Disease Information for Neuroblastoma: Definition

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  • A common neoplasm of early childhood arising from neural crest cells in the sympathetic nervous system, and characterized by diverse clinical behavior, ranging from spontaneous remission to rapid metastatic progression and death; This tumor is the most common intraabdominal malignacy of childhood, but it may also arise from thorax, neck, or rarely occur in the central nervous system; Histologic features include uniform round cells with hyperchromatic nuclei arranged in nests and separated by fibrovascular septa; Neuroblastomas may be associated with the opsoclonus-myoclonus syndrome; (From DeVita et al, Cancer: Principles and Practice of Oncology, 5th ed, pp2099-2101; Curr Opin Oncol 1998 Jan;10(1):43-51)------------------------------ Neuroblastomas are neural tumors and abnormalities that have cell origins from the neural crest; ICD-9CM CODES; 194_0 Neuroblastoma, unspecified site; 8% of all solid tumors of childhood;Male >female ratio Childhood; Infants and children; N-myc protooncogene; loss of short arm chromosome 1 (1p36) found in some children; CLINICAL: Mass in abdomen, neck, or chest; Spinal cord compression; Dancing eyes; Chronic pain; Ecchymosis around eyes; Weight loss; Fever; DIFFERENTIAL: Other childhood tumors; Wilms’ tumor; Diagnosis: Biopsy of tumor; Urinary catecholamines; Bone marrow aspiration; CT scan or MRI abdomen/chest;hope for recovery with aggressive treatment ;Surgery; Multiagent chemotherapy (eg, cyclophosphamide, vincristine, dacarbazine, melphalan, doxorubicin, cisplatin); Radiotherapy ;either death or cure ;Overall survival is >40%;Survival in stage I is >95%, whereas survival in stage II is <20% [Evans classification];[Ferris Clinical advisor 2005]

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