Neuroblastoma
Overview
Plain-Language Overview
Neuroblastoma is a type of cancer that most commonly affects young children and arises from immature nerve cells in the adrenal glands or along the spine. It involves the nervous system, specifically the sympathetic nervous system, which controls involuntary body functions. This cancer can cause a variety of symptoms depending on its location, such as a visible lump, pain, or problems with movement. It may also produce substances that affect the body’s hormones, leading to symptoms like high blood pressure or diarrhea. Early detection is important because neuroblastoma can spread to other parts of the body, impacting overall health and requiring specialized treatment.
Clinical Definition
Neuroblastoma is a malignant tumor originating from neural crest cells that form the sympathetic nervous system, most commonly arising in the adrenal medulla or paraspinal ganglia. It is the most common extracranial solid tumor in children and typically presents before age 5. The tumor is characterized by small, round, blue cells on histology and often shows amplification of the MYCN oncogene, which correlates with aggressive disease. Clinical significance includes its potential for widespread metastasis to bone marrow, liver, and lymph nodes, and its ability to produce catecholamines leading to systemic symptoms. Diagnosis and staging are critical for prognosis and guide multimodal treatment including surgery, chemotherapy, and radiation. The disease exhibits heterogeneous behavior, ranging from spontaneous regression to aggressive progression.