18 - Neuroblastoma

Abstract

This chapter discusses the pathology, incidence, clinical features, symptoms, diagnosis, and treatments of neuroblastoma. Neuroblastomas are tumors of sympathetic nerve tissue, and a tumor mass can occur anywhere along the sympathetic neural pathway. It originates from primordial neural crest cells that normally give rise to adrenal medulla and the sympathetic ganglia. It has a more varied clinical presentation of primary or metastatic disease and paraneoplastic syndromes. It is the most common extracranial solid tumor in infancy and can be diagnosed immediately after birth with metastases to the placenta. Clinical manifestations depend on the anatomic site of the primary tumor: head and neck, orbit and eye, chest, abdomen, pelvis, paraspinal area, lymph nodes, bone, lung, and brain. Nonspecific constitutional symptoms include lethargy, anorexia, pallor, weight loss, abdominal pain, weakness, and irritability. Two major recommended criteria from the International Neuroblastoma Staging System (INSS) conference for a diagnosis of neuroblastoma are as follows: unequivocal pathologic diagnosis is made from tumor tissue by light microscopy; bone marrow aspirate or trephine biopsy contains unequivocal tumor cells and increased urine or metabolites. The proposed treatment modalities used in neuroblastoma are surgery, chemotherapy, and radiotherapy. The role of each is determined by the patient's age and stage and biologic features of the tumor.