pediatrics
Neuroblastoma
Introduction
Neuroblastoma is a cancer arising in the adrenal gland or less often from the extra-adrenal sympathetic chain, including in the retroperitoneum, chest, and neck. Diagnosis is confirmed by biopsy. Treatment may include surgical resection, chemotherapy, radiation therapy, high-dose chemotherapy with stem cell transplantation, cis-retinoic acid, and immunotherapy. Neuroblastoma is the most common Overview of Pediatric Cancer and is the most common solid tumor, other than brain tumors, in children. It accounts for 7 to 8% of all childhood cancers ( Neuroblastomas are immature, undifferentiated, malignant tumors. Ganglioneuroblastomas are intermediate tumors, and ganglioneuromas are fully differentiated, benign variants of neuroblastoma; collectively, these are referred to as neural crest tumors. Most neuroblastomas produce catecholamines, which can be detected as elevated levels of urinary catecholamine breakdown products. Neuroblastomas do not typically cause severe hypertension because these tumors do not usually secreteepinephrine. Neuroblastomas may begin in the abdomen (about 65%), thorax (15 to 20%), neck, pelvis, or other sites. Neuroblastoma occurs very rarely as a primary Overview of Central Nervous System Tumors in Children . About 40 to 50% of children have localized or regional disease at diagnosis; 50 to 60% have metastases at diagnosis. Neuroblastoma may metastasize to bone marrow, bone, liver, lymph nodes, or, less commonly, skin or brain. Bone marrow metastases may cause anemia and/or thrombocytopenia. Anemia also occasionally occurs when bleeding into these highly vascular tumors causes a rapid drop in hemoglobin. Most neuroblastomas occur spontaneously, but 1 to 2% appear to be inherited. Some markers (eg,MYCNoncogene amplification, DNA index, segmental chromosomal aberrations, histopathology) correlate with progression and prognosis.MYCNamplification occurs in about 20% of neuroblastoma cases and is associated with advanced disease and unfavorable biology. Neuroblastoma is the most common cancer among infants. 