Principles of Surgery

Obtain tissue for diagnosis. Incisional or core biopsy only is recommended for patients with L2 disease,[5][Level of evidence C1] and an up-front resection should generally not be attempted. For more information about image-defined risk factors (IDRFs), see the International Neuroblastoma Risk Group Staging System (INRGSS) section.

Obtain tissue for diagnosis. Incisional or core biopsy only is recommended for patients with L2 disease,[5][Level of evidence C1] and an up-front resection should generally not be attempted. For more information about image-defined risk factors (IDRFs), see the International Neuroblastoma Risk Group Staging System (INRGSS) section.

Either incisional biopsy or percutaneous core needle biopsy are acceptable for patients with L2 disease. In a multi-institutional retrospective study, there was no significant difference in the ability to accurately obtain a primary diagnosis by percutaneous core needle biopsy compared with incisional biopsy (95.7% vs. 98.9%, P = .314) or determine MYCN copy number (92.4% vs. 97.8%; P = .111). The yield for loss of heterozygosity and tumor ploidy was lower with percutaneous core needle biopsy (dependent on the number of cores/volume of tissue obtained) than with incisional biopsy (56.1% vs. 90.9%, P < .05; and 58.0% vs. 88.5%, P < .05). Complications did not differ between the groups.[6][Level of evidence C1]

Near-total or total resection (80%–90%) of the primary tumor is recommended based on stage.

Near-total or total resection (80%–90%) of the primary tumor is recommended based on stage.

This is standard for patients with low-risk (excluding prenatally diagnosed infants who are candidates for observation) and intermediate-risk disease. In patients with L1 tumors (defined as having no image-defined surgical risk factors), the tumors are resectable with low risk of nephrectomy or life-threatening complications. Unilateral adrenal gland primary tumors, thoracic L1 disease, or neck L1 disease should be resected up front (per the surgeons’ discretion).[5][Level of evidence C1]

Near-total or total resection (80%–90%) of the primary tumor is recommended based on stage.

Minimally invasive surgery may be considered in highly selected patients with neuroblastoma if it is performed by a pediatric surgical oncologist who has expertise using this technique.[7][Level of evidence C1]; [8,9]