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Dedifferentiated Liposarcoma Masquerading as Rhabdomyosarcoma.
Anna Kobayashi1, Takanori Hirose, Eiji Kudo
1*Department of Diagnostic Pathology, Hyogo Cancer Center, Akashi Departments of †Diagnostic Pathology ‡Surgery, Tokushima Prefectural Central Hospital, Tokushima, Japan.
Applied Immunohistochemistry & Molecular Morphology : AIMM
|November 2, 2016
Summary
A rare retroperitoneal dedifferentiated liposarcoma (DDLPS) mimicked rhabdomyosarcoma in a patient. Molecular analysis confirmed DDLPS, highlighting challenges in diagnosing adult retroperitoneal tumors.
Area of Science:
- Oncology
- Pathology
- Genetics
Background:
- Retroperitoneal tumors can present diagnostic challenges, particularly when exhibiting unusual morphology.
- Distinguishing between different types of sarcomas is crucial for appropriate patient management and treatment.
Observation:
- A 74-year-old male presented with a retroperitoneal mass initially suspected to be rhabdomyosarcoma.
- Histological examination of the primary tumor revealed dedifferentiated liposarcoma (DDLPS).
- A recurrence three years later showed distinct morphological features, including a rhabdomyosarcoma-like component.
Findings:
- The recurrent tumor displayed both lipogenic and non-lipogenic components, with the latter mimicking rhabdomyosarcoma due to positive myogenin and desmin staining.
- Fluorescence in situ hybridization (FISH) for FOXO1 rearrangement was negative, ruling out a secondary rhabdomyosarcoma.
- Dual-color in situ hybridization confirmed high-level amplification of MDM2, characteristic of DDLPS.
Implications:
- This case underscores the importance of considering DDLPS in the differential diagnosis of adult retroperitoneal rhabdomyosarcoma-like tumors.
- Advanced molecular techniques like in situ hybridization are vital for accurate diagnosis, especially when the lipogenic component is absent.
- Careful distinction between DDLPS and other sarcomas is essential for guiding therapeutic strategies and improving patient outcomes.
