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Central Nervous System Undifferentiated Pleomorphic Sarcoma

MedGen UID:: 1380462
•Concept ID:: C4329641
•: Neoplastic Process

Definition

A rare undifferentiated pleomorphic sarcoma (formerly known as malignant fibrous histiocytoma) involving the central nervous system. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVCentral Nervous System Undifferentiated Pleomorphic Sarcoma

Disorder of the central nervous system
- Neoplasm of the central nervous system
  - Primary Central Nervous System Neoplasm
    - Central nervous system mesenchymal non-meningothelial tumor
      - Malignant central nervous system mesenchymal, non-meningothelial neoplasm
        Central nervous system sarcoma
        Central Nervous System Undifferentiated Pleomorphic Sarcoma

Recent clinical studies

Etiology

Potential prognostic determinants for FET::CREB fusion-positive intracranial mesenchymal tumor.

Mezzacappa FM, Smith FK, Zhang W, Gard A, Cabuk FK, Gonzalez-Gomez I, Monforte HL, Liang J, Singh O, Quezado MM, Aldape KD, Gokden M, Bridge JA, Chen J
Acta Neuropathol Commun 2024 Jan 30;12(1):17. doi: 10.1186/s40478-024-01721-2. PMID: 38291529 Free PMC Article

An integrative histopathological and epigenetic characterization of primary intracranial mesenchymal tumors, FET:CREB-fused broadening the spectrum of tumor entities in comparison with their soft tissue counterparts.

Tauziède-Espariat A, Sievers P, Larousserie F, Benzakoun J, Guillemot D, Pierron G, Duchesne M, Uro-Coste E, Roux A, Vasiljevic A, Fenouil T, Meyronet D, Mokhtari K, Polivka M, Rousseau A, Bost-Bezeaud F, Akoury S, Pallud J, Benevello C, Hasty L, Gareton A, Lechapt E, Chrétien F, Blauwblomme T, Beccaria K, Puget S, Sahm F, Varlet P; RENOCLIP-LOC
Brain Pathol 2022 Jan;32(1):e13010. Epub 2021 Jul 27 doi: 10.1111/bpa.13010. PMID: 34314078 Free PMC Article

Metastatic undifferentiated pleomorphic sarcoma causing intraoperative stroke.

Spaulding R 4th, Koumoundouros T, Parker JC Jr
Ann Clin Lab Sci 2013 Spring;43(2):172-5. PMID: 23694792

Cerebellar malignant fibrous histiocytoma: case report and literature review.

Hamlat A, Adn M, Caulet-Maugendre S, Guegan Y
Neurosurgery 2004 Mar;54(3):745-51;discussion 751-2. doi: 10.1227/01.neu.0000108982.26949.f7. PMID: 15028153

Primary sarcomas of the brain and spinal cord: a study of 18 cases.

Oliveira AM, Scheithauer BW, Salomao DR, Parisi JE, Burger PC, Nascimento AG
Am J Surg Pathol 2002 Aug;26(8):1056-63. doi: 10.1097/00000478-200208000-00011. PMID: 12170093

See all (9)

Diagnosis

Meningioma and Other Meningeal Tumors.

Bailo M, Gagliardi F, Boari N, Spina A, Piloni M, Castellano A, Mortini P
Adv Exp Med Biol 2023;1405:73-97. doi: 10.1007/978-3-031-23705-8_3. PMID: 37452935

Intracranial Angiomatoid Fibrous Histiocytoma: Case Report and Literature Review.

Alshareef MA, Almadidy Z, Baker T, Perry A, Welsh CT, Vandergrift WA 3rd
World Neurosurg 2016 Dec;96:403-409. Epub 2016 Sep 22 doi: 10.1016/j.wneu.2016.09.059. PMID: 27667574

Metastatic undifferentiated pleomorphic sarcoma causing intraoperative stroke.

Spaulding R 4th, Koumoundouros T, Parker JC Jr
Ann Clin Lab Sci 2013 Spring;43(2):172-5. PMID: 23694792

Cerebellar malignant fibrous histiocytoma.

Gelincik I
Indian J Pathol Microbiol 2012 Jul-Sep;55(3):402-5. doi: 10.4103/0377-4929.101759. PMID: 23032846

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 (40)

Therapy

Clinical and radiological characteristics of methotrexate-induced acute encephalopathy in pediatric patients with cancer.

Inaba H, Khan RB, Laningham FH, Crews KR, Pui CH, Daw NC
Ann Oncol 2008 Jan;19(1):178-84. Epub 2007 Oct 17 doi: 10.1093/annonc/mdm466. PMID: 17947226

Second neoplasms in pediatric patients with primary central nervous system tumors: the St. Jude Children's Research Hospital experience.

Broniscer A, Ke W, Fuller CE, Wu J, Gajjar A, Kun LE
Cancer 2004 May 15;100(10):2246-52. doi: 10.1002/cncr.20253. PMID: 15139071

Malignant fibrous histiocytoma of the brain in a six-year-old girl.

Biegel JA, Perilongo G, Rorke LB, Parmiter AH, Emanuel BS
Genes Chromosomes Cancer 1992 Jun;4(4):309-13. doi: 10.1002/gcc.2870040406. PMID: 1377937

Malignant fibrous histiocytoma of soft tissue in childhood.

Raney RB Jr, Allen A, O'Neill J, Handler SD, Uri A, Littman P
Cancer 1986 Jun 1;57(11):2198-201. doi: 10.1002/1097-0142(19860601)57:11<2198::aid-cncr2820571120>3.0.co;2-x. PMID: 3008978

Malignant fibrous histiocytoma complicating mitral valve replacement.

Holtzman E, Schiby G, Segal P, Priel I
J Am Coll Cardiol 1986 Apr;7(4):956-60. doi: 10.1016/s0735-1097(86)80365-5. PMID: 3007598

See all (7)

Prognosis

Potential prognostic determinants for FET::CREB fusion-positive intracranial mesenchymal tumor.

Meningioma and Other Meningeal Tumors.

Bailo M, Gagliardi F, Boari N, Spina A, Piloni M, Castellano A, Mortini P
Adv Exp Med Biol 2023;1405:73-97. doi: 10.1007/978-3-031-23705-8_3. PMID: 37452935

Myxofibrosarcoma in Head and Neck: Case Report of Unusually Aggressive Presentation.

Quimby A, Estelle A, Gopinath A, Fernandes R
J Oral Maxillofac Surg 2017 Dec;75(12):2709.e1-2709.e12. Epub 2017 Aug 16 doi: 10.1016/j.joms.2017.08.015. PMID: 28893544

Metastatic undifferentiated pleomorphic sarcoma causing intraoperative stroke.

Spaulding R 4th, Koumoundouros T, Parker JC Jr
Ann Clin Lab Sci 2013 Spring;43(2):172-5. PMID: 23694792

Primary sarcomas of the brain and spinal cord: a study of 18 cases.

Oliveira AM, Scheithauer BW, Salomao DR, Parisi JE, Burger PC, Nascimento AG
Am J Surg Pathol 2002 Aug;26(8):1056-63. doi: 10.1097/00000478-200208000-00011. PMID: 12170093

See all (18)

Clinical prediction guides

An extracranial CNS presentation of the emerging "intracranial" mesenchymal tumor, FET: CREB-fusion positive.

Tauziède-Espariat A, Pierron G, Guillemot D, Benevello C, Pallud J, Benzakoun J, Hasty L, Métais A, Chrétien F, Varlet P
Brain Tumor Pathol 2023 Jan;40(1):35-39. Epub 2022 Sep 15 doi: 10.1007/s10014-022-00443-4. PMID: 36107277

Brain parenchymal angiomatoid fibrous histiocytoma and spinal myxoid mesenchymal tumor with FET: CREB fusion, a spectrum of the same tumor type.

Kim NR, Kim SI, Park JW, Park CK, Chung CK, Choi SH, Yun H, Park SH
Neuropathology 2022 Aug;42(4):257-268. Epub 2022 Jun 22 doi: 10.1111/neup.12814. PMID: 35730186

Paraneoplastic opsoclonus-myoclonus syndrome associated with malignant fibrous histiocytoma: neuropathological findings.

Zámecník J, Cerný R, Bartos A, Jerábek J, Bojar M
Cesk Patol 2004 Apr;40(2):63-7. PMID: 15233019

Fas (Apo-1, CD95) receptor expression in childhood astrocytomas. Is it a marker of the major apoptotic pathway or a signaling receptor for immune escape of neoplastic cells?

Bodey B, Bodey B Jr, Siegel SE, Kaiser HE
In Vivo 1999 Jul-Aug;13(4):357-73. PMID: 10586378

Malignant fibrous histiocytoma of the meninges. Histological, ultrastructural, and immunocytochemical studies.

Kalyanaraman UP, Taraska JJ, Fierer JA, Elwood PW
J Neurosurg 1981 Dec;55(6):957-62. doi: 10.3171/jns.1981.55.6.0957. PMID: 6271934

See all (7)