adult-type fibrosarcoma
Digital slides
UI: - Adult-type fibrosarcoma
True fibrosarcoma is exceedingly rare, accounting for <1% of approximately 10,000 adult soft tissue sarcomas and should be diagnosed with great caution.
64% of putative fibrosarcoma are reclassified, most commonly as monophasic synovial sarcoma and solitary fibrous tumor. A rearrangement of the SYT gene must be searched systematically.
Synopsis
sex ratio 1/1
median age: 52.5 y (range 2 to 99 y)
various anatomic sites
Differential diagnosis
undifferentiated pleomorphic sarcoma
synovial sarcoma
solitary fibrous tumor
myxofibrosarcoma
malignant peripheral nerve sheath tumor
dermatofibrosarcoma protuberans
desmoplastic melanoma
low-grade fibromyxoid sarcoma
sarcomatoid carcinoma
desmoid fibromatosis (desmoid-type fibromatosis)
rhabdomyosarcomas
myofibroblastic sarcoma
spindle-cell liposarcoma
sclerosing epithelioid fibrosarcoma
fibroma-like epithelioid sarcoma
leiomyosarcoma
cellular fibrous histiocytoma
Exclusive of undifferentiated pleomorphic sarcoma, the distinction of which from fibrosarcoma is subjective, 64% of putative fibrosarcoma are reclassified, most commonly as monophasic synovial sarcoma and solitary fibrous tumor.
True fibrosarcoma is exceedingly rare, accounting for <1% of approximately 10,000 adult soft tissue sarcomas and should be diagnosed with great caution.
Cytognetics
t(2;19) (#9491323#)
No consistent abnormality has been detected among the complex karyotypes.
CGH (#11921284#)
| Gains | 12q21 | 12q14-q15 | 14q22 | 4q22 | 7q31 | 14q23-q24 | 4q21 | 4q23-q24 | 8q22 | 12q22 |
12q gains correlated with a poor outcome (#11921284#)
High-level amplifications
- |12q21|18p |1p21|4q31.3|7p21|12q14-q15|Xp22.1-p22.2|Xq22-q23|
References
Adult-type fibrosarcoma: A reevaluation of 163 putative cases diagnosed at a single institution over a 48-year period. Bahrami A, Folpe AL. Am J Surg Pathol. 2010 Oct;34(10):1504-13. PMID: #20829680#
Schmidt H, Taubert H, Wurl P, Kappler M, Lange H, Bartel F, Bache M, Holzhausen HJ, Hinze R. Gains of 12q are the most frequent genomic imbalances in adult fibrosarcoma and are correlated with a poor outcome. Genes Chromosomes Cancer. 2002 May;34(1):69-77. PMID: #11921284#