Purpose or Case Report: Neuroblastoma is recognized as having a broad spectrum of clinical behavior in children diagnosed with the disease. Some tumors exhibit aggressive characteristics and portend a poor prognosis, while others that appear aggressive spontaneously regress. Accurately identifying high risk neuroblastoma is important in determining which patients will benefit most from intense chemotherapy, which unfortunately carries a risk of significant adverse effects later in life. Historically this has been difficult, as the classification schemes vary in different parts of the world, limiting the ability to pool data and improve prognostication. In recent years, efforts among experts around the globe have led to a consensus on the most evidenced based approach to staging. The aim of this educational exhibit is to describe the new standardized language for radiology reports, which will contribute to accurate staging and improve treatment for patients with neuroblastoma. Additionally, key imaging features highlighting image defined risk factors will be presented.
Methods & Materials: The International Neuroblastoma Risk Group Task Force is a group of leading pediatric oncologists, radiologists, researchers and statisticians from around the world. In 2009, the group published a new classification system based on data collected from a large cohort of patients, representing a consensus among global experts in the field.
Results: The INRG Staging System (INRGSS) was introduced to replace the now decades-old International Neuroblastoma Staging System. The new system shifts away from surgicopathologic classification to describe tumors according to radiologic findings. In addition to differentiating local from metastatic disease, the new system further delineates local disease based on the presence or absence of “image defined risk factors” (IDRF). With this, there is a recommendation for specific terminology to be used in order to accurately characterize an IDRF. These include the words: separation, contact, encasement, compression, infiltration, and invasion.
Conclusions: As the care for Neuroblastoma patients evolves, radiologists must continue to provide value in the multidisciplinary efforts to advance the most up to date approach to staging of the disease. Now more than ever, the use of accurate and uniform language in reports will have a significant impact on the treatment of these patients.