Abstract
Background
Ganglioneuroma is a rare tumour of neural crest origin, which arises from maturation of a neuroblastoma. While previously considered to be non-functioning, they are now known to be frequently endocrinologically active.
Aims and Methods
We report a case of a massive retroperitoneal ganglioneuroma presenting with small bowel obstruction in an adult, 18 years after initial diagnosis. Urinary dopamine levels were elevated, but other catecholamines were within normal limits. This is the first report in the English-language literature of a retroperitoneal ganglioneuroma presenting with or causing intestinal obstruction. We also review the metabolic, radiological, and histological features of these tumours. Relevant publications were identified from a Medline search using the MeSH headings ‘ganglioneuroma’, ‘retroperitoneal neoplasms’ and ‘intestinal obstruction’, and also from the reference lists of retrieved articles.
Conclusions
Ganglioneuroma can grow to a massive size and present in a varied manner. It should be included in the differential diagnosis of any large retroperitoneal or mediastinal mass, includingthose causing bowel obstruction.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Geoerger B, Hero B, Harms D, Grebe J, Scheidhauer K, Berthold F. Metabolic activity and clinical features of primary ganglioneuromas.Cancer 2001; 91(10):1905–13.
de Lellis RA. The adrenal glands. In: Sternberg S, editor. Diagnostic surgical pathology. 3rd ed. Philadelphia: Lippincott, Williams & Wilkins; 1999. p. 608.
Miller RW, Young JL, Novakonic B. Childhood cancer.Cancer 1994; 75: 395–405.
Lack EE. Atlas of tumor pathology: tumors of the adrenal gland and extra-adrenal paraganglia, fasc 19, ser 3. Washington, DC: Armed Forces Institute of Pathology; 1997, p412.
Hamilton JR Koop E. Ganglioneuromas in children.Surg Cynecol Obstet 1965; 121: 803–12.
Lopez IB, Schwartz A. Neuroblastoma.Pediatr Clin North Am 1985; 32: 755–78.
Shimada H, Ambros IM, Dehner LR Hata J-I, Joshi W, Roald B. Terminology and morphologic criteria of neuroblastic tumours.Cancer 1999; 86: 349–63.
Kulkarni AV, Bilbao JM, Cusimano MD, Muller PJ. Malignant transformation of ganglioneuroma into spinal neuroblastoma in an adult.J Neurosurg 1998; 88(2): 324–7.
Moukheiber AK, Nicollas R, Roman S, Coze C, Triglia J-M. Primary pediatric neuroblastic tumors of the neck.Int J Pediatr Otorhinolaryngol 2001; 60: 155–61.
Shimada H, Ambros IM, Dehner LP et al. The International Neuroblastoma Pathology Classification (the Shimada system). Cancer 1999; 86: 364–72.
Lucas K, Gula MJ, Knisely AS, Virgi MA, Wollman M, Blatt J. Catecholamine metabolites in ganglioneuroma.Med Pediatr Oncol 1994; 22(4): 240–3.
Quak SH, Prabhakaran K, Kwok R, O’Reilly AP. Vasoactive intestinal peptide secreting tumours in children: a case report with literature review.Aust Paediatr J 1988; 24(1): 55–8.
Scherer A, Niehues T, Engelbrecht V, Modder U. Imaging diagnosis of retroperitoneal ganglioneuroma in childhood.Pediatr Radiol 2001; 31: 106–10.
Radin R, David CL, Goldfarb H, Francis IR. Adrenal and extra-adrenal retroperitoneal ganglioneuroma: imaging findings in 13 adults.Radiology 1976; 202: 703–7.
de Lellis RA. The adrenal glands. In: Sternberg S, editor. Diagnostic surgical pathology. 3rd ed. Philadelphia: Lippincott, Williams & Wilkins; 1999. p. 613.
Hoff RC, San Diego AC. Ganglioneuroma of the ileocaecal valve.Arch Pathol Lab Med 1972; 93: 549–51.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Cronin, E.M.P., Coffey, J.C., Herlihy, D. et al. Massive retroperitoneal ganglioneuroma presenting with small bowel obstruction 18 years following initial diagnosis. Ir J Med Sci 174, 63–66 (2005). https://doi.org/10.1007/BF03169133
Issue Date:
DOI: https://doi.org/10.1007/BF03169133