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Published on: April 11, 2018
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Superficial dedifferentiated liposarcoma: A clinicopathologic study.
Melanie Bourgeau1, Jatin S Gandhi1, Kristin K Deeb1
1Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, GA, 30322, USA.
Human Pathology
|February 29, 2024
Summary
Superficial dedifferentiated liposarcoma (sDDLPS) is rare. Primary sDDLPS has a better prognosis than secondary lesions from deep dedifferentiated liposarcoma (DDLPS), highlighting the need for accurate diagnosis.
Area of Science:
- Oncology
- Pathology
- Surgical Oncology
Background:
- Dedifferentiation occurs in approximately 10% of atypical lipomatous tumors/well-differentiated liposarcomas (ALT/WDLPS), primarily in deep-seated locations, conferring metastatic potential.
- Superficial dedifferentiated liposarcoma (sDDLPS) is a rare entity with poorly documented natural history and progression patterns.
Purpose of the Study:
- To investigate the clinical characteristics and outcomes of primary superficial dedifferentiated liposarcoma (sDDLPS).
- To differentiate the prognosis of primary sDDLPS from secondary lesions involving superficial tissues.
Main Methods:
- Retrospective review of a pathology database over 15 years.
- Identification of DDLPS cases involving skin or subcutaneous tissue, requiring evidence of non-lipogenic sarcoma with concurrent ALT/WDLPS and/or MDM2 amplification for primary sDDLPS diagnosis.
Main Results:
- 14 cases of DDLPS involving skin/subcutis were identified: 7 primary sDDLPS and 7 secondary lesions.
- Primary sDDLPS cases presented as undifferentiated spindle cell or pleomorphic sarcoma, with MDM2 amplification in 6/7 cases.
- Secondary lesions, particularly cutaneous metastases, showed a considerably worse prognosis compared to primary sDDLPS.
Conclusions:
- Distinguishing primary sDDLPS from secondary lesions is crucial due to their distinct prognoses.
- Superficial extensions or metastases from deep DDLPS are associated with a significantly worse outcome than tumors originating in superficial tissues.
