When is surgery appropriate for neuroblastoma?

  Neuroblastoma is highly malignant and often locally manifests extensive metastasis and fusion of regional lymph nodes, or involves vital organs and vascular nerves, making surgery difficult. In clinical practice, we will see such patients who underwent stage I surgery at the initial diagnosis, unfortunately with more residuals or with serious postoperative complications, which is obviously inappropriate. Chemotherapy first, reducing the scope of the tumor and postponing surgery should be the appropriate choice. The child’s family should ask: How should we choose to operate in one stage or postpone the surgery?  In 2009, the International Neuroblastoma Collaborative Group published imaging-based defined risk factors (IDRFs), which are intended to guide the timing of surgery and reduce surgery-related complications. In first diagnosed neuroblastoma, primary and metastatic lesions are first evaluated using CT and/or MRI, I-123 MIBG, Tc-99m MDP bone scan imaging techniques, and if one or more of the risk factors in the IDRFs are present surgery should be postponed and treated surgically after reducing the risk of surgical complications with chemotherapy.The risk factors are defined according to different sites as follows.  (1) Unilateral lesion extending into two intervening compartments: cervical-thoracic; thoracic-abdominal; abdominal-pelvic.  (2) Cervical: tumor encircling the carotid artery, and/or vertebral artery, and/or internal jugular vein; tumor extending to the skull base; tumor compressing the trachea.  (3) Cervicothoracic junction: tumor encircles the brachial plexus nerve root; tumor encircles the subclavian vessels, and/or vertebral artery, and/or carotid artery; tumor compresses the trachea.  (4) Thoracic: tumor encircling thoracic aorta and/or major branches; tumor compressing trachea and/or main bronchus; low posterior mediastinal tumor, invading the rib-spine junction between T9 and T12 (because of the vulnerability of Adamkiewicz artery here).  (5) Thoracoabdominal junction: tumor encircling the aorta and/or vena cava.  (6) Abdominal/pelvic: tumor encroaches on the hepatoportal and/or hepatoduodenal ligaments; tumor encircles a branch of the superior mesenteric artery at the mesenteric root; tumor encircles the celiac trunk and/or the beginning of the superior mesenteric artery; tumor encroaches on one or both renal tissues; tumor encircles the abdominal aorta and/or inferior vena cava; tumor encircles the iliac vessels; pelvic tumor crosses the sciatic notch.  (7) Intraspinal extension: more than 1/3 of the spinal canal in the axial plane is invaded by the tumor, and/or the circumspinal soft meningeal space is lost, and/or the spinal cord signal is abnormal.  (8) Involvement of adjacent organs/tissues: pericardium, diaphragm, kidney, liver, pancreas-duodenum, and mesentery.  The following should be documented, but not as IDRFs: multiple primary foci; pleural fluid with/without malignant cells; ascites with/without malignant cells.