the emerging molecular pathogenesis of neuroblastoma implications for improved risk assessment and targeted therapy新兴的分子发病机制对提高风险评估和靶向治疗神经母细胞瘤的影响.pdf

Review The emerging molecular pathogenesis of neuroblastoma: implications for improved risk assessment and targeted therapy Nadine Van Roy, Katleen De Preter, Jasmien Hoebeeck, Tom Van Maerken, Filip Pattyn, Pieter Mestdagh, Joëlle Vermeulen, Jo Vandesompele and Frank Speleman Address: Center for Medical Genetics, Ghent University Hospital, De Pintelaan 185, B-9000 Ghent, Belgium. Correspondence: Frank Speleman. Email: franki.speleman@ugent.be described. Most of the tumors are found in the abdomen Abstract Neuroblastoma is one of the most common solid tumors of (65%), often in the adrenal medulla, or elsewhere in the childhood, arising from immature sympathetic nervous system human body where sympathetic nervous system compo- cells. The clinical course of patients with neuroblastoma is nents are present [2]. NBs belong to the subgroup of small highly variable, ranging from spontaneous regression to wide- round blue cell tumors and can often pose a challenge to spread metastatic disease. Although the outcome for children the pathologist because of their similarities with lym- with cancer has improved considerably during the past decades, phomas, rhabdomyosarcomas, the Ewing family of tumors the prognosis of children with aggressive neuroblastoma and desmoplastic round cell tumors. remains dismal. The clinical heterogeneity of neuroblastoma mirrors the biological and genetic heterogeneity of these tumors. Ploidy and MYCN amplification have been used as genetic NB tumors are divided into different stages according to markers for risk stratificati