Alex's Lemonade Stand Foundation for Childhood Cancer

Lay Summary: Wilms tumor is the most common pediatric kidney cancer. The best predictor of clinical outcome for Wilms tumor patients is how their tumor looks under the microscope (histology). The majority of Wilms tumor patients have favorable histology tumors that respond to surgery, chemotherapy, and radiation. These tumors are usually called triphasic tumors because they contain three main cell types--epithelial cells, blastemal cells, and stromal cells.

Background

Pediatric solid tumors arise during the development of diverse tissues such as bone, muscle and adrenal gland. The tumor cells maintain many features of the normal tissue where they develop including the developmental hierarchy. As a result, cells within an individual patient's tumor has features of different developmental stages. This tumor heterogeneity is important because, in some patients, a subset of those cells survive treatment and contribute to disease relapse which is often fatal.

Osteosarcoma is a malignant and aggressive bone tumor, which primarily affects children and adolescents, with a high rate of recurrence and metastasis. The standard treatment for children with osteosarcoma is a combination of chemotherapy and surgical removal of the tumor, often requiring partial limb amputation. The 5-year survival rate for children with metastases at diagnosis is less than 30%, and sadly, there have been no new therapies for treating osteosarcoma in over 30 years.