Rhabdomyosarcoma pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Pathophysiology
Genetics
Alveolar rhabdomyosarcoma
Specific genetic abnormalities have been identified, that are specific for alveolar rhabdomyosarcomas. They include t(2;13) and t(1;13) chromosomal translocations resulting in PAX3-FKHR and PAX7-FKHR gene fusions.
Microscopic Pathology
Characteristic features on microscopic analysis are variable depending on the chondrosarcoma subtype:
Alveolar Rhabdomyosarcoma
- Characterized by Alveolus-like pattern
- Fibrous septae lined by tumor cells
- Cells may "fall-off" the septa, i.e. be detached/scattered in the alveolus-like space
- Space between fibrous septae may be filled with tumor, called solid variant of alveolar rhabdomyosarcoma
- Rhabdomyoblasts: Essentially diagnostic cells with eccentric nucleus and Moderate amount of intensely eosinophilic cytoplasm
- Nuclear pleomorphism
- Mitoses
Embryonal Rhabdomyosarcoma
- Randomly arranged small cells.
- Myxoid matrix.
- Strap cells: Tadpole like morphology.
- Rhabdomyoblasts: Essentially diagnostic cells with eccentric nucleus and Moderate amount of intensely eosinophilic cytoplasm.
Botryoid Rhabdomyosarcoma
- Malignant cells in an abundant myxoid stroma.
- Non-proliferating layer deep to the surface called "Cambium layer".
- Cambium layer is defined as cellular region deep to epithelial component.
Spindlecell Rhabdomyosarcoma
- Vesicular growth pattern.
- Spindle cells.