TY - CHAP M1 - Book, Section TI - Surgical Diseases of the Newborn: Retroperitoneal Tumors A1 - Gomella, Tricia Lacy A1 - Eyal, Fabien G. A1 - Bany-Mohammed, Fayez PY - 2020 T2 - Gomella's Neonatology: Management, Procedures, On-Call Problems, Diseases, and Drugs, 8e AB - DefinitionPrimitive malignant neoplasm that arises from neural crest tissue.Usually located in adrenal gland but can occur anywhere neural crest cells migrate.Most common extracranial solid malignancy of childhood.Incidence of 1 per 100,000 children in the United States.Clinical presentation. Typically presents as firm, fixed, irregular mass extending obliquely from costal margin, occasionally across midline, and into lower abdomen.DiagnosisLaboratory studiesTwenty-four–hour urine collection should be analyzed for vanillylmandelic acid and other catecholamine metabolites.Elevated lactate dehydrogenase is associated with poor prognosis.Radiologic studiesPlain abdominal radiograph may reveal calcifications within tumor.Computed tomography scan typically shows extrinsic compression and inferolateral displacement of kidney.Metastatic evaluation involves bone marrow aspiration and biopsy, bone scan, chest radiograph, and chest computed tomography (CT) scan.ManagementTreatment based on tumor stage. Two staging systems are used:International Neuroblastoma Risk Group Staging System (INRGSS). Uses image-defined risk factors to determine suitability for surgery.International Neuroblastoma Staging System (INSS). Takes into account the surgical staging.Complete surgical resection remains best hope for cure.Infants with type 4S disease (“special” neuroblastoma) are typically younger than 1 year old and may undergo spontaneous regression. Planned therapy should consider this well-recognized, but poorly understood, fact.Advanced tumors require multimodality therapy with surgery, radiation, and chemotherapy, but this is uncommon in neonates. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1168358393 ER -