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Dedifferentiated liposarcoma with rhabdomyoblastic differentiation
Shio Shimada1, Takashi Ishizawa, Keisuke Ishizawa
1Department of Pathology, Saitama Medical School, Morohongo 38, Moroyama, Iruma-gun, Saitama, 350-0495, Japan. shio@saitama-med.ac.jp
Virchows Archiv : an International Journal of Pathology
|July 14, 2005
Summary
Dedifferentiated liposarcoma (DDL) can exhibit rhabdomyosarcomatous areas, confirmed by specific protein markers and ultrastructural findings. These areas share common genetic alterations (mdm2 and cdk4 amplification) with other DDL components.
Area of Science:
- Oncology
- Pathology
- Genetics
Background:
- Dedifferentiated liposarcoma (DDL) typically presents with features lacking specific differentiation.
- Rare cases of DDL show specific differentiation, resembling rhabdomyosarcoma, leiomyosarcoma, or osteosarcoma.
Purpose of the Study:
- To perform a pathologic and genetic analysis of DDLs with rhabdomyosarcomatous areas.
- To investigate the genetic profiles of the rhabdomyosarcomatous components within DDLs.
Main Methods:
- Pathologic examination of three DDL cases with rhabdomyosarcomatous areas.
- Immunohistochemical analysis for muscle-specific markers (desmin, myoglobin, HHF-35, myogenin).
- Ultrastructural examination of rhabdomyoblasts.
- Real-time PCR to detect mdm2 and cdk4 amplification.
Main Results:
- Rhabdomyoblasts were identified in MFH- or fibrosarcoma-like areas of DDLs.
- Rhabdomyoblasts showed positive immunoreactivity for desmin, myoglobin, muscle actin, and myogenin.
- Ultrastructural analysis confirmed the presence of rhabdomyoblasts with muscle-specific features.
- Amplification of mdm2 and cdk4 was detected in both well-differentiated and dedifferentiated areas, including those with rhabdomyoblasts.
Conclusions:
- DDLs with rhabdomyosarcomatous areas represent a rare subtype of DDL.
- The rhabdomyosarcomatous components in DDLs exhibit genuine myogenic differentiation.
- These specific areas harbor the same genetic alterations (mdm2 and cdk4 amplification) as the broader DDL tumor, suggesting a common clonal origin.