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Hybrid Nerve Sheath Tumor

MedGen UID:
825278
•Concept ID:
C3839433
•
Neoplastic Process
Synonyms: Hybrid Nerve Sheath Neoplasm; Hybrid nerve sheath tumor; Hybrid Neurofibroma-Perineurioma; Hybrid Neurofibroma-Schwannoma; Hybrid Schwannoma-Perineurioma
SNOMED CT: Hybrid nerve sheath tumor (703710008)

Definition

A benign nerve sheath tumor characterized by the combination of histologic features seen in schwannomas, neurofibromas, and perineuriomas. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHybrid Nerve Sheath Tumor

Professional guidelines

PubMed

Neurofibroma-like Desmoplastic Melanoma: A Series of Five Cases Exploring the Role of Molecular Testing as a Diagnostic Adjunct and Highlighting the Differential Diagnosis With Diffuse-type Neurofibroma.
Baraban EG, Gru A, Guo R, Elias R, Pallavajjala A, Dudley JC, Gross JM
Am J Surg Pathol 2025 Jan 1;49(1):1-10. Epub 2024 Oct 31 doi: 10.1097/PAS.0000000000002327. PMID: 39484711

Recent clinical studies

Diagnosis

Neurofibroma-like Desmoplastic Melanoma: A Series of Five Cases Exploring the Role of Molecular Testing as a Diagnostic Adjunct and Highlighting the Differential Diagnosis With Diffuse-type Neurofibroma.
Baraban EG, Gru A, Guo R, Elias R, Pallavajjala A, Dudley JC, Gross JM
Am J Surg Pathol 2025 Jan 1;49(1):1-10. Epub 2024 Oct 31 doi: 10.1097/PAS.0000000000002327. PMID: 39484711
EMA-Positive Superficial ALK-Rearranged Myxoid Spindle Cell Neoplasm Masquerading as Perineurioma/Hybrid Nerve Sheath Tumor.
Hegazy S, Naous R
Am J Dermatopathol 2024 Sep 1;46(9):597-600. Epub 2024 May 7 doi: 10.1097/DAD.0000000000002734. PMID: 39150182
Intramuscular Hybrid Nerve Sheath Tumor of the Thigh: Case Report and Literature Review.
Chijiiwa Y, Sano J, Okamura K, Nishio J
In Vivo 2024 Mar-Apr;38(2):971-974. doi: 10.21873/invivo.13529. PMID: 38418131Free PMC Article
Benign peripheral nerve sheath tumors: an interdisciplinary diagnostic and therapeutic challenge.
Uerschels AK, Dengler NF, Chihi M, Lenkeit A, Dinger TF, Jabbarli R, Sure U, Hagenacker T, Wrede KH, Gembruch O
Neurosurg Rev 2023 Aug 18;46(1):205. doi: 10.1007/s10143-023-02107-z. PMID: 37594583Free PMC Article
Primary intraosseous hybrid nerve sheath tumor of femur: a hitherto undescribed occurrence in bone with secondary aneurysmal bone cyst formation resulting in pathological fracture.
Chow LT
Pathol Res Pract 2015 May;211(5):409-14. Epub 2014 Nov 14 doi: 10.1016/j.prp.2014.10.014. PMID: 25466465

Prognosis

Neurofibroma-like Desmoplastic Melanoma: A Series of Five Cases Exploring the Role of Molecular Testing as a Diagnostic Adjunct and Highlighting the Differential Diagnosis With Diffuse-type Neurofibroma.
Baraban EG, Gru A, Guo R, Elias R, Pallavajjala A, Dudley JC, Gross JM
Am J Surg Pathol 2025 Jan 1;49(1):1-10. Epub 2024 Oct 31 doi: 10.1097/PAS.0000000000002327. PMID: 39484711
Trigeminal hybrid nerve sheath tumor - a case report and literature review.
Goyal-Honavar A, Gupta A, Chacko G, Chacko AG
Br J Neurosurg 2023 Oct;37(5):1326-1329. Epub 2021 Jun 20 doi: 10.1080/02688697.2020.1867061. PMID: 34151661

Clinical prediction guides

Neurofibroma-like Desmoplastic Melanoma: A Series of Five Cases Exploring the Role of Molecular Testing as a Diagnostic Adjunct and Highlighting the Differential Diagnosis With Diffuse-type Neurofibroma.
Baraban EG, Gru A, Guo R, Elias R, Pallavajjala A, Dudley JC, Gross JM
Am J Surg Pathol 2025 Jan 1;49(1):1-10. Epub 2024 Oct 31 doi: 10.1097/PAS.0000000000002327. PMID: 39484711

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