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A case of dedifferentiated solitary fibrous tumor in the pelvis with TP53 mutation.
- Source :
- Virchows Archiv: European Journal of Pathology; Nov2014, Vol. 465 Issue 5, p615-621, 7p
- Publication Year :
- 2014
-
Abstract
- Solitary fibrous tumors (SFTs), initially observed in the pleura, were later found to develop in almost any extrapleural site. Dedifferentiation within SFTs was characterized only recently. We report a case of dedifferentiated SFT arising within the pelvis of a 70-year-old Japanese woman. Macroscopically, the resected tumor measured 17 × 17 × 13 cm. Histologically, the tumor displayed distinct heterologous osteosarcomatous and chondrosarcomatous components on a background of conventional SFT. Immunohistochemistry uncovered a loss of CD34 expression in the dedifferentiated area, whereas the nuclear expression of signal transducer and activator of transcription-6 ( STAT6) and NGFI-A-binding protein 2 ( NAB2) was maintained in both components. The p53 mutation 158 CGC > CAC (A158H) was found only in the dedifferentiated component. Furthermore, a fusion gene of NAB2(exon6)-STAT6(exon18) was detected in both the conventional and dedifferentiated components. The patient died of the disease 4 months after surgery. This case identifies a possible role of p53 dysfunction in the dedifferentiation process of SFT as reported in other sarcomas. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 09456317
- Volume :
- 465
- Issue :
- 5
- Database :
- Complementary Index
- Journal :
- Virchows Archiv: European Journal of Pathology
- Publication Type :
- Academic Journal
- Accession number :
- 99087239
- Full Text :
- https://doi.org/10.1007/s00428-014-1625-3