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 ¤ case report
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UNUSUAL CASE
Year : 2021  |  Volume : 17  |  Issue : 3  |  Page : 389-391
 

Ganglioneuroma presenting as an adrenal incidentaloma: Feasibility of adrenal-sparing surgery


Department of Urology, All India Institute of Medical Science, Rishikesh, Uttarakhand, India

Date of Submission26-Jul-2020
Date of Decision29-Sep-2020
Date of Acceptance13-Jan-2021
Date of Web Publication06-May-2021

Correspondence Address:
Dr. Ankur Mittal
Department of Urology, All India Institute of Medical Science, Rishikesh - 249 202, Uttarakhand
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jmas.JMAS_147_20

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 ¤ Abstract 

Adrenal ganglioneuromas (GNs) are very rare tumours that originate from neural crest cells. Most of the time, they are diagnosed incidentally as they are usually non-functional and remain asymptomatic. Nowadays, they are being detected more often due to better availability of imaging facilities such as computed tomography (CT)/magnetic resonance imaging (MRI). Minimally invasive adrenalectomy (laparoscopic or robotic) remains the standard of care for such lesions. Hereby, we report a case of a 15-year-old young girl with right adrenal incidentaloma which was diagnosed on CT with the features suggestive of GN. She underwent robot-assisted excision of the mass with adrenal-sparing surgery. Histopathology revealed benign GN and no adjuvant treatment was required. As GN is not known for recurrence or metastasis, minimal invasive adrenal-sparing surgery should be a preferred modality of choice.


Keywords: Adrenal-sparing surgery, adrenal tumour, adrenalectomy, ganglioneuroma, incidentaloma, robotic surgery


How to cite this article:
Ranjan R, Mittal A, Ranjan SK, Panwar V, Talwar HS. Ganglioneuroma presenting as an adrenal incidentaloma: Feasibility of adrenal-sparing surgery. J Min Access Surg 2021;17:389-91

How to cite this URL:
Ranjan R, Mittal A, Ranjan SK, Panwar V, Talwar HS. Ganglioneuroma presenting as an adrenal incidentaloma: Feasibility of adrenal-sparing surgery. J Min Access Surg [serial online] 2021 [cited 2021 Aug 3];17:389-91. Available from: https://www.journalofmas.com/text.asp?2021/17/3/389/315528



 ¤ Introduction Top


Adrenal incidentalomas are quite common because of better and easy availability of imaging facilities.[1] Most of them are benign and non-functioning in nature.[2] These masses can be evaluated better by adrenal protocol computed tomography (CT).[2] Treatment protocol is different for every adrenal mass, with gold standard being adrenalectomy. Adrenal ganglioneuroma (GN) accounts for 0.2%–0.5% cases of adrenal incidentalomas.[3] Here, we present a rare case of benign and non-functional adrenal GN, managed by robot-assisted laparoscopic excision of an adrenal mass, an adrenal-sparing approach. Intra- and post-operative period was uneventful. No recurrence was seen on further 1 year of follow-up.


 ¤ case report Top


A 15-year-old girl presented to our outpatient department with right adrenal incidentaloma documented on ultrasound done elsewhere. She had undergone fine needle aspiration cytology which revealed a benign adrenal mass. General physical and systemic examination was normal. Functional metabolic work-up including serum metanephrine, normetanephrine and low-dose dexamethasone suppression test cortisol was all unremarkable. Adrenal protocol CT showed an ill-defined heterogeneously enhancing suprarenal mass measuring 39 mm × 48 mm × 49 mm arising from the lateral limb of the right adrenal. Few arcuate as well as punctuate foci of central calcification were seen within the lesion with delayed contrast washout [Figure 1]. This adrenal lesion had pre-contrast-34 HU, post-contrast venous-102 HU, delayed-77 HU, absolute washout-43 and relative washout was 24.5; suggestive of GN.
Figure 1: Adrenal protocol computed tomography showing ill-defined heterogeneously enhancing lesion at the right suprarenal region

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She underwent robot-assisted excision of the adrenal mass with adrenal sparing surgery as there was 4 cm × 4 cm mass originating from the lateral limb of the adrenal. Intra-operative and post-operative period was uneventful, and she was discharged on day 1 of surgery. Histopathology revealed a well-circumscribed lesion with mature ganglion and Schwann cells with foci of calcification, suggestive of GN [Figure 2]. Further follow-up till 1 year through clinical examination and ultrasonography was unremarkable.
Figure 2: Histopathology revealed a well-circumscribed lesion with mature ganglion and Schwann cells with foci of calcification

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 ¤ discussion Top


Tumours of the suprarenal gland are extremely rare; however, since the development of advanced imaging modalities, such as high-resolution ultrasonography, CT and magnetic resonance imaging (MRI), these are much more commonly diagnosed.[2],[3] Many of these lesions are asymptomatic and picked up during investigation of an unrelated problem.[2] The incidence of such adrenal incidentaloma is 0.2% in young, while 3% in >50 years of age and 7% in >70 years of age.[1] In >85% of cases, these are benign and non-functioning.[3]

GN is a rare, well-differentiated tumour arising from the neural crest cells.[2] GNs are most typically found in retroperitoneum (32%–52%) and posterior mediastinum (39%–43%) and usually seen in children and younger adults; however, adrenal GNs are most frequently seen in the fourth and fifth decades of life, with an equal preponderance in both sexes.[2],[4] They are discovered incidentally and accounts for 0.3%–2% of all adrenal incidentalomas.[3] Ultrasound usually shows a well-defined homogeneous, hypoechogenic mass in the suprarenal region.[5] CT scan demonstrates low attenuation (<40 HU) and punctate or discrete calcifications (42%–60%) on unenhanced CT and shows homogeneous or heterogeneous attenuation with gradual and delayed enhancement on contrast-enhanced CT.[2],[5] On MRI, it shows low-signal intensity on T1-weighted (T1-W) images and heterogeneously high-signal intensity on T2-W images with gradual and delayed enhancement with contrast.[1],[5] Vessel involvement is not seen in GN.[4] Definitive diagnosis is through histopathological examination.[3]

Adrenalectomy has been the gold standard in the management of adrenal GN.[2],[3] However, with the advent of minimally invasive approach, adrenal-sparing operations have been often reported, even for unilateral tumours. Robotic assistance enhances our armamentarium in accomplishing this approach. Overall, prognosis after surgical resection seems to be excellent, without any recurrences or metastasis or need for adjuvant therapy.[3] Partial adrenalectomy is a safe and feasible option in the hands of appropriately trained surgeons. We thereby recommend organ-sparing approach even in unilateral benign tumours wherever safe and feasible.


 ¤ conclusion Top


GN should be considered as a rare differential diagnosis of any benign looking adrenal mass. As GN is not known for recurrence or metastasis, minimal invasive adrenal-sparing surgery should be a preferred modality of choice.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
 ¤ References Top

1.
Sarwal A, Khullar R, Sharma A, Soni V, Baijal M, Chowbey P. Laparoscopic adrenalectomy for ganglioneuroma presenting as an adrenal incidentaloma. J Minim Access Surg 2019;15:259-61.  Back to cited text no. 1
    
2.
Mylonas KS, Schizas D, Economopoulos KP. Adrenal ganglioneuroma: What you need to know. World J Clin Cases 2017;5:373-7.  Back to cited text no. 2
    
3.
Zhang C, Sun F, Jiang H, Wang L, Zhang Z, Xu Y, et al. Case Report Cystic adrenal ganglioneuroma: A case report and review of the literature. Int J Clin Exp Med 2018;11:10083-7.  Back to cited text no. 3
    
4.
Sucandy I, Akmal YM, Sheldon DG. Ganglioneuroma of the adrenal gland and retroperitoneum: A case report. N Am J Med Sci 2011;3:336-8.  Back to cited text no. 4
    
5.
Shin YR, Kim KA. Imaging features of various adrenal neoplastic lesions on radiologic and nuclear medicine imaging. AJR Am J Roentgenol 2015;205:554-63.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2]



 

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