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Central nervous system sarcoma

MedGen UID:
272444
•Concept ID:
C1332892
•
Neoplastic Process
Synonyms: Central Nervous System Sarcoma; central nervous system sarcoma; CNS Sarcoma; CNS sarcoma; Sarcoma of Central Nervous System; sarcoma of central nervous system; Sarcoma of CNS; sarcoma of CNS; Sarcoma of the Central Nervous System; sarcoma of the central nervous system; Sarcoma of the CNS; sarcoma of the CNS
 
Monarch Initiative: MONDO:0002217

Definition

A sarcoma that arises from the central nervous system. [from NCI]

Professional guidelines

PubMed

Proton Therapy for Spinal Tumors: A Consensus Statement From the Particle Therapy Cooperative Group.
Chhabra AM, Snider JW, Kole AJ, Stock M, Holtzman AL, Press R, Wang CJ, Li H, Lin H, Shi C, McDonald M, Soike M, Zhou J, Sabouri P, Mossahebi S, Colaco R, Albertini F, Simone CB II,
Int J Radiat Oncol Biol Phys 2024 Nov 15;120(4):1135-1148. Epub 2024 Aug 23 doi: 10.1016/j.ijrobp.2024.04.007. PMID: 39181272
Primary central nervous system sarcoma with DICER1 mutation-treatment results of a novel molecular entity in pediatric Peruvian patients.
Diaz Coronado RY, Mynarek M, Koelsche C, Mora Alferez P, Casavilca Zambrano S, Wachtel Aptowitzer A, Sahm F, von Deimling A, Schüller U, Spohn M, Sturm D, Pfister SM, Morales La Madrid A, Sernaque Quintana R, Sarria Bardales G, Negreiros Chinchihuara T, Ojeda Medina L, Garcia-Corrochano Medina P, Campos Sanchez DA, Ponce Farfan J, Rutkowski S, Garcia Leon JL
Cancer 2022 Feb 15;128(4):697-707. Epub 2021 Oct 21 doi: 10.1002/cncr.33977. PMID: 34674226
Management of unusual presentations of acute leukemia.
Baer MR
Hematol Oncol Clin North Am 1993 Feb;7(1):275-92. PMID: 8449862

Recent clinical studies

Etiology

Proton Therapy for Spinal Tumors: A Consensus Statement From the Particle Therapy Cooperative Group.
Chhabra AM, Snider JW, Kole AJ, Stock M, Holtzman AL, Press R, Wang CJ, Li H, Lin H, Shi C, McDonald M, Soike M, Zhou J, Sabouri P, Mossahebi S, Colaco R, Albertini F, Simone CB II,
Int J Radiat Oncol Biol Phys 2024 Nov 15;120(4):1135-1148. Epub 2024 Aug 23 doi: 10.1016/j.ijrobp.2024.04.007. PMID: 39181272
Radiation-Induced Sarcomas of the Central Nervous System: A Systematic Review.
Yamanaka R, Hayano A
World Neurosurg 2017 Feb;98:818-828.e7. Epub 2016 Nov 9 doi: 10.1016/j.wneu.2016.11.008. PMID: 27838424

Diagnosis

DICER1-associated central nervous system sarcoma with neural lineage differentiation: a case report.
Yao K, Duan Z, Feng J, Yan C, Qi X
Diagn Pathol 2022 Sep 24;17(1):72. doi: 10.1186/s13000-022-01252-1. PMID: 36153506Free PMC Article
Central nervous system sarcoma with ATXN1::DUX4 fusion expands the concept of CIC-rearranged sarcoma.
Satomi K, Ohno M, Kubo T, Honda-Kitahara M, Matsushita Y, Ichimura K, Narita Y, Ichikawa H, Yoshida A
Genes Chromosomes Cancer 2022 Nov;61(11):683-688. Epub 2022 Jul 13 doi: 10.1002/gcc.23080. PMID: 35715887
Primary central nervous system sarcoma with DICER1 mutation-treatment results of a novel molecular entity in pediatric Peruvian patients.
Diaz Coronado RY, Mynarek M, Koelsche C, Mora Alferez P, Casavilca Zambrano S, Wachtel Aptowitzer A, Sahm F, von Deimling A, Schüller U, Spohn M, Sturm D, Pfister SM, Morales La Madrid A, Sernaque Quintana R, Sarria Bardales G, Negreiros Chinchihuara T, Ojeda Medina L, Garcia-Corrochano Medina P, Campos Sanchez DA, Ponce Farfan J, Rutkowski S, Garcia Leon JL
Cancer 2022 Feb 15;128(4):697-707. Epub 2021 Oct 21 doi: 10.1002/cncr.33977. PMID: 34674226
DICER1-associated central nervous system sarcoma in children: comprehensive clinicopathologic and genetic analysis of a newly described rare tumor.
Kamihara J, Paulson V, Breen MA, Laetsch TW, Rakheja D, Shulman DS, Schoettler ML, Clinton CM, Ward A, Reidy D, Pinches RS, Weiser DA, Mullen EA, Schienda J, Meyers PA, DuBois SG, Nowak JA, Foulkes WD, Schultz KAP, Janeway KA, Vargas SO, Church AJ
Mod Pathol 2020 Oct;33(10):1910-1921. Epub 2020 Apr 14 doi: 10.1038/s41379-020-0516-1. PMID: 32291395

Therapy

Radiation-Induced Sarcomas of the Central Nervous System: A Systematic Review.
Yamanaka R, Hayano A
World Neurosurg 2017 Feb;98:818-828.e7. Epub 2016 Nov 9 doi: 10.1016/j.wneu.2016.11.008. PMID: 27838424

Prognosis

DICER1-associated central nervous system sarcoma with neural lineage differentiation: a case report.
Yao K, Duan Z, Feng J, Yan C, Qi X
Diagn Pathol 2022 Sep 24;17(1):72. doi: 10.1186/s13000-022-01252-1. PMID: 36153506Free PMC Article
Primary central nervous system sarcoma with DICER1 mutation-treatment results of a novel molecular entity in pediatric Peruvian patients.
Diaz Coronado RY, Mynarek M, Koelsche C, Mora Alferez P, Casavilca Zambrano S, Wachtel Aptowitzer A, Sahm F, von Deimling A, Schüller U, Spohn M, Sturm D, Pfister SM, Morales La Madrid A, Sernaque Quintana R, Sarria Bardales G, Negreiros Chinchihuara T, Ojeda Medina L, Garcia-Corrochano Medina P, Campos Sanchez DA, Ponce Farfan J, Rutkowski S, Garcia Leon JL
Cancer 2022 Feb 15;128(4):697-707. Epub 2021 Oct 21 doi: 10.1002/cncr.33977. PMID: 34674226
DICER1 tumor predisposition syndrome: an evolving story initiated with the pleuropulmonary blastoma.
González IA, Stewart DR, Schultz KAP, Field AP, Hill DA, Dehner LP
Mod Pathol 2022 Jan;35(1):4-22. Epub 2021 Oct 1 doi: 10.1038/s41379-021-00905-8. PMID: 34599283Free PMC Article

Clinical prediction guides

Primary central nervous system sarcoma with DICER1 mutation-treatment results of a novel molecular entity in pediatric Peruvian patients.
Diaz Coronado RY, Mynarek M, Koelsche C, Mora Alferez P, Casavilca Zambrano S, Wachtel Aptowitzer A, Sahm F, von Deimling A, Schüller U, Spohn M, Sturm D, Pfister SM, Morales La Madrid A, Sernaque Quintana R, Sarria Bardales G, Negreiros Chinchihuara T, Ojeda Medina L, Garcia-Corrochano Medina P, Campos Sanchez DA, Ponce Farfan J, Rutkowski S, Garcia Leon JL
Cancer 2022 Feb 15;128(4):697-707. Epub 2021 Oct 21 doi: 10.1002/cncr.33977. PMID: 34674226

Recent systematic reviews

Radiation-Induced Sarcomas of the Central Nervous System: A Systematic Review.
Yamanaka R, Hayano A
World Neurosurg 2017 Feb;98:818-828.e7. Epub 2016 Nov 9 doi: 10.1016/j.wneu.2016.11.008. PMID: 27838424

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