Pictorial Urology

232 Urology Journal    Vol 5    No 4    Autumn 2008

Retroperitoneal Ganglioneuroma
Urol J. 2008;5:232. 

www.uj.unrc.ir

A 45-year-old man was diagnosed with retroperitoneal ganglioneuroma. The tumor was a large encapsulated 
mass of firm consistency with a homogeneous, solid, grayish white-cut surface, having a focally edematous 
appearance (Figures, Top). Microscopically, the overall appearance of ganglioneuroma resembled that of a 
neurofibroma (it is designated as a schwannian steroma-dominant tumor) except for the presence of numerous 
collections of abnormal but fully mature ganglion cells, often with more than one nucleus (Figures, Bottom).

Neuroblastic tumors constitute 4 basic morphologic categories: neuroblastoma; ganglioneuroblastoma, 
intermixed; ganglioneuroma; and ganglioneuroblastoma, nodular.(1) Ganglioneuromas are fully 
differentiated and invariably benign, mostly seen in the elderly. They can be multiple and/or associated 
with other independent types of neural/neuroendocrine neoplasms. Their most common location is 
the posterior mediastinum and the retroperitoneum. Although catecholamine synthesis is an almost 
constant feature of all the neurogenic tumors, ganglioneuromas rarely lead to symptoms.(1) Thorough 
microscopic examination is crucial; areas of different colors or consistency are particularly suspicious 
for harboring less differentiated foci, namely ganglioneuroblastoma. If there is a minor component of 
scattered collections of differentiating neuroblasts and/or maturing ganglion cells, the tumor is named 
maturing subtype,(2) which blends with the intermixed subtype of ganglioneuroblastoma, from which it 
differs in that the immature foci do not form distinct microscopic nests. Exceptionally, the Schwann cell 
component of the ganglioneuroma may show features of a malignant nerve sheath tumor.(3)

Samad Zare, Mahmood Parvin, Seyed Mohammad Ghohestani
Departments of Urology and Pathology, Shahid Labbafinejad Medical Center, Shahid Beheshti University (MC), Tehran, Iran 

E-mail: drzaree@yahoo.com

REFERENCES
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recommendations by the International Neuroblastoma Pathology Committee. Cancer. 1999;86:349-63.

2. Umehara S, Nakagawa A, Matthay KK, et al. Histopathology defines prognostic subsets of ganglioneuroblastoma, nodular. 
Cancer. 2000;89:1150-61. 

3. Geoerger B, Hero B, Harms D, Grebe J, Scheidhauer K, Berthold F. Metabolic activity and clinical features of primary 
ganglioneuromas. Cancer. 2001;91:1905-13.