Rhabdomyosarcoma (RMS) Flashcards
What is Rhabdomyosarcoma (RMS)?
A malignant tumor of striated muscle, constituting about 40% of sarcoma cases in children.
What are the most common sites of Rhabdomyosarcoma?
The head and neck area, abdomen, GU region, extremities, and trunk.
What are the distinct histologic variants of Rhabdomyosarcoma?
Embryonal (most common) and Alveolar.
What characterizes Embryonal Rhabdomyosarcoma?
Occurs in the head and neck or GU regions and presents in younger patients.
What characterizes Alveolar Rhabdomyosarcoma?
Less common than embryonal, typically occurs in the legs, chest, abdomen, and genital/anal regions. Carries a worse prognosis and presents during the teenage years. Often characterized by translocations on chromosome 13.
What genetic mutation may be related to familial soft tissue sarcoma cases?
The p53 oncogene mutation.
What is the common age group for RMS cases?
Common in children under 10 years old
Which age group tends to have a better outlook for RMS?
Children aged 1 to 9 tend to have a better outlook than infants or older children and adults
Is RMS more common in boys or girls?
More common in boys vs girls
What are the most likely sites of distant metastasis?
The most likely sites of distant metastasis are the lung and bone marrow.
What tests are done to detect metastases?
A bone marrow biopsy and chest CT scan are done to detect metastases.
What chemotherapy agents are commonly used for RMS treatment?
Vincristine and Actinomycin-D.
What should be spared during chemotherapy in RMS treatment?
The bone marrow in the pelvic wings should be spared, if possible.
What is the radiation dose for microscopic residual disease in RMS?
3600 cGy for microscopic residual disease.
What is the radiation dose for lymph node disease in RMS?
4140 cGy for lymph node disease.
