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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: 35715142
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
Primary mesenchymal liver tumors: radiological spectrum, differential diagnosis, and pathologic correlation.
Harman M, Nart D, Acar T, Elmas N
Abdom Imaging 2015 Jun;40(5):1316-30. doi: 10.1007/s00261-014-0268-9. PMID: 25311993

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: 35715142
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
Primary thoracic sarcomas.
Gladish GW, Sabloff BM, Munden RF, Truong MT, Erasmus JJ, Chasen MH
Radiographics 2002 May-Jun;22(3):621-37. doi: 10.1148/radiographics.22.3.g02ma17621. PMID: 12006691
Retroperitoneal sarcomas.
Eilber FC, Eilber KS, Eilber FR
Curr Treat Options Oncol 2000 Aug;1(3):274-8. doi: 10.1007/s11864-000-0040-y. PMID: 12057171
Extraskeletal osteosarcoma.
Chung EB, Enzinger FM
Cancer 1987 Sep 1;60(5):1132-42. doi: 10.1002/1097-0142(19870901)60:5<1132::aid-cncr2820600536>3.0.co;2-l. PMID: 3475157

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
Malignant fibrous histiocytoma of the head and neck: CT and MR imaging findings.
Park SW, Kim HJ, Lee JH, Ko YH
AJNR Am J Neuroradiol 2009 Jan;30(1):71-6. Epub 2008 Oct 8 doi: 10.3174/ajnr.A1317. PMID: 18842757Free PMC Article
Malignant fibrous histiocytoma: between the past and the present.
Al-Agha OM, Igbokwe AA
Arch Pathol Lab Med 2008 Jun;132(6):1030-5. doi: 10.5858/2008-132-1030-MFHBTP. PMID: 18517265
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

Systemic therapy in non-conventional cancers of the larynx.
Tan E, Mody MD, Saba NF
Oral Oncol 2018 Jul;82:61-68. Epub 2018 May 26 doi: 10.1016/j.oraloncology.2018.05.005. PMID: 29909903
Radiation therapy for retroperitoneal sarcoma.
Tuan J, Vitolo V, Vischioni B, Iannalfi A, Fiore MR, Fossati P, Orecchia R
Radiol Med 2014 Oct;119(10):790-802. Epub 2014 Mar 18 doi: 10.1007/s11547-013-0350-3. PMID: 24638910
Chemotherapy in adult soft tissue sarcoma.
Jain A, Sajeevan KV, Babu KG, Lakshmaiah KC
Indian J Cancer 2009 Oct-Dec;46(4):274-87. doi: 10.4103/0019-509X.55547. PMID: 19749457
Therapy insight: malignant primary cardiac tumors.
Reardon MJ, Walkes JC, Benjamin R
Nat Clin Pract Cardiovasc Med 2006 Oct;3(10):548-53. doi: 10.1038/ncpcardio0653. PMID: 16990840
Retroperitoneal sarcomas.
Eilber FC, Eilber KS, Eilber FR
Curr Treat Options Oncol 2000 Aug;1(3):274-8. doi: 10.1007/s11864-000-0040-y. PMID: 12057171

Prognosis

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
Clinicopathological characteristics and survival of malignant fibrous histiocytoma of the bone: A population-based study using the SEER database.
Liu B, Wei H, Ren YJ, Zou D, Zhang K, Ma Q, Xiao X
PLoS One 2020;15(6):e0232466. Epub 2020 Jun 3 doi: 10.1371/journal.pone.0232466. PMID: 32492019Free PMC Article
Dedifferentiated liposarcoma.
Nascimento AG
Semin Diagn Pathol 2001 Nov;18(4):263-6. PMID: 11757866
Retroperitoneal sarcomas.
Eilber FC, Eilber KS, Eilber FR
Curr Treat Options Oncol 2000 Aug;1(3):274-8. doi: 10.1007/s11864-000-0040-y. PMID: 12057171
Adult urological sarcoma.
Russo P, Brady MS, Conlon K, Hajdu SI, Fair WR, Herr HW, Brennan MF
J Urol 1992 Apr;147(4):1032-6; discussion 1036-7. doi: 10.1016/s0022-5347(17)37456-6. PMID: 1552580

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: 35715142
The Management of Head and Neck Sarcoma.
Han S, Yin X, Xu W, Wang Y, Han W
J Craniofac Surg 2020 Mar/Apr;31(2):e189-e192. doi: 10.1097/SCS.0000000000006162. PMID: 31934973
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
Low-Grade Malignant Fibrous Histiocytoma Originating From the Medial Rectus Muscle.
Yan J
J Craniofac Surg 2018 Jul;29(5):e465-e467. doi: 10.1097/SCS.0000000000004481. PMID: 29538190
Dedifferentiated liposarcoma.
Nascimento AG
Semin Diagn Pathol 2001 Nov;18(4):263-6. PMID: 11757866

Recent systematic reviews

Global frequency and distribution of head and neck sarcomas in adulthood: a systematic review.
Mannelli G, Bassani S, Cosi G, Fermi M, Gazzini L, Liberale C, Mazzetti L, Parrinello G, Saibene AM, Molteni G, Comini LV
Eur Arch Otorhinolaryngol 2024 May;281(5):2553-2567. Epub 2024 Feb 21 doi: 10.1007/s00405-024-08477-4. PMID: 38381151
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
Isolated Limb Perfusion and Infusion for Extremity Soft Tissue Sarcoma: A Contemporary Systematic Review and Meta-Analysis.
Neuwirth MG, Song Y, Sinnamon AJ, Fraker DL, Zager JS, Karakousis GC
Ann Surg Oncol 2017 Dec;24(13):3803-3810. Epub 2017 Oct 11 doi: 10.1245/s10434-017-6109-7. PMID: 29022281
Sarcomas other than Kaposi sarcoma occurring in immunodeficiency: interpretations from a systematic literature review.
Bhatia K, Shiels MS, Berg A, Engels EA
Curr Opin Oncol 2012 Sep;24(5):537-46. doi: 10.1097/CCO.0b013e328355e115. PMID: 22729152Free PMC Article

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      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

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

    Reviews

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