|Year : 2015 | Volume
| Issue : 1 | Page : 20-22
Retroperitoneal ganglioneuroma, a rare cause of abdominal pain
Palepu Sneha, Md Jawed Akther, G Satyam, Mandava Sumanth
Department of General Surgery, Mamata Medical College, Mamata General Hospital, Khammam, Telangana, India
|Date of Web Publication||23-Mar-2015|
Md Jawed Akther
Department of General Surgery, Mamata Medical College, Mamata General Hospital, Khammam, Telangana - 507 002
Source of Support: None, Conflict of Interest: None
Retroperitoneal ganglioneuroma (RGN) is a very rare entity especially in female older children and adolescents. It most often manifests as an asymptomatic mass. The relative rarity of this tumor in conjunction with the lack of definitive imaging findings makes its preoperative diagnosis difficult and is usually based on histopathological findings after surgical excision. We report a case of RGN, and suggest its consideration as one of the differential diagnosis in patients with abdominal pain.
Keywords: Ganglioneuroma, histopathology, retroperitoneum
|How to cite this article:|
Sneha P, Akther MJ, Satyam G, Sumanth M. Retroperitoneal ganglioneuroma, a rare cause of abdominal pain. Saudi Surg J 2015;3:20-2
| Introduction|| |
Ganglioneuroma (GN) is a rare benign tumor of neural crest origin with a reported incidence of one per million population, most common in the posterior mediastinum and the retroperitoneum. , It usually manifests as an asymptomatic mass discovered on routine screening.  Preoperative diagnosis of retroperitoneal GN (RGN) is often difficult and is usually based on histopathological findings after surgical excision. ,
| Case Report|| |
A 30-year-old female presented with right upper abdominal pain for 15 days, moderate in nature, dragging type and radiating to right loin. The pain was not related to food intake, and she had no associated changes in bowel habits. She denied experiencing nausea, vomiting, fever, chills, weight loss or other constitutional symptoms. No other significant history was present. On examination, abdomen was soft and mild tenderness was elicited on deep palpation in right hypochondrium. There was no palpable mass or organomegaly. She was normotensive with a blood pressure of 110/70 mmHg.
Ultrasonography revealed a heterogeneously hyperechoic well-capsulated mass lesion with minimal vascularity in the subhepatic region causing minimal compression of the right kidney inferiorly. It appeared to be separate from right kidney and liver. Computed tomography (CT) of abdomen [[Figure 1]] revealed 10 cm × 10 cm well-defined, hypodense lesion with multiple tiny calcific specks in the subhepatic region causing mild mass effect on the right kidney along with mild displacement of hepatic flexure of colon. On contrast examination, the lesion showed faint enhancement. Magnetic resonance imaging [[Figure 2]] revealed a large intra-abdominal right subhepatic mass lesion anterior to right kidney measuring approximately 10.5 cm × 6.4 cm × 11.6 cm appearing hypointense on T1-weight, and mixed intensity pattern in T2-weight. The mass was displacing the hepatic flexure medially without significant displacement of the right kidney and adjacent retroperitoneal structures. Postcontrast scans revealed peripheral enhancement of the capsule and punctate blotchy enhancement of the mass lesion suggestive of poor vascularity.
|Figure 1: Contrast enhanced computed tomography abdomen showing retroperitoneal mass to the right of inferior vena cava and anterior to the right kidney|
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|Figure 2: Magnetic resonance imaging showing mass in the subhepatic region|
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Intra-operatively, 11 cm × 8 cm × 6 cm well-circumscribed firm mass was identified subhepatically in the retroperitoneum to the right of inferior vena cava and anterior to right kidney that was dissected from the above structures easily and was excised [[Figure 3]]. Postoperative recovery was uneventful.
Biopsy revealed well-encapsulated tumor tissue with spindle-shaped tumor cells showing wavy nucleus arranged in transverse and longitudinal bundles and sheets [[Figure 4]]. Clusters of ganglion cells with abundant cytoplasm and eccentrically placed 1-3 large round vesicular nuclei having prominent macro nucleoli were seen. Areas of cystic degeneration, myxoid change, and neuromatous stroma were also observed, suggestive of GN.
|Figure 4: (a) Cut-section showing myxoid and grey white areas; (b) H and E, ×10; (c and d) Cluster of ganglion cells (H and E, ×40)|
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| Discussion|| |
Ganglioneuroma is a rare benign tumor of neural crest origin. The reported incidence of GN is one per million population.  It occurs commonly in older children and the median age at diagnosis is approximately 7 years. There is a slight female predominance, ranging from 1.13:1 to 1.5:1. , GNs are mostly sporadic with familial predisposition. Association with Turner's syndrome and multiple endocrine neoplasia II has been reported. 
They may be primary, occurring de-novo or secondarily from neuroblastoma (NB) either after chemotherapy or by maturation.  There are rare reports of metastatic GN. It is believed that these tumors represent metastases of NB or ganglioneuroblastoma (GNB) that have subsequently matured to GN. ,
As a primary retroperitoneal tumor, it constitutes only a small percentage of 0.72-1.6%.  It is most commonly found in the posterior mediastinum and the retroperitoneum.  Geoerger et al. found a predominance of thoracic (41.5%) and abdominal, nonadrenal tumors (37.5%) compared with adrenal GN (21%) and neck (8%).  Unusual sites include the spermatic cord, heart, bone, and intestine. An interesting feature is the tendency to partially or completely surround blood vessels without compromising the lumen in most cases. 
Ganglioneuroma most often manifests as an asymptomatic mass discovered on a routine radiographic study or ultrasound. Sometimes GN causes local mass effect and patients present with cough, dyspnea or abdominal pain.  In rare cases, GN secretes sufficient quantities of vanillyl mandelic acid or homovanillic acid to manifest with flushing and other symptoms of catecholamine excess. 
Neuroblastoma, GNB, and GN represent tumors of neural crest origin with a continuous spectrum of neuronal maturation. GN is composed of ganglion cells and mature Schwann cells (mature stroma). Cellular atypia, mitotic activity, and necrosis are not features of GN. GN averages 8 cm in diameter and may appear encapsulated, although a true capsule is infrequent. The tumors are firm, white to yellow in color, and may appear trabeculated or whorled. ,
Preoperative diagnosis of RGN is often difficult and is usually based on histopathological findings after surgical excision of the tumor. , CT is the most commonly used imaging modality for assessment because it reveals the extent of the tumor, organ of origin, regional invasion, vascular encasement, adenopathy, and calcification. 
Geoerger et al. stated that no tumor progression was noted after treatment was completed.  Nevertheless, these tumors may be slowly progressive and late recurrences have been described.  Therefore, long-term follow-up studies are necessary to assess the malignant potential in these tumors. 
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]