Neuroblastoma and Ganglioneuroma

NEUROBLASTOMA AND GANGLIONEUROMA

Neuroblastoma is a malignant tumor of neural crest origin that may arise anywhere along the sympathetic ganglia or within the adrenal medulla. The median age of diagnosis is 2 years; however occurrence is skewed toward younger children, with nearly 35% of cases occurring under 1 year of age and the remainder under 10 years of age. Seventy-five percent of neuroblastomas originate within the abdomen or pelvis, and half of these occur within the adrenal medulla, whereas 20% originate within the posterior mediastinum and 5% within the neck [1]. Neuroblastomas and ganglioneuromas are tumors of the primitive neuroblast cells from the sympathetic nervous system in ganglia and the adrenal medulla. They may represent a continuum of neuronal maturation and are the most common malignancy found in children, representing ~7–10% of all childhood cancers. For neuroblastomas, the median age of diagnosis is 18 months, with approx. Over 50% have metastatic disease at presentation and over 90% have elevated catecholamines, but only rarely are there presenting emergency symptoms because of the excess catecholamines similar to those seen with phaeochromocytoma, such as hypertensive encephalopathy or cardiac failure. Subjects may also have paraneoplastic phenomena such as secretory diarrhoea from vasoactive intestinal peptide. Chromogranin A elevation parallels neuroblastoma disease stage [2, 3]. Because of their more mature ganglion cells which are histologically benign, ganglioneuromas are often metabolically inactive and asymptomatic. They are found incidentally or with compressive symptoms mostly in the posterior mediastinum or retroperitoneum. In a case series of 49 pediatric and young adult subjects, diagnosed at a mean age of 79 months (aged 18 months to 26 years), lesions showed a propensity towards extra-adrenal locales (79% vs. 21%) [2, 4]. Approx. 40% of subjects had evidence of catecholamine excess. Although some metabolic activity has been detected in a portion of these tumors, either positive scintigraphy scans or elevated catecholamine levels in the plasma and urine, this was not associated with malignancy or recurrence of tumor [2].

  1. Alexander F. Neuroblastoma. Urol Clin North Am. 2000 Aug;27(3):383-92, vii.
  2. Fung MM, Viveros OH, O'Connor DT. Diseases of the adrenal medulla. Acta Physiol (Oxf). 2008 Feb;192(2):325-35.
  3. Hsiao RJ, Seeger RC, Yu AL, O’Connor DT. Chromogranin A in children with neuroblastoma. Serum concentration parallels disease stage and predicts survival. J Clin Invest 1990c;85:1555–1559
  4. Geoerger B, Hero B, Harms D, Grebe J, Scheldhauer K, Berthold F. Metabolic activity and clinical features of primary ganglioneuromas. Cancer 2001;91:1905–1913