Skip to main content
Log in

Incidental ganglioneuromas: a presentation of 14 surgical cases and literature review

  • Original Article
  • Published:
Journal of Endocrinological Investigation Aims and scope Submit manuscript

Abstract

Background and aims

Ganglioneuromas are benign tumors which originate from the neural crest. This tumor affects mainly young patients rather than adult ones, and its most frequent localizations are mediastinum, retroperitoneum, adrenal glands and cervical region. Usually, ganglioneuromas are discovered as incidentalomas since they are often asymptomatic, even if they could present sympathetic or mass-related symptoms. To obtain a definitive diagnosis, histological exam is necessary since CT scan and MRI are not capable of distinguishing ganglioneuromas from other tumors, such as neuroblastomas or pheocromocytomas. The surgical excision is the chosen treatment and it offers an excellent prognosis.

Methods

We conducted a retrospective analysis of our cases of ganglioneuroma from 2004 to 2014; this study aims to compare our experience with literature review (2000–2014). Data about patients’ features, tumor localization, symptoms, treatment and follow-up were analyzed and reported in detailed tables.

Results

Between 2004 and 2014 we treated 14 patients affected by ganglioneuroma. For all of them the diagnosis was incidental; 9 out of 12 (64.3 %) patients presented an adrenal mass; in 2 patients (14.3 %) the tumor was localized in cervical region; in other 2 patients (14.3 %) the tumor was in the retroperitoneum and one patient (7.1 %) presented a ganglioneuroma in the costo-vertebral space. All our patients underwent surgical removal and none of them present surgery-related complications or recurrences to date.

Conclusions

Our data widen the knowledge about ganglioneuroma and confirm that the surgical approach has an excellent prognosis with very low incidence of surgery-related complications and recurrences.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Subscribe and save

Springer+ Basic
$34.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or eBook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Albuquerque BS, Farias TP, Dias FL, Torman D (2013) Surgical management of parapharyngeal ganglioneuroma: case report and review of the literature. ORL J Otorhinolaryngol Relat Spec 75(4):240–244

    Article  PubMed  Google Scholar 

  2. Lynch NP, Neary PM, Fitzgibbon JF, Andrews EJ (2013) Successful management of presacral ganglioneuroma: a case report and a review of the literature. Int J Surg Case Rep 4(10):933–935

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  3. Lonergan GJ, Schwab CM, Suarez ES, Carlson CL (2002) Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation. Radiographics 22(4):911–934

    Article  PubMed  Google Scholar 

  4. De Bernardi B, Gambini C, Haupt R, Granata C, Rizzo A, Conte M, Tonini GP et al (2008) Retrospective study of childhood ganglioneuroma. J Clin Oncol 26(10):1710–1716

    Article  PubMed  Google Scholar 

  5. Geoerger B, Hero B, Harms D, Grebe J, Scheidhauer K, Berthold F (2001) Metabolic activity and clinical features of primary ganglioneuromas. Cancer 91:1905–1913

    Article  CAS  PubMed  Google Scholar 

  6. Titos García A, Ramírez Plaza CP, Ruiz Diéguez P, Marín Camero N, Santoyo Santoyo J (2011) Ganglioneuroma as an uncommon cause of adrenal tumor. Endocrinol Nutr 58(8):443–445

    Article  PubMed  Google Scholar 

  7. Jain M, Shubha BS, Sethi S, Banga V, Bagga D (1999) Retroperitoneal ganglioneuroma: report of a case diagnosed by fine-needle aspiration cytology, with review of the literature. Diagn Cytopathol 21(3):194–196

    Article  CAS  PubMed  Google Scholar 

  8. Erem C, Ucuncu O, Nuhoglu I, Cinel A, Cobanoglu U, Demirel A, Koc E et al (2009) Adrenal ganglioneuroma: report of a new case. Endocrine 35(3):293–296

    Article  CAS  PubMed  Google Scholar 

  9. Kaufman MR, Rhee JS, Fliegelman LJ, Constantino PD (2001) Ganglioneuroma of the parapharyngeal space in a pediatric patient. Otolaryngol Head Neck Surg 124:702–704

    Article  CAS  PubMed  Google Scholar 

  10. Skaggs DL, Roberts JM, Codsi MJ, Meyer BC, Moral LA, Masso PD (2000) Mild gait abnormality and leg discomfort in a child secondary to extradural ganglioneuroma. Am J Orthop 29:111–114

    CAS  PubMed  Google Scholar 

  11. Scherer A, Niehues T, Engelbrecht V, Modder U (2001) Imaging diagnosis of retroperitoneal ganglioneuroma in childhood. Pediatr Radiol 31:106–111

    Article  CAS  PubMed  Google Scholar 

  12. Califano L, Zupi A, Mangone GM, Long F (2001) Cervical ganglioneuroma: report of a case. Otolaryngol Head Neck Surg 124(1):115–116

    Article  CAS  PubMed  Google Scholar 

  13. Otal P, Mezghani S, Hassissene S, Maleux G, Colombier D, Rousseau H, Joffre F (2001) Imaging of retroperitoneal ganglioneuroma. Eur Radiol 11(6):940–945

    Article  CAS  PubMed  Google Scholar 

  14. Menschik D, Lovvorn H, Hill A, Kelly P, Jones DP (2002) An unusual etiology of hypertension in a 5-year-old boy. Pediatr Nephrol 17:524–526

    Article  PubMed  Google Scholar 

  15. Chang CY, Hsieh YL, Hung GY, Pan CC, Hwang B (2003) Ganglioneuroma presenting as an asymptomatic huge posterior mediastinal and retroperitoneal tumor. J Chin Med Assoc 66(6):370–374

    PubMed  Google Scholar 

  16. Stárek I, Mihál V, Novák Z, Pospísilová D, Vomácka J, Vokurka J (2004) Pediatric tumors of the parapharyngeal space. Three case reports and a literature review. Int J Pediatr Otorhinolaryngol 68(5):601–606

    Article  PubMed  Google Scholar 

  17. Cannon TC, Brown HH, Hughes BM, Wenger AN, Flynn SB, Westfall CT (2004) Orbital ganglioneuroma in a patient with chronic progressive proptosis. Arch Ophthalmol 122(11):1712–1714

    Article  PubMed  Google Scholar 

  18. Yavascaoglu I, Vuruskan H, Kordan Y, Caliskan Z, Oktay B (2004) Coincidental adrenal ganglioneuroma—a case presenting with enuresis nocturna. Int Urol Nephrol 36(4):479–480

    Article  PubMed  Google Scholar 

  19. Przkora R, Perez-Canto A, Ertel W, Heyde CE (2006) Ganglioneuroma : primary tumor or maturation of a suspected neuroblastoma? Eur Spine J 15(3):363–365

    Article  PubMed Central  PubMed  Google Scholar 

  20. Cannady SB, Chung BJ, Hirose K, Garabedian N, Van Den Abbeele T, Koltai PJ (2006) Surgical management of cervical ganglioneuromas in children. Int J Pediatr Otorhinolaryngol 70(2):287–294

    Article  PubMed  Google Scholar 

  21. Shome D, Vemuganti GK, Honavar SG (2006) Choroidal ganglioneuroma in a patient with neurofibromatosis type 1: a case report. Eye (Lond) 20(12):1450–1451

    Article  CAS  Google Scholar 

  22. Pratap A, Tiwari A, Pandey S, Yadav RP, Agrawal A, Sah BP, Bajracharya T et al (2007) Ganglioneuroma of small bowel mesentery presenting as acute abdomen. J Pediatr Surg 42(3):573–575

    Article  PubMed  Google Scholar 

  23. Qureshi SS, Medhi SS (2008) Large adrenal ganglioneuroma with left inferior vena cava: implications for surgery. Pediatr Surg Int 24(4):455–457

    Article  PubMed  Google Scholar 

  24. Patterson AR, Barker CS, Loukota RA, Spencer J (2009) Ganglioneuroma of the mandible resulting from metastasis of neuroblastoma. Int J Oral Maxillofac Surg 38(2):196–198

    Article  CAS  PubMed  Google Scholar 

  25. Zhang WQ, Liu JF, Zhao J, Zhao SY, Xue Y (2009) Tumor with watery diarrhoea, hypokalaemia in a 3-year-old girl. Eur J Pediatr 168(7):859–862

    Article  PubMed  Google Scholar 

  26. Soccorso G, Puls F, Richards C, Pringle H, Nour S (2009) A ganglioneuroma of the sigmoid colon presenting as leading point of intussusception in a child: a case report. J Pediatr Surg 44(1):e17–e20

    Article  PubMed  Google Scholar 

  27. Zugor V, Schott GE, Kühn R, Labanaris AP (2009) Retroperitoneal ganglioneuroma in childhood—a presentation of two cases. Pediatr Neonatol 50(4):173–176

    Article  PubMed  Google Scholar 

  28. Dimou J, Russell JH, Jithoo R, Pitcher M (2009) Sacral ganglioneuroma in a 19-year-old woman. J Clin Neurosci 16(12):1692–1694

    Article  PubMed  Google Scholar 

  29. Shah SR, Purcell GP, Malek MM, Kane TD (2010) Laparoscopic right adrenalectomy for a large ganglioneuroma in a 12-year-old. J Laparoendosc Adv Surg Tech A 20(1):95–96

    Article  PubMed  Google Scholar 

  30. Kattepura S, Alexander B, Kini U, Das K (2010) Sporadic synchronous ganglioneuromas in a child—case report and review. J Pediatr Surg 45(4):822–825

    Article  PubMed  Google Scholar 

  31. Cai J, Zeng Y, Zheng H, Qin Y, K T, Zhao J (2010) Retroperitoneal ganglioneuroma in children: CT and MRI features with histologic correlation. Eur J Radiol 75(3):315–320

    Article  PubMed  Google Scholar 

  32. Lin PC, Lin SH, Chou SH, Chen YW, Chang TT, Wu JR, Jaw TS et al (2010) Ganglioneuroma of posterior mediastinum in a 6-year-old girl: imaging for pediatric intrathoracic incidentaloma. Kaohsiung J Med Sci 26(9):496–501

    Article  PubMed  Google Scholar 

  33. Al-Khiary H, Ayoubi A, Elkhamary SM (2010) Primary orbital ganglioneuroma in a 2-year-old healthy boy. Saudi J Ophthalmol 24(3):101–104

    Article  PubMed Central  PubMed  Google Scholar 

  34. Jawaid W, Solari V, Mahmood N, Jesudason EC (2010) Excision of ganglioneuroma from skull base to aortic arch. J Pediatr Surg 45(10):e29–e32

    Article  PubMed  Google Scholar 

  35. Do SI, Kim GY, Ki KD, Huh CY, Kim YW, Lee J, Park YK et al (2011) Ganglioneuroma of the uterine cervix—a case report. Hum Pathol 42(10):1573–1575

    Article  PubMed  Google Scholar 

  36. Retrosi G, Bishay M, Kiely EM, Sebire NJ, Anderson J, Elliott M, Drake DP et al (2011) Morbidity after ganglioneuroma excision: is surgery necessary? Eur J Pediatr Surg 21(1):33–37

    Article  CAS  PubMed  Google Scholar 

  37. Eassa W, El-Sherbiny M, Jednak R, Capolicchio JP (2012) The anterior approach to retroperitoneoscopic adrenalectomy in children: technique. J Pediatr Urol 8(1):35–39

    Article  PubMed  Google Scholar 

  38. Demir HA, Ozdel S, Kaçar A, Senel E, Emir S, Tunç B (2012) Ganglioneuroma in a child with hereditary spherocytosis. Turk J Pediatr 54(2):187–190

    PubMed  Google Scholar 

  39. Vasiliadis K, Papavasiliou C, Fachiridis D, Pervana S, Michaelides M, Kiranou M, Makridis C (2012) Retroperitoneal extra-adrenal ganglioneuroma involving the infrahepatic inferior vena cava, celiac axis and superior mesenteric artery: a case report. Int J Surg Case Rep 3(11):541–543

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  40. Camelo M, Aponte LF, Lugo-Vicente H (2012) Dopamine-secreting adrenal ganglioneuroma in a child: beware of intraoperative rebound hypertension. J Pediatr Surg 47(9):E29–E32

    Article  PubMed  Google Scholar 

  41. Elkaoui H, Bounaim A, Ait Ali A, Zentar A, Sair K (2012) Sacral ganglioneuroma. Presse Med 41(6 Pt 1):672–674

    Article  PubMed  Google Scholar 

  42. Urata S, Yoshida M, Ebihara Y, Asakage T (2013) Surgical management of a giant cervical ganglioneuroma. Auris Nasus Larynx 40(6):577–580

    Article  PubMed  Google Scholar 

  43. Kara T, Oztunali C (2013) Radiologic findings of thoracic scoliosis due to giant ganglioneuroma. Clin Imaging 37(4):767–768

    Article  PubMed  Google Scholar 

  44. Meng QD, Ma XN, Wei H, Pan RH, Jiang W, Chen FS (2013) Lipomatous ganglioneuroma of the retroperitoneum. Asian J Surg. doi:10.1016/j.asjsur.2013.07.011

  45. Nasseh H, Shahab E (2013) Retroperitoneal ganglioneuroma mimicking right adrenal mass. Urology 82(6):e41–e42

    Article  PubMed  Google Scholar 

  46. Kacagan C, Basaran E, Erdem H, Tekin A, Kayikci A, Cam K (2014) Case report: large adrenal ganglioneuroma. Int J Surg Case Rep 5(5):253–255

    Article  PubMed Central  PubMed  Google Scholar 

  47. Adas M, Koc B, Adas G, Ozulker F, Aydin T (2014) Ganglioneuroma presenting as an adrenal incidentaloma: a case report. J Med Case Rep 29(8):131

    Article  Google Scholar 

  48. Koktener A, Kosehan D, Akin K, Bozer M (2014) Incidentally Found Retroperitoneal Ganglioneuroma in an Adult. Indian J Surg, Epub

    Google Scholar 

  49. Alimoglu O, Caliskan M, Acar A, Hasbahceci M, Canbak T, Bas G (2012) Laparoscopic excision of a retroperitoneal ganglioneuroma. JSLS 16(4):668–670

    Article  PubMed Central  PubMed  Google Scholar 

  50. Ma J, Liang L, Liu H (2012) Multiple cervical ganglioneuroma: a case report and review of the literature. Oncol Lett 4(3):509–512

    PubMed Central  CAS  PubMed  Google Scholar 

  51. Hayat J, Ahmed R, Alizai S, Awan MU (2011) Giant ganglioneuroma of the posterior mediastinum. Interact Cardiovasc Thorac Surg 13(3):344–345

    Article  PubMed  Google Scholar 

  52. Gary C, Robertson H, Ruiz B, Zuzukin V, Walvekar RR (2010) Retropharyngeal ganglioneuroma presenting with neck stiffness: report of a case and review of literature. Skull Base 20(5):371–374

    Article  PubMed Central  PubMed  Google Scholar 

  53. Dario C, Luigi P, Laura Z, Valentina S, Luigi C, Sandro N, Emilio D et al (2009) Adrenal schwannoma incidentally discovered—a case report. J Chin Clin Med 4:43–46

    Google Scholar 

  54. Zografos GN, Farfaras A, Vasiliadis G, Pappa T, Aggeli C, Vassilatou E, Kaltsas G et al (2010) Laparoscopic resection of large adrenal tumors. JSLS 14(3):364–368

    Article  PubMed Central  PubMed  Google Scholar 

  55. Radin R, David CL, Goldfarb H, Francis IR (1997) Adrenal and extra-adrenal retroperitoneal ganglioneuroma: imaging findings in 13 adults. Radiology 202(3):703–707

    Article  CAS  PubMed  Google Scholar 

  56. Ichikawa T, Ohtomo K, Araki T, Fujimoto H, Nemoto K, Nanbu A, Onoue M et al (1996) Ganglioneuroma: computed tomography and magnetic resonance features. Br J Radiol 69(818):114–121

    Article  CAS  PubMed  Google Scholar 

  57. Materazzi G, Berti P, Conte M, Faviana P, Miccoli P (2010) Image of the month—quiz case. Ganglioneuroma. Arch Surg 145(1):99–100

    Article  PubMed  Google Scholar 

  58. Guo YK, Yang ZG, Li Y, Deng YP, Ma ES, Min PQ, Zhang XC (2007) Uncommon adrenal masses: CT and MRI features with histopathologic correlation. Eur J Radiol 62(3):359–370

    Article  PubMed  Google Scholar 

  59. Nelms JK, Diner EK, Lack EE, Patel SV, Ghasemian SR, Verghese M (2004) Retroperitoneal ganglioneuroma encasing the celiac and superior mesenteric arteries. Sci World J 18(4):974–977

    Article  Google Scholar 

  60. Moschovi M, Arvanitis D, Hadjigeorgi C, Mikraki V, Tzortzatou-Stathopoulou F (1997) Late malignant transformation of dormant ganglioneuroma? Med Pediatr Oncol 28(5):377–381

    Article  CAS  PubMed  Google Scholar 

  61. Kulkarni AV, Bilbao JM, Cusimano MD, Muller PJ (1998) Malignant transformation of ganglioneuroma into spinal neuroblastoma in an adult. Case report. J Neurosurg 88(2):324–327

    Article  CAS  PubMed  Google Scholar 

  62. Kimura S, Kawaguchi S, Wada T, Nagoya S, Yamashita T, Kikuchi K (2002) Rhabdomyosarcoma arising from a dormant dumbbell ganglioneuroma of the lumbar spine: a case report. Spine 27:513–517

    Article  Google Scholar 

  63. Drago G, Pasquier B, Pasquier D, Pinel N, Rouault-Plantaz V, Dyon JF, Durand C et al (1997) Malignant peripheral nerve sheath tumor arising in a “de novo” ganglioneuroma: a case report and review of the literature. Med Pediatr Oncol 28:216–222

    Article  CAS  PubMed  Google Scholar 

  64. Sánchez-Galán A, Barrena S, Vilanova-Sánchez A, Martín SH, Lopez-Fernandez S, García P, Lopez-Santamaria M et al (2014) Ganglioneuroma: to operate or not to operate. Eur J Pediatr Surg 24(1):25–30

    Article  PubMed  Google Scholar 

  65. Cerullo G, Marrelli D, Rampone B, Miracco C, Caruso S, Di Martino M, Mazzei MA et al (2007) Presacral ganglioneuroma: a case report and review of literature. World J Gastroenterol 13(14):2129–2131

    PubMed Central  PubMed  Google Scholar 

Download references

Acknowledgement

This research received no specific Grant from any funding agency in the public, commercial, or not-for-profit sectors.

Conflict of interest

The authors report no conflicts of interest.

Informed consent

This work was prepared after written informed consent was obtained from the patients or their relatives.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to C. Spinelli.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Spinelli, C., Rossi, L., Barbetta, A. et al. Incidental ganglioneuromas: a presentation of 14 surgical cases and literature review. J Endocrinol Invest 38, 547–554 (2015). https://doi.org/10.1007/s40618-014-0226-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s40618-014-0226-y

Keywords

Navigation