Osteosarcoma
What is the standard treatment and protocol number for osteosarcoma? What is the first line treatment for relapsed disease?
MAP therapy (AOST0331-like) as upfront therapy, relapsed is regorafenib (stivarga)
What is the current standard therapy offered for rhabdomyosarcoma?
High dose VAC (D9803-like)
What is the standard treatment for Ewings sarcoma?
AEWS0031-like (or sometimes AEWS1221-like), VDC/IE
Name the protocols available for Wilms tumor patients.
EE4A (low risk), DD4A (standard risk or patients with metastasis at diagnosis), Regimen M
Name the only two liver tumors that actually arise from hepatocytes.
Hepatoblastoma and Hepatocellular carcinoma
What pre assessments are required for MAP therapy? How often are they done?
audiogram prior to every cisplatin, ECHOs every 150 mg/m2 until 300 mg/m2, then with every cycle (prior to cycle 5 and cycle 6)
What are considered the low risk sites of disease for rhabdomyosarcoma? What are the sites of intermediate or high risk?
High risk: Parameningeal, extremities, vaginal, chest, pelvis, bladder
Is Ewing sarcoma radiation sensitive? What chemotherapy changes if radiation is given?
Yes, local control can be either surgical or radiation or both if positive margins with surgical resection, typical course is 5580 cGy
What is the most common site of metastasis of clear cell sarcomas?
Brain
What protocol is used to treat germ cell tumors?
PEb or BEP (BEP for > 13 yo or high risk), study AGCT1531
When is local control usually done in patients with osteosarcoma?
week 10 after 2 cycles of MAP
What is the standard chemotherapy for relapsed rhabdomyosarcoma?
Cyclophosphamide, vinorelbine and temsirolimus
What is the most common therapy given to relapsed Ewing sarcoma patients?
VOIT (VCR, oral irinotecan and temozolomide) for about one year or until progression
Name the reasons why Wilms tumor patients would be upstaged.
Metastasis still at week 6 imaging, poor cytogenetics (loss of LOH at 1q and 16 p, 1q gain), anaplasia
What preassessments are required prior to germ cell therapy?
True or False: Patients with osteosarcoma never receive radiation.
False. They receive radiation for difficult to treat areas or relapses for symptom control, they just require higher doses of radiation
Are there any new studies open for rhabdomyosarcoma?
Yes, ARST 2031, replaces VCR with vinorelbine and decreases cyclo to 1200 mg/m2 and adds maintenance for 6 months
Does upfront therapy change based on local or metastatic disease at diagnosis?
Therapy is the same regardless of metastasis but local control will change and prognosis is worse with metastasis
What regimen is given to patients diagnosed with clear cell sarcoma?
Regimen I (low dose and high dose options)
What is the standard amount of cycles for germ cell therapy?
4
Name the criteria required for early discharge from high dose methotrexate admissions.
Methotrexate level <1, creatinine at or below upper age limit AND <125% of admission creatinine, tolerating po fluids and leucovorin, able to return to clinic within 48-72 hours
What genetic fusion is present in a fusion positive rhabdomyosarcoma? Does this change the prognosis?
FOXO1 fusion, makes more aggressive and more likely to relapse
How often are ECHOs required for Ewings patients?
Every other cycle of doxorubicin (cycles 1, 5 and 13)
What genetic syndromes predispose an individual to Wilms tumor?
Beckweidmann syndrome and WAGR
What is the most likely place for metastasis for liver tumors?
lungs