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Malignant fibrous histiocytoma

MedGen UID:
87248
•Concept ID:
C0334463
•
Neoplastic Process
Synonyms: Malignant fibrohistiocytic tumors; Undifferentiated pleomorphic sarcoma
SNOMED CT: Malignant fibrous histiocytoma (34360000); Malignant fibroxanthoma (34360000); Malignant fibrous histiocytoma (443439001); Undifferentiated high grade pleomorphic sarcoma (34360000); Fibrous histiocytoma, malignant (34360000); Fibroxanthoma, malignant (34360000)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0002142
Orphanet: ORPHA2023

Definition

An aggressive sarcoma of soft tissues or bone that can arise from any part of the body, clinically presenting as swelling, mass, pain, pathological fracture and occasional systemic features and is characterized by high local recurrence and significant metastasis. [from ORDO]

Professional guidelines

PubMed

Management of Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma.
Crago AM, Cardona K, Koseła-Paterczyk H, Rutkowski P
Surg Oncol Clin N Am 2022 Jul;31(3):419-430. doi: 10.1016/j.soc.2022.03.006. PMID: 35715142Free PMC Article
Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma: Updates on Classification and Management.
Soleymani T, Aasi SZ, Novoa R, Hollmig ST
Dermatol Clin 2019 Jul;37(3):253-259. Epub 2019 Apr 16 doi: 10.1016/j.det.2019.02.001. PMID: 31084719
Clinicopathologic features, diagnosis, and treatment of malignant fibrous histiocytoma of bone.
Papagelopoulos PJ, Galanis EC, Sim FH, Unni KK
Orthopedics 2000 Jan;23(1):59-65; quiz 66-7. doi: 10.3928/0147-7447-20000101-18. PMID: 10642003

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

Recent clinical studies

Etiology

Management of Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma.
Crago AM, Cardona K, Koseła-Paterczyk H, Rutkowski P
Surg Oncol Clin N Am 2022 Jul;31(3):419-430. doi: 10.1016/j.soc.2022.03.006. PMID: 35715142Free PMC Article
Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma: Updates on Classification and Management.
Soleymani T, Aasi SZ, Novoa R, Hollmig ST
Dermatol Clin 2019 Jul;37(3):253-259. Epub 2019 Apr 16 doi: 10.1016/j.det.2019.02.001. PMID: 31084719
Infarct-associated bone sarcomas.
Domson GF, Shahlaee A, Reith JD, Bush CH, Gibbs CP
Clin Orthop Relat Res 2009 Jul;467(7):1820-5. Epub 2009 Feb 20 doi: 10.1007/s11999-009-0744-7. PMID: 19229663Free PMC Article
Sarcoma.
Skubitz KM, D'Adamo DR
Mayo Clin Proc 2007 Nov;82(11):1409-32. doi: 10.4065/82.11.1409. PMID: 17976362
Radiation-induced sarcoma.
Patel SR
Curr Treat Options Oncol 2000 Aug;1(3):258-61. doi: 10.1007/s11864-000-0037-6. PMID: 12057168

Diagnosis

Pleomorphic Dermal Sarcoma.
Saleh JS, Whittington CP, Bresler SC, Patel RM
Surg Pathol Clin 2024 Mar;17(1):153-158. Epub 2023 Aug 5 doi: 10.1016/j.path.2023.06.007. PMID: 38278604
Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma: Updates on Classification and Management.
Soleymani T, Aasi SZ, Novoa R, Hollmig ST
Dermatol Clin 2019 Jul;37(3):253-259. Epub 2019 Apr 16 doi: 10.1016/j.det.2019.02.001. PMID: 31084719
Soft Tissue Tumors Rarely Presenting Primary in Bone; Diagnostic Pitfalls.
Sbaraglia M, Righi A, Gambarotti M, Vanel D, Picci P, Dei Tos AP
Surg Pathol Clin 2017 Sep;10(3):705-730. Epub 2017 Jun 29 doi: 10.1016/j.path.2017.04.013. PMID: 28797510
Atypical fibroxanthoma/malignant fibrous histiocytoma.
Marcet S
Dermatol Ther 2008 Nov-Dec;21(6):424-7. doi: 10.1111/j.1529-8019.2008.00241.x. PMID: 19076618
Myofibrosarcoma.
Fisher C
Virchows Arch 2004 Sep;445(3):215-23. Epub 2004 Jun 2 doi: 10.1007/s00428-004-1038-9. PMID: 15173943

Therapy

Rare skin cancers.
Kanitakis J
Cancer Treat Res 2009;146:323-8. doi: 10.1007/978-0-387-78574-5_26. PMID: 19415213
Sarcoma.
Skubitz KM, D'Adamo DR
Mayo Clin Proc 2007 Nov;82(11):1409-32. doi: 10.4065/82.11.1409. PMID: 17976362
Radiation-induced sarcoma.
Patel SR
Curr Treat Options Oncol 2000 Aug;1(3):258-61. doi: 10.1007/s11864-000-0037-6. PMID: 12057168
Malignant fibrous histiocytoma.
Restrepo JP, Handler SD, Saull SC, Raney RB Jr
Otolaryngol Head Neck Surg 1987 Apr;96(4):362-5. doi: 10.1177/019459988709600409. PMID: 3035462
Radiation-induced soft-tissue sarcoma.
Davidson T, Westbury G, Harmer CL
Br J Surg 1986 Apr;73(4):308-9. doi: 10.1002/bjs.1800730420. PMID: 3697664

Prognosis

Intracranial malignant fibrous histiocytoma.
Amezcua-Gutiérrez MA, Zamora Gómez SE
Med Clin (Barc) 2018 Nov 21;151(10):e61. Epub 2018 Apr 21 doi: 10.1016/j.medcli.2018.02.021. PMID: 29685317
Primary malignant fibrous histiocytoma of the breast: a case report and review of 38 chinese cases.
Hu Y, Li X, Deng Y, Zhou M, Zhang S
Clin Breast Cancer 2012 Oct;12(5):382-5. Epub 2012 Apr 21 doi: 10.1016/j.clbc.2012.03.011. PMID: 22521730
Dupuytren's contracture and sarcoma.
Wilbrand S, Ekbom A, Gerdin B
J Hand Surg Br 2002 Feb;27(1):50-2. doi: 10.1054/jhsb.2001.0652. PMID: 11895346
Dedifferentiated liposarcoma.
Nascimento AG
Semin Diagn Pathol 2001 Nov;18(4):263-6. PMID: 11757866
Radiation-induced sarcoma.
Patel SR
Curr Treat Options Oncol 2000 Aug;1(3):258-61. doi: 10.1007/s11864-000-0037-6. PMID: 12057168

Clinical prediction guides

Management of Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma.
Crago AM, Cardona K, Koseła-Paterczyk H, Rutkowski P
Surg Oncol Clin N Am 2022 Jul;31(3):419-430. doi: 10.1016/j.soc.2022.03.006. PMID: 35715142Free PMC Article
Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma: Updates on Classification and Management.
Soleymani T, Aasi SZ, Novoa R, Hollmig ST
Dermatol Clin 2019 Jul;37(3):253-259. Epub 2019 Apr 16 doi: 10.1016/j.det.2019.02.001. PMID: 31084719
An approach to pleomorphic sarcomas: can we subclassify, and does it matter?
Goldblum JR
Mod Pathol 2014 Jan;27 Suppl 1:S39-46. doi: 10.1038/modpathol.2013.174. PMID: 24384852
Dedifferentiated liposarcoma.
Nascimento AG
Semin Diagn Pathol 2001 Nov;18(4):263-6. PMID: 11757866
Comparative ultrastructure of soft-tissue myxoid tumors.
Vuzevski VD, van der Heul RO
Ultrastruct Pathol 1988 Jan-Feb;12(1):87-105. doi: 10.3109/01913128809048478. PMID: 3281341

Recent systematic reviews

Immunohistochemical Characteristics of Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma: A Systematic Review and Meta-Analysis.
Ørholt M, Abebe K, Aaberg F, Rasmussen LE, Daugaard S, Loya AC, Herly M, Vester-Glowinski PV
Am J Dermatopathol 2022 Dec 1;44(12):913-920. doi: 10.1097/DAD.0000000000002305. PMID: 36395448
Bone Infarcts and Tumorigenesis-Is There a Connection? A Mini-Mapping Review.
Konarski W, Poboży T, Hordowicz M, Śliwczyński A, Kotela I, Krakowiak J, Kotela A
Int J Environ Res Public Health 2022 Jul 29;19(15) doi: 10.3390/ijerph19159282. PMID: 35954639Free PMC Article
Surgical excision margins for fibrohistiocytic tumors, including atypical fibroxanthoma and undifferentiated pleomorphic sarcoma: A probability model based on a systematic review.
Jibbe A, Worley B, Miller CH, Alam M
J Am Acad Dermatol 2022 Oct;87(4):833-840. Epub 2021 Sep 26 doi: 10.1016/j.jaad.2021.09.036. PMID: 34587553
Oral Health and Molecular Aspects of Malignant Fibrous Histiocytoma Patients: A Systematic Review of the Literature.
Zhurakivska K, Troiano G, Montella M, Lo Muzio L, Fiorillo L, Cervino G, Cicciù M, D'Amico C, Rullo R, Laino G, Di Stasio D, Laino L
Int J Environ Res Public Health 2020 Feb 23;17(4) doi: 10.3390/ijerph17041426. PMID: 32102165Free PMC Article
Primary hepatic malignant fibrous histiocytoma: clinicopathologic characteristics and prognostic value of ezrin expression.
Li YR, Akbari E, Tretiakova MS, Hart J, Akbari M, Urbanski SJ, Gao ZH
Am J Surg Pathol 2008 Aug;32(8):1144-58. doi: 10.1097/PAS.0b013e3181659791. PMID: 18545143

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    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

    Reviews

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