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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
Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 2.
Chokoeva AA, Tchernev G, Cardoso JC, Patterson JW, Dechev I, Valkanov S, Zanardelli M, Lotti T, Wollina U
Int J Immunopathol Pharmacol 2015 Jun;28(2):178-86. Epub 2015 Mar 26 doi: 10.1177/0394632015575977. PMID: 25816393

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
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 tumors.
Stadler FJ, Scott GA, Brown MD
Semin Cutan Med Surg 1998 Jun;17(2):141-52. doi: 10.1016/s1085-5629(98)80007-2. PMID: 9669607

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
The rare primary bone sarcomas: imaging-pathological correlation.
Berkeley R, Andrei V, Saifuddin A
Skeletal Radiol 2021 Aug;50(8):1491-1511. Epub 2021 Jan 7 doi: 10.1007/s00256-020-03692-6. PMID: 33410967
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
Atypical fibroxanthoma and malignant fibrous histiocytoma.
Withers AH, Brougham ND, Barber RM, Tan ST
J Plast Reconstr Aesthet Surg 2011 Nov;64(11):e273-8. Epub 2011 Jun 12 doi: 10.1016/j.bjps.2011.05.004. PMID: 21664889
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

Sarcoma.
Skubitz KM, D'Adamo DR
Mayo Clin Proc 2007 Nov;82(11):1409-32. doi: 10.4065/82.11.1409. PMID: 17976362
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
Radiation-induced sarcoma.
Patel SR
Curr Treat Options Oncol 2000 Aug;1(3):258-61. doi: 10.1007/s11864-000-0037-6. PMID: 12057168
Thymectomy and malignancy.
Masaoka A, Yamakawa Y, Niwa H, Fukai I, Saito Y, Tokudome S, Nakahara K, Fujii Y
Eur J Cardiothorac Surg 1994;8(5):251-3. doi: 10.1016/1010-7940(94)90155-4. PMID: 8043287
Liposarcoma.
Springfield D
Clin Orthop Relat Res 1993 Apr;(289):50-7. PMID: 8472432

Prognosis

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
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 tumors.
Stadler FJ, Scott GA, Brown MD
Semin Cutan Med Surg 1998 Jun;17(2):141-52. doi: 10.1016/s1085-5629(98)80007-2. PMID: 9669607
Thymectomy and malignancy.
Masaoka A, Yamakawa Y, Niwa H, Fukai I, Saito Y, Tokudome S, Nakahara K, Fujii Y
Eur J Cardiothorac Surg 1994;8(5):251-3. doi: 10.1016/1010-7940(94)90155-4. PMID: 8043287
Liposarcoma.
Springfield D
Clin Orthop Relat Res 1993 Apr;(289):50-7. PMID: 8472432

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
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 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
Dedifferentiated liposarcoma.
Nascimento AG
Semin Diagn Pathol 2001 Nov;18(4):263-6. PMID: 11757866
Thymectomy and malignancy.
Masaoka A, Yamakawa Y, Niwa H, Fukai I, Saito Y, Tokudome S, Nakahara K, Fujii Y
Eur J Cardiothorac Surg 1994;8(5):251-3. doi: 10.1016/1010-7940(94)90155-4. PMID: 8043287

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
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
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
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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