Expanding the differential of cutaneous epithelioid tumors: A case of dedifferentiated liposarcoma with epithelioid features involving the skin, with review of the literature

J Cutan Pathol. 2020 Jun;47(6):554-560. doi: 10.1111/cup.13650. Epub 2020 Feb 11.


Liposarcomas are categorized into four distinct histopathological subtypes: atypical lipomatous tumors (ALT)/well-differentiated liposarcoma (WDL), dedifferentiated, myxoid, and pleomorphic. Dedifferentiated liposarcomas account for approximately 18% of all liposarcomas, characteristically arising in the deep soft tissue. They are reported to have lower rates of metastasis compared to other pleomorphic sarcomas.1 -3 The classic histopathologic appearance is ALT/WDL admixed or juxtaposed with a predominantly nonlipogenic sarcoma. Epithelioid features are rare, appearing in as few as 3% of tumors, and have not previously been reported in a superficial location. Herein, we present a 57-year-old male with intradermal and subcutaneous metastasis of his known deep dedifferentiated liposarcoma with epithelioid features. By H&E the tumor featured cords and sheets of crowded, plump, epithelioid cells with thick nuclear membranes and prominent nucleoli, which raised a broad differential including carcinoma and melanoma. By immunohistochemistry the tumor was diffusely positive for MDM2 and CDK4, on the other hand stains for Sox10, Melan A, MITF, CKAE1/3, desmin, and S100 protein were negative. This case serves as an opportunity to raise awareness of this rare morphological subtype, which can involve the skin and mimic epithelial and melanocytic malignancies. It can be a potential diagnostic pitfall, especially if metastases are the first presentation.

Keywords: MDM2; cutaneous; dedifferentiated; epithelioid; liposarcoma; metastasis.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Cyclin-Dependent Kinase 4 / metabolism
  • Diagnosis, Differential
  • Doxorubicin / therapeutic use
  • Epithelioid Cells / pathology*
  • Fatal Outcome
  • Female
  • Humans
  • Immunohistochemistry / methods
  • Liposarcoma / diagnosis
  • Liposarcoma / drug therapy
  • Liposarcoma / metabolism*
  • Liposarcoma / surgery
  • Lost to Follow-Up
  • Male
  • Middle Aged
  • Neoplasm Metastasis / pathology
  • Proto-Oncogene Proteins c-mdm2 / metabolism
  • Skin Neoplasms / pathology*
  • Soft Tissue Neoplasms / pathology*
  • Subcutaneous Tissue / pathology
  • Topoisomerase II Inhibitors / therapeutic use


  • Topoisomerase II Inhibitors
  • Doxorubicin
  • MDM2 protein, human
  • Proto-Oncogene Proteins c-mdm2
  • Cyclin-Dependent Kinase 4