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Ganglioneuroma of the adrenal gland and retroperitoneum: A case report.
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MedLine Citation:
PMID:  22540109     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Iswanto Sucandy; Yasir M Akmal; David G Sheldon
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  North American journal of medical sciences     Volume:  3     ISSN:  1947-2714     ISO Abbreviation:  N Am J Med Sci     Publication Date:  2011 Jul 
Date Detail:
Created Date:  2012-04-27     Completed Date:  2012-10-02     Revised Date:  2013-05-29    
Medline Journal Info:
Nlm Unique ID:  101521411     Medline TA:  N Am J Med Sci     Country:  India    
Other Details:
Languages:  eng     Pagination:  336-8     Citation Subset:  -    
Affiliation:
Department of Surgery, Abington Memorial Hospital, Abington, PA., USA.
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Journal Information
Journal ID (nlm-ta): N Am J Med Sci
Journal ID (iso-abbrev): N Am J Med Sci
Journal ID (publisher-id): NAJMS
ISSN: 2250-1541
ISSN: 1947-2714
Publisher: Medknow Publications & Media Pvt Ltd, India
Article Information
Copyright: © North American Journal of Medical Sciences
open-access:
Print publication date: Month: 7 Year: 2011
Volume: 3 Issue: 7
First Page: 336 Last Page: 338
ID: 3336884
PubMed Id: 22540109
Publisher Id: NAJMS-3-336
DOI: 10.4297/najms.2011.3336

Ganglioneuroma of the adrenal gland and retroperitoneum: A case report
Iswanto Sucandy, M.D.1
Yasir M Akmal, M.D.2
David G Sheldon, M.D., F.A.C.S.3
1Department of Surgery, Abington Memorial Hospital, Abington, PA., USA.
2Department of Surgery, City of Hope National Medical Center, Duarte, CA, USA.
3Department of Surgery, Kalispell Regional Medical Center, Kalispell, MT, USA.
Correspondence: Correspondence to: Iswanto Sucandy, MD. Department of General and Vascular Surgery, Abington Memorial Hospital 1200 Old York Road, Abington, PA. 19001, USA. Tel.: 215-481-7460, Fax: 215-481-2159, isucandy@amh.org

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 (Figures 1 and 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.


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.


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
1. Hayes FA,Green AA,Rao BN. Clinical Manifestation of GanglioneuromaCancerYear: 198963121112142917322
2. Georger B,Hero B,Harms D,Grebe J,Scheidhauer K,Berthold F. Metabolic Activity and Clinical Features of Primary GanglioneuromaCancerYear: 200191101905191311346873
3. Bove KE,McAdams AJ. Composite ganglioneuroblastomaArch Pathol Lab MedYear: 19811053253306894529
4. Maweja S,Materne R,Detrembleur N,et al. Adrenal GanglioneuromaAm J SurgYear: 200719468368417936435
5. Erem C,Kocak M,Cinel A,et al. Dopamine-secreting adrenal ganglioneuroma presenting with paroxysmal hypertension attacksSaudi Med JYear: 200829112212518176686
6. Arredondo Martínez F,Soto Delgado M,Benavente Fernández A,et al. Adrenal ganglioneuroma. Report of a new caseActas Urol EspYear: 200327322122512812120
7. Gupta R,Dinda AK. Ganglioneuroma of the adrenal gland: a rare caseIndian J Pathol MicrobiolYear: 200750478278418306553
8. Ito H,Fuse H,Hirano S,et al. Ganglioneuroma arising in the adrenal medulla: a case reportHinyokika KiyoYear: 199844129329503205
9. Erem C,Ucuncu O,Nuhoglu I,et al. Adrenal ganglioneuroma: report of a new caseEndocrineYear: 200935329329619367379
10. Maweja S,Materne R,Detrembleur N,et al. Adrenal ganglioneuromaAm J SurgYear: 2007194568368417936435
11. Koch CA,Brouwers FM,Rosenblatt K,et al. Adrenal ganglioneuroma in a patient presenting with severe hypertension and diarrheaEndocr Relat CancerYear: 20031019910712653673
12. Diab DL,Faiman C,Siperstein AE. Virilizing adrenal ganglioneuroma in a woman with subclinical Cushing syndromeEndocr PractYear: 200814558458718753101
13. Ozbay A,Obukhau A,Buhl L,et al. Adrenal pheochromocytoma producing vasoactive intestinal peptide and masking hypertensionHorm ResYear: 200870318819218663320
14. Mukai M,Takao T,Yoshida T,et al. Adrenal ganglioneuroma in a 14-year-old girl: a case reportHinyokika KiyoYear: 200652861962116972624
15. Lora MS,Waguespack SG,Moley JF,et al. Adrenal ganglioneuromas in children with multiple endocrine neoplasia type 2: a report of two casesJ Clin Endocrinol MetabYear: 20059074383438715827098
16. Moriwaki Y,Miyake M,Yamamoto T,et al. Retroperitoneal ganglioneuroma: a case report and review of the Japanese literatureIntern MedYear: 199231182851568049
17. Tsumatani K,Hayashi Y,Tabata S,et al. Ganglioneuroma of the adrenal gland: report of a caseHinyokika KiyoYear: 19893511189719012694826

Figures

[Figure ID: F1]
Fig. 1 

Coronal section of the right-sided adrenal ganglioneuroma



[Figure ID: F2]
Fig. 2 

Axial section of the retropancreatic mass (5.8 × 4.3 cm)



Tables
[TableWrap ID: T1] Table 1 

Previously published case reports of adrenal and retroperitonal ganglioneuromas




Article Categories:
  • Case Report

Keywords: Ganglioneuroma, retroperitoneum, adrenal gland, excision.

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