Molecular genetics of neuroblastoma and the implications for clinical management: A review of the MSKCC experience Journal Article


Authors: Mora, J.; Gerald, W. L.; Qin, J.; Cheung, N. K. V.
Article Title: Molecular genetics of neuroblastoma and the implications for clinical management: A review of the MSKCC experience
Abstract: Neuroblastoma (NB) is a biological, genetic, and morphological heterogeneous neoplasm and demonstrates diverse clinical behavior. There exist at least three clinical patterns of NB: A) spontaneously regressing widespread disease; B) not metastatic local-regional disease, and C) metastatic disease (stage 4), frequently with lethal consequences. Patients with non-stage 4 NB are expected to survive even without medical treatment whereas stage 4 patients have an overall survival rate of 20% despite multimodality therapy protocols. The clinical management of patients with NB is therefore challenged by the objective identification of cases in which noncytotoxic approaches can be safely taken. Experience in the last decade at Memorial Sloan-Kettering Cancer Center supports the hypothesis that the natural history of disease defines relevant clinical groups of NB and has distinct molecular genetic profiles allowing therapeutic approaches tailored for each group. Here we review the natural history and clinicobiological features of 113 NB cases managed uniformly in our institution in an attempt to characterize useful genetic markers to support the decision making of noncytotoxic versus cytotoxic approaches for each category of NB.
Keywords: cancer survival; clinical feature; disease course; review; cytotoxic agent; multimodality cancer therapy; treatment planning; united states; cancer staging; molecular genetics; neoplasm staging; medical decision making; metastasis; cancer regression; neuroblastoma; molecular biology; genetic marker; disease activity; metastatic neuroblastoma; disseminated neuroblastoma; humans; prognosis; human; priority journal; local-regional neuroblastoma; noncytotoxic therapy
Journal Title: The Oncologist
Volume: 6
Issue: 3
ISSN: 1083-7159
Publisher: Oxford University Press  
Date Published: 2001-06-01
Start Page: 263
End Page: 268
Language: English
DOI: 10.1634/theoncologist.6-3-263
PUBMED: 11423673
PROVIDER: scopus
DOI/URL:
Notes: Export Date: 21 May 2015 -- Source: Scopus
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MSK Authors
  1. Nai-Kong Cheung
    628 Cheung
  2. Jaime Mora
    25 Mora
  3. Jing Qin
    86 Qin
  4. William L Gerald
    375 Gerald