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Primary intrathoracic low-grade fibromyxoid sarcoma
Joseph D Jakowski1, Paul E Wakely
1Department of Pathology, The Ohio State University College of Medicine, Columbus, OH 43210, USA.
Human Pathology
|February 16, 2008
Summary
Low-grade fibromyxoid sarcoma (LGFMS) is a rare heart tumor. This case highlights LGFMS as an underrecognized cause of spindle cell neoplasms in the chest.
Area of Science:
- Oncology
- Pathology
- Cardiology
Background:
- Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft tissue tumor typically affecting young adults.
- Intrathoracic LGFMS is exceptionally rare, with few documented cases.
Observation:
- A case of LGFMS originating from the epicardial surface of the right heart in a 44-year-old woman is presented.
- Diagnostic methods included characteristic light microscopy with giant collagen rosettes, immunohistochemistry, FUS translocation analysis, and electron microscopy.
- Aspiration cytology revealed a nonspecific bland spindle cell lesion.
Findings:
- The tumor exhibited a bland spindle cell morphology with distinctive giant collagen rosettes.
- Immunohistology and FUS translocation confirmed the diagnosis.
- Electron microscopy supported a fibroblastic phenotype.
Implications:
- LGFMS may be an underdiagnosed cause of intrathoracic spindle cell neoplasms.
- Consider LGFMS in the differential diagnosis for mediastinal, pleural, cardiac, and pulmonary spindle cell lesions.
- This case expands the known locations for LGFMS within the thoracic cavity.
