%0 Journal Article
%T The emerging molecular pathogenesis of neuroblastoma: implications for improved risk assessment and targeted therapy
%A Nadine Van Roy
%A Katleen De Preter
%A Jasmien Hoebeeck
%A Tom Van Maerken
%A Filip Pattyn
%A Pieter Mestdagh
%A Jo£żlle Vermeulen
%A Jo Vandesompele
%A Frank Speleman
%J Genome Medicine
%D 2009
%I BioMed Central
%R 10.1186/gm74
%X Neuroblastoma (NB) is the most common extra-cranial solid tumor of childhood. It has an incidence of 1 to 5 per million children per year and it is responsible for approximately 15% of all childhood cancer mortality. NBs originate from immature sympathetic nervous system cells, the so-called neuroblasts. Most NBs (90%) are diagnosed before the age of 5 years, and the median age of occurrence is approximately 22 months [1]. The vast majority of tumors arise sporadically, although some familial cases are described. Most of the tumors are found in the abdomen (65%), often in the adrenal medulla, or elsewhere in the human body where sympathetic nervous system components are present [2]. NBs belong to the subgroup of small round blue cell tumors and can often pose a challenge to the pathologist because of their similarities with lymphomas, rhabdomyosarcomas, the Ewing family of tumors and desmoplastic round cell tumors.NB tumors are divided into different stages according to the localization and extension of the primary tumor and the absence or presence of distant metastases. The International Neuroblastoma Risk Grouping Staging System takes into account the extent of disease at diagnosis as well as risk factors and stages defined using imaging to classify NBs from patients from all over the world in a uniform manner [3,4]. Metastatic tumors (stage M) have a dismal prognosis, whereas patients with locoregional tumors (L1 and L2) usually have an excellent outcome. Stage Ms tumors (where 's' stands for special and meta-static disease is confined to skin, liver and/or bone marrow) are characterized by spontaneous regression or differentiation even without any form of therapy. Although localized disease can be treated by surgery alone, the standard therapy for patients with metastatic disease usually comprises intensive induction chemotherapy, local surgery and myeloablative chemotherapy followed by autologous stem cell transplant, external radiotherapy and retinoic acid tre
%U http://genomemedicine.com/content/1/7/74