malignant fibrous histiocytoma (MFH, angiomatoid fibrous histiocytoma)
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Introduction
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:
- chromosomal translocations involving EWSR1 are associated with angiomatoid fibrous histiocytoma (AFH)
- translocation t(12;22)(q13;q12) with ATF1 generates a chimeric EWSR1/ATF1 protein
- 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
Management
- malignant fibrous histiocytoma of bone see [1]
More general terms
References
- ↑ 1.0 1.1 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: https://www.ncbi.nlm.nih.gov/pubmed/12204056
Patient information
malignant fibrous histiocytoma patient information