Ann Surg Treat Res.  2021 Aug;101(2):79-84. 10.4174/astr.2021.101.2.79.

Adrenal ganglioneuroma resected for suspicious malignancy: multicenter review of 25 cases and review of the literature

• Zilberman DE
• Drori T
• Shlomai G
• Mazeh H
• Fishman B
• Golan S
• Hendel H
• Laniado M
• Dotan ZA

• KMID: 2519000
• DOI: http://doi.org/10.4174/astr.2021.101.2.79

Abstract

Purpose
We reviewed the experience with adrenal ganglioneuroma (AGN) pathologically confirmed following adrenalectomy in medium- to high-volume medical centers.
Methods
The medical records of all adrenalectomy cases in 4 medical centers between 2006 and 2020 were retrospectively reviewed for demographics, clinical, radiological and laboratory findings, surgical treatment, pathology results, and outcomes.
Results
Twenty-five out of 875 adrenalectomy cases (2.9%) were pathologically confirmed as AGN. Those patients’ average age was 40.5 years (range, 4–76 years), 13 (52.0%) were males, and 18 lesions (72.0%) were right-sided. One patient had a family history of neurofibromatosis, and another had a succinate dehydrogenase gene mutation. Abdominal/back pain attributed to mass effect was the most common symptom. All 25 patients underwent abdominal computerized tomography scanning in which the average maximal tumor diameter was 6.61 cm. The mean pre- and postcontrast Hounsfield units (HU) values were 35.2 and 59, respectively; and the mean late-phase HU value was 71.1. Twenty-two patients (88.0%) underwent minimally invasive surgery. The average tumor diameter recorded in the final pathology report was 7 cm. Isolated AGN was diagnosed in 21 cases (84.0%), and the additional components reported for the remaining 4 cases included pheochromocytoma (2), ganglioneuroblastoma (1), and neurofibroma (1). The average follow-up length was 16.8 months (range, 1–136 months), during which there was no recurrence or death.
Conclusion
AGN is a rare, slow-growing, large benign tumor with radiological characteristics similar to those seen in malignant tumor. Final diagnosis is established by pathology after surgical resection, preferably minimally invasive, with an overall excellent prognosis.

Keyword

Adrenal glands; Adrenal gland neoplasms; Benign neoplasms; Ganglioneuroma

Figure

• 

  Fig. 1 Adrenal ganglioneuroma in a 24-year-old patient. (A) Macroscopic appearance. (B) Tomographic appearance. Written informed consent has been obtained for these images.

Reference

1. Iacobone M, Torresan F, Citton M, Schiavone D, Viel G, Favia G. Adrenal ganglioneuroma: the Padua Endocrine Surgery Unit experience. Int J Surg. 2017; 41(Suppl 1):S103–S108. PMID: 28506406.

2. Spinelli C, Rossi L, Barbetta A, Ugolini C, Strambi S. Incidental ganglioneuromas: a presentation of 14 surgical cases and literature review. J Endocrinol Invest. 2015; 38:547–554. PMID: 25501841.

3. Lee JH, Chai YJ, Kim TH, Choi JY, Lee KE, Kim HY, et al. Clinicopathological features of ganglioneuroma originating from the adrenal glands. World J Surg. 2016; 40:2970–2975. PMID: 27431319.

4. Rondeau G, Nolet S, Latour M, Braschi S, Gaboury L, Lacroix A, et al. Clinical and biochemical features of seven adult adrenal ganglioneuromas. J Clin Endocrinol Metab. 2010; 95:3118–3125. PMID: 20427489.

5. Linos D, Tsirlis T, Kapralou A, Kiriakopoulos A, Tsakayannis D, Papaioannou D. Adrenal ganglioneuromas: incidentalomas with misleading clinical and imaging features. Surgery. 2011; 149:99–105. PMID: 20452635.

6. Xie J, Dai J, Zhou WL, Sun FK. Adrenal ganglioneuroma: features and outcomes of 42 cases in a Chinese population. World J Surg. 2018; 42:2469–2475. PMID: 29383425.

7. Li J, Yang CH, Li LM. Diagnosis and t reatment of 29 cases of adrenal ganglioneuroma. Eur Rev Med Pharmacol Sci. 2013; 17:1110–1113. PMID: 23661526.

8. Shawa H, Elsayes KM, Javadi S, Morani A, Williams MD, Lee JE, et al. Adrenal ganglioneuroma: features and outcomes of 27 cases at a referral cancer centre. Clin Endocrinol (Oxf). 2014; 80:342–347. PMID: 24033606.

9. Dages KN, Kohlenberg JD, Young WF Jr, Murad MH, Prokop L, Rivera M, et al. Presentation and outcomes of adrenal ganglioneuromas: a cohort study and a systematic review of literature. Clin Endocrinol (Oxf). 2021; 95:47–57. PMID: 33721367.

10. Li L, Shao J, Gu J, Wang X, Qu L. Adrenal ganglioneuromas: experience from a retrospective study in a Chinese population. Urol J. 2014; 11:1485–1490. PMID: 24807765.

11. Qing Y, Bin X, Jian W, Li G, Linhui W, Bing L, et al. Adrenal ganglioneuromas: a 10-year experience in a Chinese population. Surgery. 2010; 147:854–860. PMID: 20060552.

12. Maweja S, Materne R, Detrembleur N, de Leval L, Defechereux T, Meurisse M, et al. Adrenal ganglioneuroma. A neoplasia to exclude in patients with adrenal incidentaloma. Acta Chir Belg. 2007; 107:670–674. PMID: 18274182.

13. Lam AK. Update on adrenal tumours in 2017 World Health Organization (WHO) of endocrine tumours. Endocr Pathol. 2017; 28:213–227. PMID: 28477311.

14. Mylonas KS, Schizas D, Economopoulos KP. Adrenal ganglioneuroma: what you need to know. World J Clin Cases. 2017; 5:373–377. PMID: 29085827.

15. Lin X, Wu D, Chen C, Zheng N. Clinical characteristics of adrenal tumors in children: a retrospective review of a 15-year single-center experience. Int Urol Nephrol. 2017; 49:381–385. PMID: 27988912.

16. Fassnacht M, Kenn W, Allolio B. Adrenal tumors: how to establish malignancy? J Endocrinol Invest. 2004; 27:387–399. PMID: 15233562.

17. Suárez-Peñaranda JM, Gómez-Otero I, Muñoz JI, Pedreira-Pérez M. [Cardiac death associated with an adrenal ganglioneuroma]. Rev Esp Cardiol. 2008; 61:551–552. Spanish. PMID: 18462665.