Case 131 - Discussion

Uploaded: 2007-10-26, Updated: 2007-10-26

 

Ganglioneuroma of the Adrenal Gland

The Key Features

  • Fully differentiated, composed of mature Schwann cells, ganglion cells, fibrous tissue, and nerve fibers.

  • Arise de novo or result from the maturation of a ganglioneuroblastoma or neuroblastoma.

  • Need to carefully examine the entire specimen and look for the immature component.

Clinical Futures

 
  • Belong to the family of neuroblastic tumors that include ganglioneuromas, ganglioneuroblastomas and neuroblastomas.

  • Neuroblastic tumors are of the sympathetic nervous system that originate from neural crest sympathogonia.

  • Tumors of adolescents and young adults (40-60%), mean age 7 years.

  • Fully mature benign tumors. Mostly in posterior mediastinum, followed by the retroperitoneum (especially the presacral space), the adrenal gland, head, and neck.

  • Ganglioneuroma can arise de novo or result from the maturation of a ganglioneuroblastoma or neuroblastoma into a ganglioneuroma. They may also develop within a neuroblastoma treated with chemotherapy. Metastases in these tumors are exceedingly rare. Metastasis is thought to be the end result of matured ganglioneuroblastoma or neuroblastoma metastases rather than true ganglioneuroma metastases.

  • Usually asymptomatic regardless of their size. These tumors may be hormonally active, and hypertension, diarrhea, flushing, and virilization may occur as a result the secretion of catecholamine, vasoactive intestinal polypeptide, or androgenic hormone. As many as 37% of ganglioneuromas secrete catecholamines.

  • In rare cases, von Recklinghausen disease, Beckwith-Wiedemann syndrome, Hirschsprung disease, central failure of ventilation, and DiGeorge syndrome are associated with ganglioneuroma and ganglioneuroblastoma.

Gross Findings

 
  • Well-circumscribed, smooth or lobulated masses,  pseudocapsule.

  • Average 8 cm in size.  They are firm to the touch and have a light color ranging from white to yellow. Internally, the tumor may have a whorled appearance with trabeculae, gray-white to tan-white.

  • Calcification in 41% cases.

Microscopic Findings

 
  • Contain mature Schwann cells, ganglion cells, fibrous tissue, and nerve fibers. These tumors have no immature elements (such as neuroblasts), atypia, mitotic figures, intermediate cells, or necrosis. The presence of any these tissue characteristics excludes the diagnosis of ganglioneurom, and automatically makes the tumor a ganglioneuroblastoma or neuroblastoma.

Prognosis

 
  • Overall, patients with ganglioneuroma have a favorable prognosis.

Reference

 
  • Rha SE, Byun JY, Jung SE, et al: Neurogenic tumors in the abdomen: tumor types and imaging characteristics. Radiographics 2003 Jan-Feb; 23(1): 29-43

  • AFIP, Tumor of the adrenal gland and extra-adrenal paraganglia, 3rd series

  • http://www.webpathology.com/ (For gross and slides of ganglioneuroma)

  • http://www.emedicine.com/radio/topic293.htm

  • http://www.emedicine.com/RADIO/topic472.htm

  • http://enjoypath.com/sp/case-37/case-37.htm