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Tumorsphere Derivation and Treatment from Primary Tumor Cells Isolated from Mouse Rhabdomyosarcomas
Published on: September 13, 2019
Primary embryonal spindle cell cardiac rhabdomyosarcoma: case report
Giulio Fraternali Orcioni1, Jean Louis Ravetti, Gabriele Gaggero
1Department of Pathology, Anatomic Pathology Division, San Martino Hospital, Genoa, Italy.
Pathology, Research and Practice
|July 7, 2009
Summary
Primary cardiac rhabdomyosarcomas (RMS) are rare. This report details the first described case of spindle cell RMS in the heart, a rare variant of embryonal RMS.
Area of Science:
- Cardiovascular Pathology
- Oncology
- Histopathology
Background:
- Primary cardiac tumors are rare, with sarcomas comprising approximately 10% of primary heart neoplasms.
- Rhabdomyosarcomas (RMS) represent a minority among cardiac sarcomas.
Observation:
- A unique case of primary left atrial RMS with a leiomyosarcoma-like appearance is presented.
- Histological examination revealed tightly-packed spindle-shaped cells in fascicles.
- Immunohistochemistry showed diffuse vimentin, muscle-specific actin, desmin, myogenin, and WT-1 staining, with focal fast-myosin and sarcomeric actin positivity.
Findings:
- The tumor cells exhibited weak calponin staining and were negative for alpha-SMA, H-caldesmon, CD34, EMA, CK w.s., and S100 protein.
- Electron microscopy identified poorly differentiated spindle cells with contractile filaments.
- The final diagnosis was embryonal RMS, spindle cell variant.
Implications:
- Spindle cell RMS is a recognized variant of embryonal RMS, typically found in soft tissues.
- This case represents the first documented instance of primary cardiac spindle cell RMS, expanding the known spectrum of cardiac neoplasms.