Neuroblastoma is a type of cancer that affects the sympathetic nervous system in young children and is often difficult to treat, especially when the tumor cells carry multiple copies of the MYCN gene.

MYCN gene amplification critically drives neuroblastoma aggressiveness and poor outcomes, necessitating precise preoperative identification to guide risk-adapted therapies. Current invasive detection ...

Oncogenic MYCN drives aggressive disease in many cancers including neuroblastoma (NB). Metabolic reprogramming is essential to support cancer cell homeostasis and survival under nutrient- and oxygen-deprived conditions. MYCN directly reprograms many nodes of tumor-intrinsic metabolism, which have significant repercussions on the cells of the tumor microenvironment (TME), resulting in complex intercellular metabolic circuits that contribute to the immunosuppressive microenvironment of NB. These metabolic circuits are also regulated by the organismal and cellular circadian clock and host diet to further impact the TME and NB oncogenesis. This review discusses the mechanisms by which MYCN regulates the metabolic crosstalk between tumor, TME, and host, and provides evidence that therapeutic targeting of MYCN-reprogrammed metabolism can improve patient outcomes.

Oncogenic MYCN drives aggressive disease in many cancers including neuroblastoma (NB). Metabolic reprogramming is essential to support cancer cell homeostasis and survival under nutrient- and oxygen-deprived conditions. MYCN directly reprograms many nodes of tumor-intrinsic metabolism, which have significant repercussions on the cells of the tumor microenvironment (TME), resulting in complex intercellular metabolic circuits that contribute to the immunosuppressive microenvironment of NB. These metabolic circuits are also regulated by the organismal and cellular circadian clock and host diet to further impact the TME and NB oncogenesis. This review discusses the mechanisms by which MYCN regulates the metabolic crosstalk between tumor, TME, and host, and provides evidence that therapeutic targeting of MYCN-reprogrammed metabolism can improve patient outcomes.

MYCN amplification within the tumor has emerged as an important factor in the oncogenesis of a subset of retinoblastomas, most of which are caused by pathogenic variants in the RB1 gene. We present the case of a 3-month-old girl with unilateral cavitary retinoblastoma that initially responded well to intra-arterial chemotherapy but later recurred. After enucleation, the tumor was found to exhibit MYCN amplification, but there were no RB1 variants. Retinoblastoma tumors with MYCN amplification are known to carry a poor prognosis and often require enucleation.

Neuroblastoma can be a particularly insidious cancer. In about half of all cases, tumors regress, even without therapy.