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Central Nervous System Solitary Fibrous Tumor

MedGen UID:: 1385242
•Concept ID:: C4329640
•: Neoplastic Process

Synonyms:

Central Nervous System Solitary Fibrous Tumor/Hemangiopericytoma; CNS Solitary Fibrous Tumor; CNS Solitary Fibrous Tumor/Hemangiopericytoma

Definition

A mesenchymal, non-meningothelial neoplasm arising from the central nervous system. It is characterized by a collagenous and low cellularity spindle cell and/or hemangiopericytomatous histopathological pattern, recurrent intrachromosomal rearrangement on chromosome 12q that results in the fusion of the NAB2 and STAT6 genes, high recurrence rates, and long-term risk of systemic metastasis. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVCentral Nervous System Solitary Fibrous Tumor

Disorder by Site
- Disorder of nervous system
  - Disorder of the central nervous system
    - Neoplasm of the central nervous system
      - Primary Central Nervous System Neoplasm
        Central nervous system mesenchymal non-meningothelial tumor
        Central Nervous System Solitary Fibrous Tumor
        Central Nervous System Solitary Fibrous Tumor, Grade 1
        Central Nervous System Solitary Fibrous Tumor, Grade 2
        Central Nervous System Solitary Fibrous Tumor, Grade 3
        Adult intracranial malignant hemangiopericytoma
        Meningeal Central Nervous System Solitary Fibrous Tumor, Grade 3
        Meninges hemangiopericytoma
        Sellar Solitary Fibrous Tumor
        Optic Nerve Sheath Solitary Fibrous Tumor

Professional guidelines

PubMed

Efficacy and toxicity of photon, proton, and carbon ion radiotherapy in the treatment of intracranial solitary fibrous tumor/hemangiopericytoma.

Ton M, Deng M, Meixner E, Eichkorn T, Krämer A, Seidensaal K, Hörner-Rieber J, Lischalk J, Herfarth K, Debus J, König L
Radiat Oncol 2024 Mar 29;19(1):42. doi: 10.1186/s13014-024-02434-5. PMID: 38553768 Free PMC Article

See all (1)

Recent clinical studies

Etiology

Efficacy and toxicity of photon, proton, and carbon ion radiotherapy in the treatment of intracranial solitary fibrous tumor/hemangiopericytoma.

Clinical outcomes in central nervous system solitary-fibrous tumor/hemangiopericytoma: a STROBE-compliant single-center analysis.

Yu Y, Hu Y, Lv L, Chen C, Yin S, Jiang S, Zhou P
World J Surg Oncol 2022 May 10;20(1):149. doi: 10.1186/s12957-022-02619-w. PMID: 35538540 Free PMC Article

See all (2)

Diagnosis

Efficacy and toxicity of photon, proton, and carbon ion radiotherapy in the treatment of intracranial solitary fibrous tumor/hemangiopericytoma.

Rare hypervascular pancreatic tumors diagnosed as asynchronous metastases of central nervous system solitary fibrous tumor/hemangiopericytoma.

Mizukoshi K, Yamauchi Y, Kitaura M, Fukuda A, Seno H
Clin J Gastroenterol 2022 Feb;15(1):221-227. Epub 2021 Oct 15 doi: 10.1007/s12328-021-01533-0. PMID: 34652718

The Importance of STAT6 in a Schwannoma-like Grade III Solitary Fibrous Tumor/Hemangiopericytoma Located in the Cerebellopontine Angle and Meckel's Cave.

Oishi M, Fujisawa H, Tsuchiya K, Nakajima Y
World Neurosurg 2020 Sep;141:500-506.e1. Epub 2020 Jun 8 doi: 10.1016/j.wneu.2020.05.262. PMID: 32526357

Central Nervous System Solitary Fibrous Tumor/Hemangiopericytoma Penetrating the Skull Bone: An Unusual Presentation.

Choi J, Kim J, Cho I, Shin J
World Neurosurg 2020 Sep;141:8-13. Epub 2020 May 28 doi: 10.1016/j.wneu.2020.05.189. PMID: 32474097

See all (4)

Therapy

Efficacy and toxicity of photon, proton, and carbon ion radiotherapy in the treatment of intracranial solitary fibrous tumor/hemangiopericytoma.

See all (1)

Prognosis

Clinical outcomes in central nervous system solitary-fibrous tumor/hemangiopericytoma: a STROBE-compliant single-center analysis.

Yu Y, Hu Y, Lv L, Chen C, Yin S, Jiang S, Zhou P
World J Surg Oncol 2022 May 10;20(1):149. doi: 10.1186/s12957-022-02619-w. PMID: 35538540 Free PMC Article

See all (1)

Clinical prediction guides

Rare hypervascular pancreatic tumors diagnosed as asynchronous metastases of central nervous system solitary fibrous tumor/hemangiopericytoma.

Mizukoshi K, Yamauchi Y, Kitaura M, Fukuda A, Seno H
Clin J Gastroenterol 2022 Feb;15(1):221-227. Epub 2021 Oct 15 doi: 10.1007/s12328-021-01533-0. PMID: 34652718

The Importance of STAT6 in a Schwannoma-like Grade III Solitary Fibrous Tumor/Hemangiopericytoma Located in the Cerebellopontine Angle and Meckel's Cave.

Oishi M, Fujisawa H, Tsuchiya K, Nakajima Y
World Neurosurg 2020 Sep;141:500-506.e1. Epub 2020 Jun 8 doi: 10.1016/j.wneu.2020.05.262. PMID: 32526357

See all (2)

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Central Nervous System Solitary Fibrous Tumor

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Supplemental Content

Table of contents

Practice guidelines

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