A case report of abdominal metastatic dermatofibrosarcoma protuberans misdiagnosed as gastrointestinal stromal tumor

Diagn Pathol. 2024 Feb 22;19(1):41. doi: 10.1186/s13000-023-01430-9.

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a low-grade malignant soft-tissue tumor that originates from the skin. It has a slow onset in the early stages, non-specific clinical symptoms, low specificity, and can easily be overlooked, missed, or misdiagnosed by clinicians and pathologists. In addition, DFSP is prone to recurrence after local surgical treatment; however, distant metastasis, especially abdominal metastasis, is rare, which is also a challenge for the accurate diagnosis of DFSP when it progresses distantly. Now a case of abdominal metastasis of DFSP is reported. The patient has been treated with imatinib for ten years, and the lesion has shrunk, but because the patient has been receiving imatinib treatment, his abdominal lesion was once misdiagnosed as gastrointestinal stromal tumor. Therefore, we report on this case to enhance the understanding of the diagnosis and treatment of DFSP, and to provide reference for the pathological diagnosis and precise treatment of such patients.

Keywords: Abdominal metastatic; Dermatofibrosarcoma protuberans; Gastrointestinal stromal tumor.

Publication types

  • Case Reports

MeSH terms

  • Dermatofibrosarcoma* / diagnosis
  • Dermatofibrosarcoma* / pathology
  • Diagnostic Errors*
  • Gastrointestinal Stromal Tumors* / diagnosis
  • Gastrointestinal Stromal Tumors* / pathology
  • Humans
  • Imatinib Mesylate / therapeutic use
  • Skin Neoplasms* / diagnosis
  • Skin Neoplasms* / pathology

Substances

  • Imatinib Mesylate