Demographics
- 3rd most common malignant tumor in children (~10% of pediatric cancers), especially common in children < 4 years old.
- Leukemia is the most common malignancy of any kind.
- Brain tumor is the most common malignant solid tumor.
- Neuroblastoma is the 2nd most common malignant solid tumor.
Pathology
- Originates from neural crest cells anywhere along the sympathetic chain (sympaticoadrenal lineage).
Location
- Most commonly arises from the abdomen (~65%). Typically near the kidney (adjacent to the aorta) or in the adrenal medulla (see: adrenal gland anatomy, adrenal gland physiology).
- Can also arise from the chest (~ 15%) or rarely from the neck or pelvis but can occur anywhere along the sympathetic chain.
Presentation
- Most often presents with abdominal distention with a firm, irregular mass.
- Opsoclonus-myoclonus is a rare presentation of neuroblastoma. When opsoclonus-myoclonus ataxia syndrome presents in children, it is often (~50%) due to neuroblastoma (as opposed to in adults where it's typically due to paraneoplastic or parainfectious causes).
- The syndrome involves opsoclonus (conjugate, chaotic eye movements in any direction), myoclonus (limb jerks (non-seizure)), and ataxia (large movement incoordination), and also causes personality changes, such as irritability and other behavioral changes, as well as sleep disturbance.
Prognosis
- Prognosis is highly variable but typically worsens with increasing age (especially older than 18 months), increasing mitotic-karyorrhexis index (MKI), poor differentiation of tumor histology, and amplification of the MYCN proto-oncogene.
MYCN Proto-oncogene Amplification
- Amplification of the MYCN proto-oncogene occurs in ~ 25–33% of patients and generally results in a poor prognosis (aggressive tumor behavior).
Homer Wright Rosettes
- Homer Wright rosettes were originally described in neuroblastoma tumor; they refer to the histological finding a group of cells that cluster around a lumen filled with central neuropil (fiber meshwork). See rosettes and pseudorosettes for details regarding this histopathology.
Select Related Tumors
- Wilms tumor
- Pheochromocytoma
References
- Berger, Michael, and Dietrich Von Schweinitz. “Therapeutic Innovations for Targeting Childhood Neuroblastoma: Implications of the Neurokinin-1 Receptor System.” Anticancer Research 37, no. 11 (November 1, 2017): 5911–18.
- Brisse, Hervé J., Thomas Blanc, Gudrun Schleiermacher, Véronique Mosseri, Pascale Philippe-Chomette, Isabelle Janoueix-Lerosey, Gaelle Pierron, et al. “Radiogenomics of Neuroblastomas: Relationships between Imaging Phenotypes, Tumor Genomic Profile and Survival.” Edited by Michael Baudis. PLOS ONE 12, no. 9 (September 25, 2017): e0185190. https://doi.org/10.1371/journal.pone.0185190.
- Jiang, Manrong, Jennifer Stanke, and Jill M. Lahti. “The Connections Between Neural Crest Development and Neuroblastoma.” In Current Topics in Developmental Biology, 94:77–127. Elsevier, 2011. https://doi.org/10.1016/B978-0-12-380916-2.00004-8.
- Meena, Jagdish P., Rachna Seth, Biswaroop Chakrabarty, Sheffali Gulati, Sandeep Agrawala, and Priyanka Naranje. “Neuroblastoma Presenting as Opsoclonus-Myoclonus: A Series of Six Cases and Review of Literature.” Journal of Pediatric Neurosciences 11, no. 4 (2016): 373–77. https://doi.org/10.4103/1817-1745.199462.
- “Neuroblastoma - Symptoms and Causes.” Mayo Clinic. Accessed July 5, 2018. http://www.mayoclinic.org/diseases-conditions/neuroblastoma/symptoms-causes/syc-20351017.
Image Reference
- Homer Wright rosette
- Pathology, The Armed Forces Institute of. English: ADRENAL GLAND: HOMER WRIGHT ROSETTES IN A NEUROBLASTOMA These Homer Wright Rosettes Appear as Pale Zones with Fibrillar Matrix Corresponding to Neuritic Cell Processes. https://commons.wikimedia.org/wiki/File:Rosette.jpg.