edsair.doi.dedup.....4f190af88922e3fbdf455857544ae74a