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Epithelioid Schwannoma

MedGen UID:
1791335
•Concept ID:
C5554894
•
Neoplastic Process

Definition

A schwannoma characterized by the presence of epithelioid cells with eosinophilic cytoplasm, within a myxoid and/or hyalinized stroma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEpithelioid Schwannoma

Professional guidelines

PubMed

Mesenchymal tumours with melanocytic expression: a potential pitfall in the differential diagnosis of malignant melanoma.
Saleh JS, Whittington CP, Bresler SC, Patel RM
Pathology 2023 Mar;55(2):258-268. Epub 2022 Dec 28 doi: 10.1016/j.pathol.2022.12.340. PMID: 36639332

Recent clinical studies

Etiology

Update on Peripheral Nerve Sheath Tumors.
Martinez AP, Fritchie KJ
Surg Pathol Clin 2019 Mar;12(1):1-19. Epub 2018 Dec 17 doi: 10.1016/j.path.2018.10.001. PMID: 30709438
Epithelioid Schwannomas: An Analysis of 58 Cases Including Atypical Variants.
Hart J, Gardner JM, Edgar M, Weiss SW
Am J Surg Pathol 2016 May;40(5):704-13. doi: 10.1097/PAS.0000000000000589. PMID: 26752543
Malignant peripheral nerve sheath tumors of the eighth cranial nerve arising without prior irradiation.
Carlson ML, Jacob JT, Habermann EB, Glasgow AE, Raghunathan A, Link MJ
J Neurosurg 2016 Nov;125(5):1120-1129. Epub 2016 Jan 8 doi: 10.3171/2015.7.JNS151056. PMID: 26745487
Plexiform pigmented schwannoma of the uvea.
Saavedra E, Singh AD, Sears JE, Ratliff NB
Surv Ophthalmol 2006 Mar-Apr;51(2):162-8. doi: 10.1016/j.survophthal.2005.12.004. PMID: 16500216
Epithelioid variant of malignant peripheral nerve sheath tumor (malignant epithelioid schwannoma).
Laskin WB, Weiss SW, Bratthauer GL
Am J Surg Pathol 1991 Dec;15(12):1136-45. doi: 10.1097/00000478-199112000-00004. PMID: 1746681

Diagnosis

Epithelioid schwannoma-Rare but unequivocal.
Müller CSL
J Cutan Pathol 2024 Jan;51(1):4-6. Epub 2023 Oct 21 doi: 10.1111/cup.14553. PMID: 37864510
Mesenchymal tumours with melanocytic expression: a potential pitfall in the differential diagnosis of malignant melanoma.
Saleh JS, Whittington CP, Bresler SC, Patel RM
Pathology 2023 Mar;55(2):258-268. Epub 2022 Dec 28 doi: 10.1016/j.pathol.2022.12.340. PMID: 36639332
Update on Peripheral Nerve Sheath Tumors.
Martinez AP, Fritchie KJ
Surg Pathol Clin 2019 Mar;12(1):1-19. Epub 2018 Dec 17 doi: 10.1016/j.path.2018.10.001. PMID: 30709438
SMARCB1/INI1 Loss in Epithelioid Schwannoma: A Clinicopathologic and Immunohistochemical Study of 65 Cases.
Jo VY, Fletcher CDM
Am J Surg Pathol 2017 Aug;41(8):1013-1022. doi: 10.1097/PAS.0000000000000849. PMID: 28368924
Benign epithelioid schwannoma.
Kindblom LG, Meis-Kindblom JM, Havel G, Busch C
Am J Surg Pathol 1998 Jun;22(6):762-70. doi: 10.1097/00000478-199806000-00015. PMID: 9630185

Therapy

Malignant epithelioid schwannoma of the mandible and the skull base.
Habal MB
J Craniofac Surg 1997 Sep;8(5):417-21. doi: 10.1097/00001665-199708050-00016. PMID: 9482085

Prognosis

Update on Peripheral Nerve Sheath Tumors.
Martinez AP, Fritchie KJ
Surg Pathol Clin 2019 Mar;12(1):1-19. Epub 2018 Dec 17 doi: 10.1016/j.path.2018.10.001. PMID: 30709438
SMARCB1/INI1 Loss in Epithelioid Schwannoma: A Clinicopathologic and Immunohistochemical Study of 65 Cases.
Jo VY, Fletcher CDM
Am J Surg Pathol 2017 Aug;41(8):1013-1022. doi: 10.1097/PAS.0000000000000849. PMID: 28368924
Malignant peripheral nerve sheath tumors of the eighth cranial nerve arising without prior irradiation.
Carlson ML, Jacob JT, Habermann EB, Glasgow AE, Raghunathan A, Link MJ
J Neurosurg 2016 Nov;125(5):1120-1129. Epub 2016 Jan 8 doi: 10.3171/2015.7.JNS151056. PMID: 26745487
Benign epithelioid schwannoma.
Kindblom LG, Meis-Kindblom JM, Havel G, Busch C
Am J Surg Pathol 1998 Jun;22(6):762-70. doi: 10.1097/00000478-199806000-00015. PMID: 9630185
Mediastinal malignant epithelioid schwannoma.
Fukai I, Masaoka A, Yamakawa Y, Niwa H, Eimoto T
Chest 1995 Aug;108(2):574-5. doi: 10.1378/chest.108.2.574. PMID: 7634904

Clinical prediction guides

SWI/SNF complex-deficient soft tissue neoplasms: An update.
Schaefer IM, Hornick JL
Semin Diagn Pathol 2021 May;38(3):222-231. Epub 2020 Jun 5 doi: 10.1053/j.semdp.2020.05.005. PMID: 32646614Free PMC Article
SMARCB1/INI1 Loss in Epithelioid Schwannoma: A Clinicopathologic and Immunohistochemical Study of 65 Cases.
Jo VY, Fletcher CDM
Am J Surg Pathol 2017 Aug;41(8):1013-1022. doi: 10.1097/PAS.0000000000000849. PMID: 28368924
Malignant peripheral nerve sheath tumors of the eighth cranial nerve arising without prior irradiation.
Carlson ML, Jacob JT, Habermann EB, Glasgow AE, Raghunathan A, Link MJ
J Neurosurg 2016 Nov;125(5):1120-1129. Epub 2016 Jan 8 doi: 10.3171/2015.7.JNS151056. PMID: 26745487
Syndecan-1 expression in different soft tissue tumours.
Orosz Z, Kopper L
Anticancer Res 2001 Jan-Feb;21(1B):733-7. PMID: 11299835
Primary mesenchymal tumors of the urinary bladder. A histological and immunohistochemical study of 30 cases.
Kunze E, Theuring F, Krüger G
Pathol Res Pract 1994 Apr;190(4):311-32. doi: 10.1016/S0344-0338(11)80404-8. PMID: 8078801

Recent systematic reviews

Malignant peripheral nerve sheath tumors of the eighth cranial nerve arising without prior irradiation.
Carlson ML, Jacob JT, Habermann EB, Glasgow AE, Raghunathan A, Link MJ
J Neurosurg 2016 Nov;125(5):1120-1129. Epub 2016 Jan 8 doi: 10.3171/2015.7.JNS151056. PMID: 26745487

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