Wilms Tumor Flashcards
Treatment for very low risk WT
Very low risk:
<2 yo < 550 grams FH WT- observation only
Low risk:
FH WT no LOH- vincristine, dactinomycin
19 weeks
Average risk: Stage 1-11 FH WT with LOH 1p 16q( both) Stage 3 FH WT Vincristine, dactinomycin and doxorubicin Abdo or flank RT for stage III
High risk:
Stage IV WT
Diffuse Anaplasia
Stage III for LOH 16q and 1p
Vincristine, dactinomycin and doxorubicin
Abdo or flank RT for abdominal stage III
Regimen M- cyclo and VP 16 for those whose Kung Mets don’t resolve by 6 weeks
Definition and Treatment for low risk WT
Stage I-II Favourable histology WT( cannot have both LOH 1p, 16 q)
Vincristine, dactinomycin for 18 weeks
Definition and treatment of average risk WT
Definition:
Stage III FH WT without LOH
Stage I-II WT with LOH of 1p and 16q
Treatment:
Vincristine, dactinomycin and doxorubicin for 24 weeks
Abdominal RT for stage III disease
Definition and treatment of high risk WT
Definition:
Stage III WT with LOH of 1p and 16q
Stage IV WT
Diffuse anaplasia
Treatment:
Regimen M
vincristine, dactinomycin and doxorubicin + cyclo and etoposide
Regimen I VIncristine, doxo, cyclo and etoposide
RT for abdominal stage III and for diffuse anaplasia
Who gets RT in WT
Abdominal Stage III disease
Stage I-II with focal or diffuse anaplasia
Stage III SLURRP Spillage LN positive Unresectable Relapsed Rupture or biopsy Peritoneal implants
Almost always whole abdo RT unless there was a flank biopsy or intraoperative tumor spillage only on the flank
What are indications for neoadjuvant chemo in COG for Wilms tumor
Bilateral disease
Diffuse pulmonary Mets making GA unsafe
Would have to remove adjacent organs ( other than adrenal) to completely resect
Tumor above level of hepatic veins
What bio pathways are involved in Wilms tumor
WT1
WT2 ( IGF2)
WTX ( beta catenin pathway)
Prognostic factors in Wilms tumor
Size ( < 550 g in infants with FH Wilms is good) Age- older age bad Anaplasia- the most important LOH at 1p and 16q 1q amplification Abdominal Stage III or above Metastatic disease Blastemal predominance ( post chemo only)
