International Society of Paediatric Oncology (SIOP) classification of Wilms tumors according to tumor histology as assessed after prenephrectomy chemotherapy

Classification	Comments
Low risk
Completely necrotic	No viable tumor persists (100% necrotic).
Intermediate risk
Regressive type	Chemotherapy-induced changes comprise more than two-thirds of the tumor; the remaining tumor comprises 1, 2, or 3 Wilms tumor components in any proportion.
Mixed type*	2 or 3 components present, none of which occupies more than two-thirds of the viable tumor*.
Epithelial type*	Epithelial component comprises more than two-thirds of the viable tumor; the remaining viable tumor comprises stromal (up to one-third) and blastemal (up to 10%) components*.
Stromal type*	Stromal component comprises more than two-thirds of the viable tumor; the remaining viable tumor comprises epithelial (up to one-third) and blastemal (up to 10%) components*.
Focal anaplasia*	Localized anaplasia in any tumor type*.
High risk
Blastemal type*	Blastemal component comprises more than two-thirds of the viable tumor; the remaining viable tumor comprises epithelial and/or stromal components in any proportion*.
Diffuse anaplasia*	Nonlocalized anaplasia in any tumor type, regardless of the extent of necrosis*.

* More than one-third of the tumour is viable.

Adapted from: Vujanić GM, Sandstedt B, Harms D, et al. Revised International Society of Paediatric Oncology (SIOP) working classification of renal tumors of childhood. Med Pediatr Oncol 2002; 38:79.

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International Society of Paediatric Oncology (SIOP) classification of Wilms tumors according to tumor histology as assessed after prenephrectomy chemotherapy