Fibrosarcomatous transformation of dermatofibrosarcoma protuberans - abdominal wall
Presentation
Lump over anterior abdominal wall since 5 years, now presented with sudden increase in size.
Patient Data
Well defined heterogeneously enhancing mass lesion is seen in the anterior abdominal wall at right paramedian location in infraumbilical location in subcutaneous plane abutting the umbilicus medially. A satellite nodule is also seen in the adjacent region communicating with above mentioned lesion lesion by fibrous strand with surrounding stranding and haziness. Underlying rectus appears to be unremarkable.
Case Discussion
This 30-year-old female presented with lump over anterior abdominal wall since 5 years with sudden increase in size. Now presented with increase in size and pain. On examination, per abdomen - soft, mobile hard lump over anterior abdominal wall was palpated below umbilicus. Skin over the lump - fixed.
CECT abdomen was done that revealed well defined heterogeneously enhancing mass lesion in the anterior abdominal wall with satellite nodules, fibrous strand with surrounding stranding and haziness.
Biopsy was performed that revealed: Cellular spindle cell neoplasm composed of elongated cells in interlacing fascicles. The cells have elongated, wavy nuclei with fine chromatin with inconspicuous to tiny nucleoli. No necrosis seen. On IHC, these spindle cells are positive for CD34 (heterogeneous) while negative for CK, SMA,DESMIN, TLE1, SOX10, S-100. H3k27me3 is intact in spindle cells. p53 expression is increased in areas of cd34 loss. Features are consistent with fibrosarcoma arising in background of dermatofibrosarcoma protuberans.