GANGLIONEUROME PRESACRE

FATMA FITOURI | SONDES SAHLI | ZOHRA RAHAL | MOHAMED MOURAD HAMZAOUI |

La tunisie chirurgicale - 2018 ; Vol 2018

Resumé

Introduction

Les ganglioneuromes présacrés sont des lésions rares, généralement bénignes. Les patients consultent quand la masse devient symptomatique.

But

Nous rapportons un cas d’une localisation pré sacrée d’un ganglioneurome. Cette localisation est peu décrite dans la littérature. Ce cas souligne l'importance d’une excision complète, qui permet de prévenir une récidive à long terme.

Cas rapporté

C'était une fillette de 4 ans présentant une boiterie, en rapport avec une masse fessière gauche l’examen histologique de la masse a confirmé qu’il s’agit d’un ganglioneurome présacré.

Mots Clés

ganglioneuroma, gluteal mass, surgery, ganglioneurome, masse fessière, chirurgie

Introduction :

Ganglioneuromas are benign tumors that originate from neural crest cells, forming the sympathetic nervous system. They are composed of mature ganglion cells (1). Young patients are most commonly affected, especially females (2). Ganglioneuromas develop predominantly in the posterior mediastinum and retroperitoneum (3). They are rarely localized in the presacral space. Imaging techniques such as computed tomography (CT) and MRI have become more widely performed, the number of ganglioneuromas detected incidentally has increased. We report our experience with a case of presacral ganglionuroma (4).

Article

Case report   

We report a case of 4-year-old girl presenting with a left buttock mass and lameness walking. Physical examination of the abdomen was normal, temperature was 37°C. The positive findings were limited to the buttock, where a large, firm, non-tender, fixed mass, measuring 120 millimeters x 85 millimeters, centrally located between the medial quadrants was found (figure 1). Rectal examination revealed the same mass, centrally and posterior located and very firm to the palpating finger. Urinalysis was negative. Routine blood work was normal. Investigative imaging (ultrasonography and CT, SCAN) revealed an enhancing retroperitoneal tumor lying anterior to the sacrum and lumbosacral junction (figure 2). Biopsy of the lesion suggested a ganglioneuroma. The tumor was exposed via a posterior approach and completely excised (figures 3,4). The patient was discharged into seven days. The specimen was a firm tumor mass weighing 256 grams. Histology confirmed the diagnosis of a ganglioneuroma with no evidence of neuroblastoma.

Discussion

Ganglioneuroma are rare benign tumor arising from neural crest cells that develops in the autonomic nervous system (5). Ganglioneuromas are most commonly occurred in the patient aged between 10 and 30 years old with a slight female predominance. The more common sites are the posterior mediastinum and retroperitoneum (especially arising from the adrenal medulla) (3). They are typically slow growing tumors with a tendency to remain clinically silent for a considerable period and their exert mass effect on spinal and sympathetic nerves causing neural dysfunction (6). Furthmore, functional ganglioneuromas that were found to release peptides such as vasoactive intestinal peptide, somatostatins and neuropeptide Y, have been documented in the literature (6). In case of catechol-amines-secreting ganglioneuromas, hypertensive spells upon tumor manipulation have been reported during resection of metabolically active ganglioneuromas, a preoperative alpha blockade might be indicated and can safely performed (1). MRI is considered the investigation of choice. Ganglioneuromas may show as heterogeneous mass on T2-weighted MRI (4). Optimal management for ganglioneuromas is complete surgical resection. Adjuvant chemotherapy or radiotherapy is not indicated due to the benign nature of disease (4,7). In our case the posterior approach gave excellent access to the lesion and facilated its full excision. In the literature, this approach was indicated due to the involvement of sacral nerves roots (4). Recurrence and malignant transformation of these tumors is rare and surgical excision provides effective cure.

Conclusion

Ganglioneuromas are rare tumors of neurogenic origin that are slow growing and remain dormant for years. The diagnosis is often suggested by characteristics features on imaging and is confirmed at histologic examination. Complete excision is the treatment of choice with low rates of recurrence in the long term.

 

Figure 1: Left buttock mass

 

 

Figure 2: Magnetic resonance imaging showing mass neighbouring to the sacrum and lumbosacral junction

 

 

Figure 3: The tumor exposed via a posterior approach

 

 

Références

  1. Zaghal A, Pitcher G, Rooney S, Hitt S, Kirby P, Shilyansky J: Cathecholamine-secreting extra-adrenal pelvic ganglioneuroma in a child presenting with diaphores: A case report and review of literature. J Ped Surg Case Reports. 2 (7): 373-6, 2014
  2. Ozkara E, Ozbek Z, Baspinar M, Yildirim GD, Atasoy MA, Arslantas A, Presacral Ganglioneuroma: Case Report. WScJ, 5(3),135-9, 2014
  3. Dimou J, Russel JH, Jithoo R, Pitcher M. Sacral ganglioneuroma in a 19-year-o woman. J Clin Neurosci 16(12): 1692-4, 2009
  4. Lynch NP, Neary PM, Fitzgibbon JF, Andrews EJ. Successful management of presacral ganglioneuroma: A case report and a review of the literature. Int J Surg Case Rep 4(10): 933-5, 2013
  5. Balaj C, Oliver A, Lemarie C, Hubert J, Laurent V, Regent D. Retroperitoneal ganglioneuroma revealed as an ‘’incidentaloma’’ in a healthy volunteer. Diagnostic and Interventional Imaging 96:93-6, 2015

6. Zugor V, Schott GE, Khün R,Labanaris AP. Retroperitoneal Ganglioneuroma in childhood-A Presentation of two Cases. Pediatr Neonatal 50(4):173-6, 2009

7. Subramanya K, Betty A, Usha K, Kanishka D. Sporadic synchronous ganglioneuromas in a child –case report and review. J Ped Surg 45(4): 822-5, 2010