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malignant fibrous histiocytoma (MFH, angiomatoid fibrous histiocytoma)
most commonly diagnosed sarcoma of extremities.
Epidemiology:
- older adults
- peak incidence 7th decade
- angiomatoid variant occurs in children & adolescents
Microscopic pathology: (subtypes)
- myxoid
- giant cell
- inflammatory
- angiomatoid (children & adolescents)
- pleomorphic
Genetics:
- beta-catenin mutations/accumulation are seen in some cases these cases tended to have higher MIB-1 labelling
- other implicated genes:
- MFHAS1, PRUNE
- chromosomal translocations involving EWSR1 are associated with angiomatoid fibrous histiocytoma (AFH)
a) translocation t(12;22)(q13;q12) with ATF1 generates a chimeric EWSR1/ATF1 protein
b) translocation t(2;22)(q33;q12) with CREB1 generates a EWSR1/CREB1 fusion gene that is most common genetic abnormality in this tumor type
- chromosomal translocation t(12;16)(q13;p11.2) involving FUS with ATF1 generates a chimeric FUS/ATF1 protein
Clinical manifestations:
- painless mass
- most common sites
a) lower extremity
b) upper extremity
c) retroperitoneum
Management:
- malignant fibrous histiocytoma of bone see [1]
Interactions
disease interactions
General
histiocytoma
soft tissue sarcoma (STS)
References
- National Cancer Institute - Osteosarcoma/Bone Fibrous
Histiocytoma Treatment
http://www.nci.nih.gov/cancertopics/pdq/treatment/osteosarcoma/healthprofessional
- DeVita et al. Cancer, Principles & Practice of Oncology.
Lippincott, Williams & Wilkins 6th ed. 2001
- Sakamoto A, Oda Y, Adachi T, Saito T, Tamiya S, Iwamoto Y,
Tsuneyoshi M.
Beta-catenin accumulation and gene mutation in exon 3 in
dedifferentiated liposarcoma and malignant fibrous
histiocytoma.
Arch Pathol Lab Med. 2002 Sep;126(9):1071-8.
PMID: 12204056
Databases & Images
OMIM 612160
images related to malignant fibrous histiocytoma