U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Malignant central nervous system mesenchymal, non-meningothelial neoplasm

MedGen UID:
233147
•Concept ID:
C1334571
•
Neoplastic Process
Synonyms: central nervous system mesenchymal non-meningothelial tumor, malignant; malignant central nervous system mesenchymal non-meningothelial tumor; malignant central nervous system mesenchymal non-meningothelial tumour; Malignant Central Nervous System Mesenchymal, Non-Meningothelial Neoplasm; malignant central nervous system mesenchymal, non-meningothelial neoplasm; Malignant Central Nervous System Mesenchymal, Non-Meningothelial Tumor; malignant central nervous system mesenchymal, non-meningothelial tumor; malignant central nervous system mesenchymal, non-meningothelial tumour; Malignant Central Nervous System Soft Tissue Neoplasm; malignant central nervous system soft tissue neoplasm; Malignant Central Nervous System Soft Tissue Tumor; malignant central nervous system soft tissue tumor; malignant central nervous system soft tissue tumour; Malignant CNS Soft Tissue Neoplasm; malignant CNS soft tissue neoplasm; Malignant CNS Soft Tissue Tumor; malignant CNS soft tissue tumor; malignant CNS soft tissue tumour; Malignant Soft Tissue Neoplasm of Central Nervous System; malignant soft tissue neoplasm of central nervous system; Malignant Soft Tissue Neoplasm of CNS; malignant soft tissue neoplasm of CNS; Malignant Soft Tissue Neoplasm of the Central Nervous System; malignant soft tissue neoplasm of the central nervous system; Malignant Soft Tissue Neoplasm of the CNS; malignant soft tissue neoplasm of the CNS; Malignant Soft Tissue Tumor of Central Nervous System; malignant soft tissue tumor of central nervous system; Malignant Soft Tissue Tumor of CNS; malignant soft tissue tumor of CNS; Malignant Soft Tissue Tumor of the Central Nervous System; malignant soft tissue tumor of the central nervous system; Malignant Soft Tissue Tumor of the CNS; malignant soft tissue tumor of the CNS; malignant soft tissue tumour of central nervous system; malignant soft tissue tumour of CNS; malignant soft tissue tumour of the central nervous system; malignant soft tissue tumour of the CNS; Soft Tissue Cancer of Central Nervous System; soft tissue cancer of central nervous system; Soft Tissue Cancer of the Central Nervous System; soft tissue cancer of the central nervous system
 
Monarch Initiative: MONDO:0037740

Definition

A metastasizing mesenchymal, non-meningothelial neoplasm that arises from the central nervous system. [from NCI]

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: 37312212Free 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: 32997853Free 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

Diagnosis

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: 37312212Free PMC Article
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: 36737790Free 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: 35266242Free 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: 32997853Free PMC Article
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

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: 35266242Free 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: 32997853Free PMC Article
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

Clinical prediction guides

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: 37312212Free PMC Article
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: 36737790Free 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: 32997853Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Consumer resources

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...