Rhabdomyosarcoma Flashcards
What is rhabdomyosarcoma?
A connective tissue malignancy arising from undifferentiated mesenchymal cells, typically developing into muscle.
Where does rhabdomyosarcoma begin?
In striated muscle.
What are the four histologic subtypes of rhabdomyosarcoma defined by IRS?
- Embryonal
- Botryoid
- Alveolar
- Pleomorphic
What percentage of rhabdomyosarcoma cases are embryonal?
Approximately 60%.
What is the typical prognosis for embryonal rhabdomyosarcoma?
Intermediate prognosis.
What is a characteristic feature of botryoid rhabdomyosarcoma?
Grapelike appearance and encapsulation of the tumor.
Where does botryoid rhabdomyosarcoma commonly present?
In mucosal lined organs such as the vagina, bladder, and nasopharynx.
What is the prognosis for alveolar rhabdomyosarcoma?
Poor prognosis; 54% survival at 5 years.
What age group is primarily affected by alveolar rhabdomyosarcoma?
Teenagers.
What genetic disorder greatly increases the risk of developing rhabdomyosarcoma?
Li-Fraumeni Syndrome.
What is a common symptom of genitourinary rhabdomyosarcoma?
Hematuria or urinary obstruction.
What is the male to female ratio for rhabdomyosarcoma?
1.5 : 1.
What are common imaging studies used to diagnose rhabdomyosarcoma?
- Ultrasound
- CT
- MRI
- PET CT
What are the common routes of spread for rhabdomyosarcoma?
- Local invasion
- Lymphatic spread
- Circulatory system
What are poor prognostic factors for rhabdomyosarcoma?
- Age <1 or >10 years
- Unfavorable primary site
- Tumor >5 cm
- Alveolar or undifferentiated histology
- Residual disease post-surgery
- Stage IV metastasis
