Rhabdomyosarcoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2]

Overview

Rhabdomyosarcoma arises from the skeletal muscle cells. Development of alveolar rhabdomyosarcoma is result of specific genetic mutations. The pathogenesis of rhabdomyosarcoma include t(2;13) and t(1;13) chromosomal translocations. The microscopic pathology of rhabdomyosarcoma depends on the histological subtype.

Pathophysiology

Rhabdomyosarcoma arises from the skeletal muscle cells.

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 rhabdomyosarcoma 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

Alveolar Rhabdomyosarcoma- intermediate magnification

Alveolar Rhabdomyosarcoma - high magnification

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