The diagnosis of malignant ossifying fibromyxoid tumor should only be made in the presence of areas of typical tumor or with a prior history of typical tumor
Other unusual features
Mucinous microcysts
Vascular invasion
Necrosis
Lack of myxoid stroma
Microcalcifications
Associated epidermoid cysts
Chondroid differentiation with binucleate chondrocytes
Multinucleated giant cells
Richard L Kempson MD
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Original posting/updates: 2/3/07, 8/17/08, 11/20/11
Supplemental studies
Immunohistology
Four Largest Series
Including Other Reports
S100
60-94%
60-94%
Desmin
10-50%
10 to 50%
Smooth muscle actin
0-5%
2 to 50%
GFAP
7%
7-40%
CD57
not tested
60%
NSE
not tested
60%
Keratin
5-13%
0-13%
EMA
0-2%
0-2%
Neurofilament
90%
negative
MUC4
12%
not tested
Data for benign tumors from four largest series: Enzinger 1989, Folpe 2003, Miettinen 2008, Graham 2011
Malignant tumors are reported more frequently positive for keratin, EMA and smooth muscle actin and less frequently positive for S100 and desmin (Graham 2011)
INI1 staining shows a mosaic pattern with loss of expression in 30-60% of cells in 74% of cases (Graham 2011)
Kempson RL, Fletcher CDM, Evans HL, Henrickson MR, Sibley RS. Tumors of the Soft Tissues, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 30, 2001
Fletcher CDM, Unni KK, Mertens F. Pathology and Genetics of Tumours of Soft Tissue and Bone, World Health Organization Classification of Tumours 2002
Weiss SW, Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors, 4th edition, 2001
Folpe AL, Weiss SW. Ossifying fibromyxoid tumor of soft parts: a clinicopathologic study of 70 cases with emphasis on atypical and malignant variants. Am J Surg Pathol. 2003 Apr;27(4):421-31.
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Kilpatrick SE, Ward WG, Mozes M, Miettinen M, Fukunaga M, Fletcher CD. Atypical and malignant variants of ossifying fibromyxoid tumor. Clinicopathologic analysis of six cases. Am J Surg Pathol 1995 Sep;19(9):1039-46
Williams SB, Ellis GL, Meis JM, Heffner DK. Ossifying fibromyxoid tumour (of soft parts) of the head and neck: a clinicopathological and immunohistochemical study of nine cases. J Laryngol Otol 1993 Jan;107(1):75-80
Enzinger FM, Weiss SW, Liang CY. Ossifying fibromyxoid tumor of soft parts. A clinicopathological analysis of 59 cases. Am J Surg Pathol 1989 Oct;13(10):817-27
Miettinen M, Finnell V, Fetsch JF. Ossifying fibromyxoid tumor of soft parts--a clinicopathologic and immunohistochemical study of 104 cases with long-term follow-up and a critical review of the literature. Am J Surg Pathol. 2008 Jul;32(7):996-1005.
Graham RP, Dry S, Li X, Binder S, Bahrami A, Raimondi SC, Dogan A, Chakraborty S, Souchek JJ, Folpe AL. Ossifying fibromyxoid tumor of soft parts: a clinicopathologic, proteomic, and genomic study. Am J Surg Pathol. 2011 Nov;35(11):1615-25