Soft Tissue Tumors Flashcards
What is the most common soft tissue tumor in childhood?
Rhabdomyosarcoma is the most common soft tissue tumor of childhood and makes up about 5% of all childhood cancers.
What syndrome has a higher incidence of rhabdomyosarcoma?
Li-Fraumeni syndrome
What tissue/cell type is affected by rhabdomyosarcoma?
Rhabdomyosarcoma arises from the same embryonic mesenchyme as striated skeletal muscle.
What are the two main histologic substypes of rhabdomyosarcoma?
Embryonal, found in 60-70% of cases, has the more favorable prognosis. Alveolar is found in the other 20-30% of cases and has a worse prognosis.
Which sites are common for the botryoid subtype of rhabdomyosarcoma?
Vagina, bladder, nasopharynx, and middle ear.
How does rhabdomyosarcoma present?
The most common presentation is a mass lesion, most commonly found on the head and neck, then in the GU region, followed by extremity and truncal tumors.
What is the treatment for rhabdomyosarcoma?
Surgical excision, if possible, is key. Chemotherapy is indicated for all children with rhabdomyosarcoma. Radiation therapy is typically used as well, but depends on the extent of tumor resection.
What chemotherapeutic agents are commonly used in the treatment of low and intermediate risk rhabdomyosarcoma?
Vincristine, actinomycin D, and high-dose cyclophosphamide.
What chemotherapeutic agents are commonly used in the treatment of high risk rhabdomyosarcoma?
Vincristine, actinomycin D, and high-dose cyclophosphamide are first-line. Additional agents may be used as well, including doxorubacin, ifosfamide, and etoposide.
Describe the typical presentation and management of fibrosarcoma.
It is the most common soft tissue sarcoma in children <1 year of age. It occurs most often in the extremities and rarely metastasizes. Surgical excision is the treatment of choice.
Describe the typical presentation of synovial sarcoma.
It occurs around the knee or thigh and is characterized by a nonrandom translocation, t(X;18).
Describe the typical presentation of leiomyosarcoma.
It is the most common retroperitoneal soft tissue tumor in children and typically arises in the GI tract. Patients with AIDS and post-transplant patients on immunosuppression are at higher risk.
