Praxis Medical Insights

Est. 2024 • Clinical Guidelines Distilled

Made possible by volunteer editors from the University of Calgary & University of Alberta

Last Updated: 10/15/2025

Diagnostic Considerations for Spindle Cell Lesions

Introduction to Diagnostic Possibilities

  • The American Gastroenterological Association suggests that inflammatory fibroid polyp is the leading diagnosis for gastrointestinal tract lesions with IHC positive for CD34 and negative for CD117 (c-kit) 1, 2
  • The National Comprehensive Cancer Network recommends considering dermatofibrosarcoma protuberans (DFSP) for skin or soft tissue lesions with CD34 positivity, storiform or fascicular pattern of bland spindled cells, and negative Factor XIIIa 3, 4

Diagnostic Criteria for Specific Lesions

  • Inflammatory fibroid polyp is characterized by IHC positive for CD34, negative for CD117 (c-kit), and shows spindle-cell proliferation with mixed inflammatory infiltrate, typically located in the submucosa 1, 2
  • Dermatofibrosarcoma protuberans (DFSP) is characterized by CD34 positivity, storiform or fascicular pattern of bland spindled cells, and negative Factor XIIIa, with weak MDM2 positivity being non-specific 3, 4

Critical Diagnostic Information

  • Anatomic location, such as gastrointestinal tract versus skin/soft tissue, is crucial for diagnosis, as suggested by the European Society for Medical Oncology 5
  • CD117 (c-kit) status must be negative to exclude GIST, according to the American Gastroenterological Association 1, 2, 5
  • Factor XIIIa negativity helps narrow the diagnosis to DFSP, as recommended by the National Comprehensive Cancer Network 3, 4

Molecular Testing

  • COL1A1-PDGFB fusion (t(17;22)) by FISH or PCR should be considered if DFSP is suspected, as suggested by the National Comprehensive Cancer Network 3, 4