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Central nervous system mesenchymal non-meningothelial tumor

MedGen UID:: 234104
•Concept ID:: C1332893
•: Neoplastic Process

Synonyms:	central nervous system mesenchymal non-meningothelial tumor; Central Nervous System Mesenchymal, Non-Meningothelial Neoplasm; central nervous system mesenchymal, non-meningothelial neoplasm; Central Nervous System Mesenchymal, Non-Meningothelial Tumor; central nervous system mesenchymal, non-meningothelial tumor; central nervous system mesenchymal, non-meningothelial tumour; Central Nervous System Soft Tissue Neoplasm; central nervous system soft tissue neoplasm; Central Nervous System Soft Tissue Tumor; central nervous system soft tissue tumor; central nervous system soft tissue tumour; CNS Soft Tissue Neoplasm; CNS soft tissue neoplasm; CNS Soft Tissue Tumor; CNS soft tissue tumor; CNS soft tissue tumour; Mesenchymal Non-Meningothelial Tumor of the Central Nervous System; mesenchymal non-meningothelial tumor of the central nervous system; mesenchymal non-meningothelial tumour of the central nervous system; Mesenchymal, Non-Meningothelial Tumor of Central Nervous System; mesenchymal, non-meningothelial tumor of central nervous system; Mesenchymal, Non-Meningothelial Tumor of CNS; mesenchymal, non-meningothelial tumor of CNS; Mesenchymal, Non-Meningothelial Tumor of the CNS; mesenchymal, non-meningothelial tumor of the CNS; mesenchymal, non-meningothelial tumour of central nervous system; mesenchymal, non-meningothelial tumour of CNS; mesenchymal, non-meningothelial tumour of the CNS; non-meningothelial mesenchymal tumor; non-meningothelial mesenchymal tumour; Soft Tissue Neoplasm of Central Nervous System; soft tissue neoplasm of central nervous system; Soft Tissue Neoplasm of CNS; soft tissue neoplasm of CNS; Soft Tissue Neoplasm of the Central Nervous System; soft tissue neoplasm of the central nervous system; Soft Tissue Neoplasm of the CNS; soft tissue neoplasm of the CNS; Soft Tissue Tumor of Central Nervous System; soft tissue tumor of central nervous system; Soft Tissue Tumor of CNS; soft tissue tumor of CNS; Soft Tissue Tumor of the Central Nervous System; soft tissue tumor of the central nervous system; Soft Tissue Tumor of the CNS; soft tissue tumor of the CNS; soft tissue tumour of central nervous system; soft tissue tumour of CNS; soft tissue tumour of the central nervous system; soft tissue tumour of the CNS

Monarch Initiative:	MONDO:0003244

Definition

A benign or malignant mesenchymal neoplasm originating in the central nervous system or the meninges and showing fibrous, fibrohistiocytic, adipose, myoid, endothelial, chondroid or osseous, but not meningothelial differentiation. Depending on the histological features and clinical behavior of these neoplasms, their grade ranges from benign (WHO grade I) to highly malignant (WHO grade IV). (Adapted from WHO) [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVCentral nervous system mesenchymal non-meningothelial tumor

Disease
- 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
        Benign Central Nervous System Mesenchymal, Non-Meningothelial Neoplasm
        Central nervous system chondroma
        Central nervous system hemangioma
        Central Nervous System Cavernous Hemangioma
        Central nervous system leiomyoma
        Central nervous system lipoma
        Central Nervous System Angiolipoma
        Central nervous system hibernoma
        Intracranial Lipoma
        Spinal cord lipoma
        Central Nervous System Myofibroblastoma
        Central Nervous System Rhabdomyoma
        Intracranial Fibrous Histiocytoma
        Central Nervous System Desmoid Fibromatosis
        Central Nervous System Inflammatory Myofibroblastic 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
        Malignant central nervous system mesenchymal, non-meningothelial neoplasm
        Central Nervous System Epithelioid Hemangioendothelioma
        Central nervous system sarcoma
        Brain sarcoma
        Central nervous system angiosarcoma
        Central nervous system extraskeletal osteosarcoma
        Central nervous system fibrosarcoma
        Central Nervous System Kaposi Sarcoma
        Central nervous system leiomyosarcoma
        Central Nervous System Mesenchymal Chondrosarcoma
        Central nervous system rhabdomyosarcoma
        Central Nervous System Undifferentiated Pleomorphic Sarcoma
        Intracranial extraskeletal myxoid chondrosarcoma
        Intracranial liposarcoma
        Meningeal sarcoma
        Spinal cord sarcoma

Recent clinical studies

Etiology

Intracranial mesenchymal tumor with (novel) COX14::PTEN rearrangement.

d'Amati A, Gianno F, Scuccimarri L, Lastilla M, Messina R, Signorelli F, Zimatore DS, Barresi S, Miele E, Alaggio R, Rossi S, Maiorano E, Ingravallo G, Giangaspero F, Antonelli M
Acta Neuropathol Commun 2023 Jun 13;11(1):95. doi: 10.1186/s40478-023-01596-9. PMID: 37312212 Free PMC Article

The spectrum of rare central nervous system (CNS) tumors with EWSR1-non-ETS fusions: experience from three pediatric institutions with review of the literature.

Lopez-Nunez O, Cafferata B, Santi M, Ranganathan S, Pearce TM, Kulich SM, Bailey KM, Broniscer A, Rossi S, Zin A, Nasrallah MP, Li MM, Zhong Y, Miele E, Alaggio R, Surrey LF
Brain Pathol 2021 Jan;31(1):70-83. Epub 2020 Nov 6 doi: 10.1111/bpa.12900. PMID: 32997853 Free PMC Article

Intracranial hemangiopericytoma: study of 12 cases.

Alén JF, Lobato RD, Gómez PA, Boto GR, Lagares A, Ramos A, Ricoy JR
Acta Neurochir (Wien) 2001;143(6):575-86. doi: 10.1007/s007010170062. PMID: 11534674

See all (3)

Diagnosis

Intracranial mesenchymal tumor with (novel) COX14::PTEN rearrangement.

Mesenchymal non-meningothelial tumors of the central nervous system: a literature review and diagnostic update of novelties and emerging entities.

Tauziède-Espariat A, Hasty L, Métais A, Varlet P
Acta Neuropathol Commun 2023 Feb 3;11(1):22. doi: 10.1186/s40478-023-01522-z. PMID: 36737790 Free PMC Article

Embryonal and non-meningothelial mesenchymal tumors of the central nervous system - Advances in diagnosis and prognostication.

Meredith DM, Alexandrescu S
Brain Pathol 2022 Jul;32(4):e13059. Epub 2022 Mar 9 doi: 10.1111/bpa.13059. PMID: 35266242 Free PMC Article

The spectrum of rare central nervous system (CNS) tumors with EWSR1-non-ETS fusions: experience from three pediatric institutions with review of the literature.

Mesenchymal, non-meningothelial tumors of the central nervous system.

Jellinger K, Paulus W
Brain Pathol 1991 Jan;1(2):79-87. doi: 10.1111/j.1750-3639.1991.tb00643.x. PMID: 1669699

See all (8)

Prognosis

Embryonal and non-meningothelial mesenchymal tumors of the central nervous system - Advances in diagnosis and prognostication.

Meredith DM, Alexandrescu S
Brain Pathol 2022 Jul;32(4):e13059. Epub 2022 Mar 9 doi: 10.1111/bpa.13059. PMID: 35266242 Free PMC Article

The spectrum of rare central nervous system (CNS) tumors with EWSR1-non-ETS fusions: experience from three pediatric institutions with review of the literature.

Meningeal osteochondroma simulating meningioma with metaplastic change: a rare golf-ball-like lesion of non-meningothelial mesenchymal origin.

Majumdar K, Mandal S, Thakkar R, Saran RK, Srivastava AK
Brain Tumor Pathol 2014 Jan;31(1):62-7. Epub 2013 Mar 2 doi: 10.1007/s10014-013-0138-0. PMID: 23456087

Intracranial hemangiopericytoma: study of 12 cases.

Alén JF, Lobato RD, Gómez PA, Boto GR, Lagares A, Ramos A, Ricoy JR
Acta Neurochir (Wien) 2001;143(6):575-86. doi: 10.1007/s007010170062. PMID: 11534674

See all (4)

Clinical prediction guides

Intracranial mesenchymal tumor with (novel) COX14::PTEN rearrangement.

Mesenchymal non-meningothelial tumors of the central nervous system: a literature review and diagnostic update of novelties and emerging entities.

Tauziède-Espariat A, Hasty L, Métais A, Varlet P
Acta Neuropathol Commun 2023 Feb 3;11(1):22. doi: 10.1186/s40478-023-01522-z. PMID: 36737790 Free PMC Article

The spectrum of rare central nervous system (CNS) tumors with EWSR1-non-ETS fusions: experience from three pediatric institutions with review of the literature.

See all (3)

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Central nervous system mesenchymal non-meningothelial tumor

Definition

Term Hierarchy

Recent clinical studies

Etiology

Diagnosis

Prognosis

Clinical prediction guides

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Table of contents

Clinical resources

Consumer resources

Reviews

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