Ganglioneuromas are benign, well differentiated tumors arising from the sympathetic nervous system. However, they arise rarely in the adrenal glands. Ganglioneuroma is a very rare (0–6% of incidentalomas) tumor that arises from sympathetic ganglion/chain and is made of mature ganglion cells, Schwann cells, neurites and nerve fibers. The most common sites are the posterior mediastinum and the retroperitoneal space [1] . Most cases of adrenal ganglioneuromas are incidentally diagnosed since they are mostly asymptomatic and produce symptoms rarely due to compression of neighboring structures. Retroperitoneal ganglioneuromas are usually non-secreting and asymptomatic but when they reach large sizes they cause pressure symptoms locally [2]. We hereby present a rare case of an adult female patient presenting with pain in abdomen diagnosed with a non-secretory adrenal mass who underwent laparoscopic excision of the mass. She was finally diagnosed on pathological examination to be an adrenal ganglioneuroma, one of the very rare tumors reported in the medical world literature [3][4].

Adrenal ganglioneuromas (GN) are rare tumors gaining origin from the neural crest cells of the sympathetic nervous system. This family of ganglion cell tumors includes GN which is benign, ganglioneuroblastoma which are of intermediate differentiation, and neuroblastoma which is a highly malignant lesion. Neuroblastoma and ganglioneuroblastoma mostly occur in infants and children, whereas GN has more prevalence in adolescents and young adults.

Laparoscopic adrenalectomy is now the gold standard in management of small adrenal masses. The standard treatment for benign adrenal tumors is laparoscopic or/minimal access surgery. Large tumors (> 6 cm) are relative contraindications to laparoscopic adrenalectomy as they are more in favor of a diagnosis of adrenal carcinoma. We present in the following report a peculiar presentation of this condition and its laparoscopic excision followed by histopathology proving a diagnosis of ganglioneuroma.

A 40-year-old female presented with a history of vague pain in abdomen since 15 days and not associated with local flank swelling, constipation, or anorexia. There was no significant past surgical or medical history. No history of recent travel and family history of colitis or inflammatory bowel disease. On physical examination, the abdomen was soft, there was no distension or tenderness.

Computed tomography scan of the abdomen and pelvis with contrast study was suggestive of a right suprarenal mass lesion measuring approximately 4x2 cm with internal necrotic and calcified areas and moderate relatively homogenous with post contrast enhancement suspicious of neurogenic tumor (Figure 1A). Routine laboratory investigations including thyroid function tests were within normal range (Table 1).

Laparoscopic excision of the suprarenal mass was performed and sent for histopathological examination (Figure 1B).

On gross examination of specimen was a solid firm well delineated mass of size 4x3x2 cm brown in colour on the outside and cut section revealed pale greyish white appearance with peripheral golden yellow tissue with focal calcification (Figure 1C). Microscopic sections of the histopathological specimen showed ganglion cells and Schwann cells arranged in interlacing bundles suggestive of ganglioneuroma (Schwannian stroma dominant neuroblastic tumors) with peripheral normal adrenal parenchyma and no evidence of malignancy. On immunohistochemistry ganglion cells showed positivity for chromogranin and S-100 Protein was positive in fibres and focally in ganglion cells (Figure 2).

Ganglioneuromas can occur in the central nervous system or in the peripheral nervous system (sympathetic) [1]. It usually affects children and young patients below the age 20. Females are affected more than males [2]. The most common site of GN are retroperitoneum (40–50%) followed by mediastinal location (30–40%), or cervical region (up to 10%) and only rarely in adrenal gland [3] [4] [5].

The incidence of adrenal ganglioneuroma is 1/ million population being most commonly sporadic in occurrence but could be associated with other syndromes like multiple endocrine neoplasia type 2/neurofibromatosis type 2.

Ganglioneuromas are clinically asymptomatic and mostly hormonally inactive. Despite being benign and hormonally inactive in nature, GNs may cause pressure symptoms due to compression of their adjacent structures [5] [6]. Up to one-third of patients have elevated catecholamine levels but they rarely develop symptoms due to their excess.

Ganglioneuroma (GN) arises from the neural crest cells -the sympathetic ganglia and the adrenals. It is composed of Schwann cells, ganglion cells and fibrous tissues. The differential diagnosis of a tumor with high resemblance to ganglioneuroma is a neuroblastoma. In neuroblastoma, levels of urinary homovanillic acid (HVA) and vanillylmandelic acid (VMA) are usually raised, while the levels of urinary HVA and VMA in ganglioneuroma are within normal ranges [7]. Neuroblasts are not a part of mature GN. Thus the meta iodo benzyl guanidine uptake of a GN is lower than that of a neuroblastoma.

The GN should be diagnosed when the following findings are noted in a case of an adrenal mass: (1) non secreting, (2) absent vessel involvement, (3) calcifications in the gland, and (4) magnetic resonance imaging (MRI) findings of a non-enhanced T1-weighted signal with late and gradual enhancement. The final treatment for adrenal ganglioneuromas is resection by either open or laparoscopic method [8][9][10].

Adrenal ganglioneuromas occur rarely and difficult in diagnosing preoperatively since symptoms vary and are mostly nonspecific. Due to widespread utilization of imaging modalities like abdominal ultrasonography, computed tomography and magnetic resonance imaging, detection of such tumor has increased. Thus, ganglioneuroma should not be missed as a differential diagnosis of an adrenal mass. Histopathologic examination plays a crucial role in diagnosis.

1. Chang CY, Hsieh YL, Hung GY, Pan CC, Hwang B. Ganglioneuroma presenting as an asymptomatic huge posterior mediastinal and retroperitoneal tumor. J Chin Med Assoc 2003 Jun;66(6):370–4.   [Pubmed]    
2. Zugor V, Amann K, Schrott KM, Schott GE. Retroperitoneal ganglioneuroma. [Article in German]. Aktuelle Urol 2005 Aug;36(4):349–52.   [Pubmed]    
3. Erem C, Ucuncu O, Nuhoglu I, Adrenal ganglioneuroma: report of a new case. Endocrine 2009 Jun;35(3):293–6.   [CrossRef]   [Pubmed]    
4. Arredondo Martínez F, Soto Delgado M, Benavente Fernández A, et al. Adrenal ganglioneuroma. Report of a new case. [Article in Spanish]. Actas Urol Esp 2003 Mar;27(3):221–5.   [Pubmed]    
5. Nieman LK. Approach to the patient with an adrenal incidentaloma. J Clin Endocrinol Metab 2010 Sep;95(9):4106–13.   [CrossRef]   [Pubmed]    
6. Hayes FA, Green AA, Rao BN. Clinical manifestations of ganglioneuroma. Cancer 1989 Mar 15;63(6):1211–4.   [Pubmed]    
7. Bove KE, McAdams AJ. Composite ganglioneuroblastoma. An assessment of the significance of histological maturation in neuroblastoma diagnosed beyond infancy. Arch Pathol Lab Med 1981 Jun;105(6):325–30.   [Pubmed]    
8. Sucandy I, Akmal YM, Sheldon DG. Ganglioneuroma of the adrenal gland and retroperitoneum: A case report. N Am J Med Sci 2011 Jul;3(7):336–8.   [CrossRef]   [Pubmed]    
9. Lonergan GJ, Schwab CM, Suarez ES, Carlson CL. Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation. Radiographics 2002 Jul-Aug;22(4):911–34.   [Pubmed]    
10. Moriwaki Y, Miyake M, Yamamoto T, et al. Retroperitoneal ganglioneuroma: a case report and review of the Japanese literature. Intern Med 1992 Jan;31(1):82–5.   [CrossRef]   [Pubmed]