North American Journal of Medical Sciences

CASE REPORT
Year
: 2011  |  Volume : 3  |  Issue : 7  |  Page : 336--338

Ganglioneuroma of the adrenal gland and retroperitoneum: A case report


Iswanto Sucandy1, Yasir M Akmal2, David G Sheldon3,  
1 Department of Surgery, Abington Memorial Hospital, Abington, PA., USA
2 Department of Surgery, City of Hope National Medical Center, Duarte, CA, USA
3 Department of Surgery, Kalispell Regional Medical Center, Kalispell, MT, USA

Correspondence Address:
Iswanto Sucandy
Department of General and Vascular Surgery, Abington Memorial Hospital 1200 Old York Road, Abington, PA. 19001
USA

Abstract

Context: Ganglioneuromas are benign tumors of the sympathetic nervous system that rarely arise in the adrenal gland. Majority of cases are detected incidentally since they are usually asymptomatic. Up to the current era of laparoscopic adrenal mass excision, this unusual entity has not been adequately reported in the surgical literature. Case Report: A 51 year old male with history of hypertension was found to have abdominal bruit during a regular physical examination. A 4 cm right adrenal mass with upper pole calcification and a 6 cm retro-pancreatic mass were subsequently found on a computed tomography scan. Endoscopic ultrasound-guided needle biopsy was indeterminate. Preoperative endocrine evaluation showed mildly elevated vanillyl mandelic acid with normal 24-hour cathecolamine, metanephrine and cortisol levels. Histopathologic examination after an uneventful laparoscopic excision was consistent with ganglioneuroma. Conclusions: Ganglioneuroma occurs rarely in adrenal gland and preoperative diagnosis is difficult since symptoms are usually nonspecific. Due to widespread utilization of abdominal imaging, however, it should be included in differential diagnosis of adrenal or retroperitoneal mass. Histopathologic examination is currently the mainstay of diagnosis.



How to cite this article:
Sucandy I, Akmal YM, Sheldon DG. Ganglioneuroma of the adrenal gland and retroperitoneum: A case report.North Am J Med Sci 2011;3:336-338


How to cite this URL:
Sucandy I, Akmal YM, Sheldon DG. Ganglioneuroma of the adrenal gland and retroperitoneum: A case report. North Am J Med Sci [serial online] 2011 [cited 2020 Oct 25 ];3:336-338
Available from: https://www.najms.org/text.asp?2011/3/7/336/86152


Full Text

 Introduction



Ganglioneuromas, benign tumors of the sympathetic nervous system, are rare to arise in the adrenal gland. They are usually asymptomatic and hormonally silent. Majority of cases are detected incidentally during work-up for unrelated conditions. They usually develop from the great sympathetic chains, extending from the base of the skull to the neck, posterior mediastinum, retroperitoneum, and adrenal glands.

Currently, histopathologic examination is the only tool to diagnose ganglioneuroma and to differentiate it from other neural crest tumors. Up to the current era of laparoscopic adrenal mass excision, this unusual entity has not been adequately reported in the surgical literature.

 Case Report



A 51 year old man was referred to Geisinger clinic by his primary care physician for an abdominal bruit that was found during a regular physical examination. Past medical and surgical histories were only significant for mild hypertension. Abdominal computed tomography (CT) scan revealed a 4 cm right adrenal mass with upper pole calcifications and a 6 cm retropancreatic mass [Figure 1] and [Figure 2]. Both masses are mainly hypodense on arterial imaging and heterogenous on delayed phase images. Endoscopic ultrasound (EUS) guided needle biopsy was indeterminate. Preoperative endocrine evaluation only showed mildly elevated vanillyl mandelic acid (VMA) with a normal 24-hour cathecolamine, metanephrine and cortisol levels. Intraoperatively, the tumors were able to be separated off the surrounding structures without major difficulty. Histopathologic examination after a complete laparoscopic excision was consistent with the diagnosis of ganglioneuroma. {Figure 1}{Figure 2}

 Discussion



Ganglioneuroma (GN) originates from cells of the neural crest that include the sympathetic ganglias and the adrenal glands. It is composed of ganglion cells, neurites, schwann cells, and fibrous tissues. This tumor affects children and young people preferentially with three-fifth developed before age 20. Females are more prone to be affected than males. Majority of GNs are thoracic and retroperitoneal in location while adrenal GNs are rare.

Clinical symptoms of GNs are non-spesific, mosty hormonally silent and related to their size/locations. Despite their generally benign nature, GNs may come to attention due to compression of their neighboring structures [1] . Approximately up to 30% of patients were found to have elevated plasma and urinary catecholamine but they rarely develop symptoms of vasoactive amines excess. Immature element such as neuroblast is not part of mature GN explaining lower rate of metaiodobenzylguanidine (MIBG) uptake (57%) compared to neuroblastoma (92%) [2] .

Diagnosis of GN should be suggested when an adrenal tumor harbors the following: (1) no hormonal hypersecretion, (2) presence of punctate or discrete calcifications, (3) absence of vessel involvement, and (4) a low non-enhanced T1-weighted signal with a late and gradual enhancement on dynamic magnetic resonance imaging (MRI). The treatment for this condition is complete surgical resection through either an open or laparoscopic approach.

The most significant differential diagnosis of ganglioneuroma is neuroblastoma. Increased level of urinary noradrenaline, dopamine, HVA, and VMA are frequently encountered in neuroblastoma, while the level of urinary cathecolamine, HVA, and VMA are usually normal in ganglioneuroma [3] . In the present review of 14 cases displayed in [Table 1], an increased urinary catecholamine level and sex hormone were noted in several patients, therefore pharmacological diagnostic tests for such hormonal activities may not necessarily be discriminative in the differential diagnosis of these two related tumors.{Table 1}

 Conclusions



Ganglioneuroma occurs rarely in adrenal gland and preoperative diagnosis is difficult since symptoms vary and oftentimes nonspecific. Due to widespread utilization of abdominal ultrasonography, CT scan and MRI, detection of such tumor has increased, therefore differential diagnosis of an adrenal/retroperitoneal mass should include ganglioneuroma. Histopathologic examination is currently the mainstay of diagnosis.[17]

References

1Hayes FA. Green AA and Rao BN. Clinical Manifestation of Ganglioneuroma. Cancer 1989; 63: 1211-1214.
2Georger B, Hero B, Harms D, Grebe J, Scheidhauer K, Berthold F. Metabolic Activity and Clinical Features of Primary Ganglioneuroma. Cancer 2001; 91 (10): 1905-1913.
3Bove KE, McAdams AJ. Composite ganglioneuroblastoma. Arch Pathol Lab Med 1981; 105: 325-330.
4Maweja S, Materne R, Detrembleur N, et al. Adrenal Ganglioneuroma. Am J Surg 2007; 194: 683-684.
5Erem C, Kocak M, Cinel A, et al. Dopamine-secreting adrenal ganglioneuroma presenting with paroxysmal hypertension attacks. Saudi Med J 2008; 29(1):122-125.
6Arredondo Martínez F, Soto Delgado M, Benavente Fernández A, et al. Adrenal ganglioneuroma. Report of a new case. Actas Urol Esp 2003;27(3):221-225.
7Gupta R, Dinda AK. Ganglioneuroma of the adrenal gland: a rare case. Indian J Pathol Microbiol. 2007; 50(4):782-784.
8Ito H, Fuse H, Hirano S, et al. Ganglioneuroma arising in the adrenal medulla: a case report. Hinyokika Kiyo 1998; 44(1):29-32.
9Erem C, Ucuncu O, Nuhoglu I, et al. Adrenal ganglioneuroma: report of a new case. Endocrine 2009;35(3):293-296.
10Maweja S, Materne R, Detrembleur N, et al. Adrenal ganglioneuroma. Am J Surg 2007;194(5):683-684.
11Koch CA, Brouwers FM, Rosenblatt K, et al. Adrenal ganglioneuroma in a patient presenting with severe hypertension and diarrhea. Endocr Relat Cancer. 2003; 10(1): 99-107.
12Diab DL, Faiman C, Siperstein AE. Virilizing adrenal ganglioneuroma in a woman with subclinical Cushing syndrome. Endocr Pract 2008; 14(5): 584-587.
13Ozbay A, Obukhau A, Buhl L, et al. Adrenal pheochromocytoma producing vasoactive intestinal peptide and masking hypertension. Horm Res 2008; 70(3): 188-192.
14Mukai M, Takao T, Yoshida T, et al. Adrenal ganglioneuroma in a 14-year-old girl: a case report. Hinyokika Kiyo. 2006; 52(8): 619-621.
15Lora MS, Waguespack SG, Moley JF, et al. Adrenal ganglioneuromas in children with multiple endocrine neoplasia type 2: a report of two cases. J Clin Endocrinol Metab 2005; 90(7):4383-4387.
16Moriwaki Y, Miyake M, Yamamoto T, et al. Retroperitoneal ganglioneuroma: a case report and review of the Japanese literature. Intern Med 1992; 31(1): 82-85.
17Tsumatani K, Hayashi Y, Tabata S, et al. Ganglioneuroma of the adrenal gland: report of a case. Hinyokika Kiyo 1989; 35(11):1897-1901.