Objective:

To report a rare case of dedifferentiated liposarcoma (DDLPS) with heterologous myogenic differentiation and testicular metastasis.

Approach:

Key Findings:

• DDLPS with heterologous myogenic differentiation is rare and can be misclassified as rhabdomyosarcoma.
• Testicular metastasis of DDLPS is extremely rare, with only isolated case reports documented in the literature.
• MDM2 and CDK4 gene amplification is critical for accurate diagnosis of DDLPS.
• Postoperative findings indicated early pulmonary metastases, highlighting the aggressive nature of this tumor.

Interpretation:

This case highlights the critical importance of MDM2/CDK4 FISH testing in accurately diagnosing DDLPS with heterologous differentiation, particularly when myogenic markers are expressed.

Limitations:

• The rarity of the case limits broader applicability of findings.
• Only isolated case reports of testicular metastasis exist in literature.

Conclusion:

Accurate diagnosis of DDLPS with heterologous differentiation is crucial for appropriate management.

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