Case Reports in Oncology
Case Rep Oncol 2025;18:1057-1062
DOI: 10.1159/000547075
Received: March 14, 2025
Accepted: June 16, 2025
Published online: June 25, 2025
@ 2025 The Author(s). OPEN Published by S. Karger AG, Basel www.karger.com/cro
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Case Report
Adrenal Incidentaloma Masquerading as Malignancy: A Rare Case of Ganglioneuroma with Nodal Metastasis
Shahrukh Memonª Saurabh Rajb Rudrakshi Mahaldarb
ªDepartment of Urology, Nizam Institute of Medical Science, Hyderabad, India; bDepartment of General Surgery, All India Institute of Medical Sciences, Kalyani, India
Keywords
Adrenal ganglioneuroma · Incidentaloma . Lymph node metastasis · Adrenocortical carcinoma mimic . Surgical management
Abstract
Introduction: Adrenal ganglioneuromas are rare benign tumours, accounting for 0.3%-2% of adrenal incidentalomas. They arise from Schwann and ganglion cells and often mimic adrenal cortical carcinoma clinically and radiologically. Despite their benign nature, met- astatic deposits in lymph nodes have been reported, suggesting tumour maturation. Di- agnosis is typically confirmed through postoperative histopathology. Case Presentation: A male patient in his early 60s with a history of hypertension presented with an adrenal incidentaloma. Imaging revealed a well-defined, hypodense right adrenal mass (35 HU) with calcifications and an enlarged aortocaval lymph node, raising suspicion for carcinoma. Biochemical analysis showed a non-functional adrenal tumour. Intraoperatively, the mass appeared benign, but the lymph node deposit suggested malignancy. Histopathological examination confirmed right adrenal ganglioneuroma with metastatic deposits in the aortocaval lymph nodes. The patient recovered well postoperatively, with normal serum cortisol levels and no signs of recurrence at 6-month follow-up. Conclusion: Adrenal ganglioneuromas can present as adrenal incidentalomas with lymph node involvement, mimicking malignancy. Surgery remains the gold standard for treatment, and postoperative histopathology is crucial for diagnosis. In such cases, no adjuvant therapy or stringent follow-up is required after surgical removal.
@ 2025 The Author(s). Published by S. Karger AG, Basel
Karger’s
Case Rep Oncol 2025;18:1057-1062
DOI: 10.1159/000547075
@ 2025 The Author(s). Published by S. Karger AG, Basel www.karger.com/cro
Memon et al .: Ganglioneuroma with Nodal Metastasis: A Diagnostic Dilemma
Case Reports in Oncology
Introduction
Adrenal ganglioneuromas are rare, benign tumours, accounting for approximately 0.3%-2% of all adrenal incidentalomas [1]. They originate from benign neuroectodermal cells, specifically Schwann and ganglion cells. Clinically and radiologically, adrenal ganglioneuromas often mimic adrenocortical carcinoma, presenting a diagnostic challenge. These tumours are typically meta- bolically silent, which further complicates the clinical picture. On imaging, an unenhanced atten- uation value of less than 40 Hounsfield units (HU) and stippled calcifications should prompt consideration of this diagnosis [2]. Despite their benign nature, distinguishing ganglioneuromas from malignant adrenal masses based on imaging alone remains difficult. Consequently, the ma- jority of adrenal ganglioneuromas are diagnosed postoperatively through histopathological ex- amination. Histological analysis is crucial to confirm the tumour’s benign nature and exclude the possibility of malignancy or metastatic disease. Several case series and reviews have detailed imaging features, clinical presentation, and outcomes of adrenal ganglioneuromas, emphasizing their variable radiological appearances and the importance of histopathological confirmation [3-5]. Given their rarity and overlapping presentation with malignant adrenal tumours, adrenal gan- glioneuromas require a high index of suspicion for accurate diagnosis and appropriate management.
Case Presentation
A male patient in his early 60s with a 4-year history of hypertension presented with an adrenal incidentaloma. The case was investigated through detailed radiological and biochemical evaluation. Radiological imaging revealed a well-defined, hypodense lesion in the right adrenal gland, mea- suring 4.8 × 5.0 × 6.7 cm, with an unenhanced attenuation of +35 Hounsfield units (HU) and moderate enhancement in the delayed phase (+54 HU). Stippled calcifications were noted, along with an enlarged aortocaval lymph node measuring 1.7 x 0.8 cm, raising suspicion for adreno- cortical carcinoma (Fig. 1, 2). Biochemical analysis indicated a non-functional adrenal tumour.
Surgical exploration was performed to assess the nature of the mass and its relationship to adjacent structures. Intraoperatively, the tumour measured approximately 6 x 7 cm, was well encapsulated, and lacked neovascularity or infiltration into surrounding tissues. A solitary, firm inter-aortocaval lymph node (2 cm) was identified. Gross cut section analysis was performed (Fig. 3).
Histopathological examination confirmed a diagnosis of right adrenal ganglioneuroma compressing normal adrenal tissue, with no signs of atypia, mitosis, blastemal component, or necrosis (Fig. 4, 5). Interestingly, metastatic deposits of ganglioneuroma were identified within the aortocaval lymph node (Fig. 6).
Postoperatively, the patient showed uneventful recovery but remained hypertensive, with normal early morning serum cortisol levels. The surgical drain was removed on the second postoperative day, and the patient was discharged the same day. The patient was monitored for 6 months because there is no established follow-up protocol for adrenal ganglioneuromas. Abdominal imaging and biochemical analysis were routine at 3 and 6 months, with no evidence of recurrence.
Discussion
Adrenal ganglioneuromas are rare, benign tumours that can present as adrenal in- cidentalomas. Although they are usually metabolically silent, approximately 30% of adrenal ganglioneuromas may present with elevated catecholamine levels [6]. In this case, the
Karger’s
Case Reports in Oncology
@ 2025 The Author(s). Published by S. Karger AG, Basel www.karger.com/cro
Memon et al .: Ganglioneuroma with Nodal Metastasis: A Diagnostic Dilemma
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patient presented with an adrenal incidentaloma and regular biochemical markers, which is consistent with the typical presentation of adrenal ganglioneuroma.
Clinically and radiologically, the tumour closely resembled adrenocortical carcinoma with lymph node metastasis due to the presence of a hypodense adrenal mass with cal- cifications and an enlarged aortocaval lymph node. Although PET-CT is a valuable modality for assessing adrenal masses and evaluating metastatic spread, it was not performed in our case due to institutional constraints. Additionally, the CT findings were already strongly suggestive of malignancy, and a decision was made to proceed with surgical excision without delay. However, intraoperative findings were ambiguous; the adrenal mass appeared be- nign, while the lymph node deposit suggested malignancy. The final histopathological ex- amination confirmed the diagnosis of right adrenal ganglioneuroma compressing normal adrenal tissue, with metastatic deposits of ganglioneuroma in the aortocaval lymph nodes.
A literature review indicates that adrenal ganglioneuromas with lymph node deposits suggest tumour maturation rather than true metastasis [7]. The reported incidence of lymph node deposits in adrenal ganglioneuromas is low. In a study by Shawa et al. [8] approxi- mately 7% of patients exhibited nodal involvement, interpreted as ganglioneuromatous
Karger’?
Case Reports in Oncology
Case Rep Oncol 2025;18:1057-1062
DOI: 10.1159/000547075
@ 2025 The Author(s). Published by S. Karger AG, Basel www.karger.com/cro
Memon et al .: Ganglioneuroma with Nodal Metastasis: A Diagnostic Dilemma
Karger’?
| Case Reports | Case Rep Oncol 2025;18:1057-1062 | |
|---|---|---|
| DOI: 10.1159/000547075 | @ 2025 The Author(s). Published by S. Karger AG, Basel www.karger.com/cro | |
| in Oncology | Memon et al .: Ganglioneuroma with Nodal Metastasis: A Diagnostic Dilemma | |
maturation rather than actual metastatic spread, reinforcing the benign nature of these lesions. This reinforces the benign nature of the tumour despite the presence of lymph node involvement. Surgery remains the gold standard for treating adrenal ganglioneuroma [3, 8]. Postoperatively, the patient recovered well and remains under regular follow-up with no subsequent need for adjuvant therapy, consistent with the expected benign course of the disease.
A multicenter series of 27 adrenal ganglioneuromas highlighted their benign clinical course and imaging variability, often leading to diagnostic uncertainty and unnecessary adrenalectomies [3]. Additional reports support our findings of occasional lymph node involvement interpreted as tumour maturation rather than true malig- nancy [4, 5].
Conclusion
Adrenal ganglioneuromas are benign tumours that may present with lymph node deposits, representing tumour maturation rather than true metastasis. They can mimic adrenocortical carcinoma clinically and radiologically, necessitating consideration in dif- ferential diagnosis. Surgery is the mainstay of treatment, with postoperative histopathology confirming diagnosis. Follow-up and adjuvant therapy are typically unnecessary. The au- thors have completed the CARE Checklist for this case report, which is attached as online supplementary Material (for all online suppl. material, see https://doi.org/10.1159/ 000547075).
Patient’s Perspective
I felt relieved after the surgery and thankful that it was not cancer. The follow-up visits gave me peace of mind, and knowing I did not need more treatment made me feel confident.
Acknowledgments
We want to thank Grammarly (https://app.grammarly.com) for editing the English language.
Statement of Ethics
All procedures were performed by the 1964 Declaration of Helsinki’s ethical standards and later amendments. Ethical approval is not required for this study by local or national guidelines. All adult participants provided written informed consent to participate in this study. Written informed consent was obtained from the individual(s) to publish the details of their medical care and any accompanying images.
Conflict of Interest Statement
The authors have no conflicts of interest to declare.
Karger’«
Case Rep Oncol 2025;18:1057-1062
DOI: 10.1159/000547075
@ 2025 The Author(s). Published by S. Karger AG, Basel www.karger.com/cro
Memon et al .: Ganglioneuroma with Nodal Metastasis: A Diagnostic Dilemma
Case Reports in Oncology
Funding Sources
The authors did not receive any funding for this research.
Author Contributions
Conceptualization and writing - review and editing: S.M .; data curation, resources, validation, and supervision: S.M., S.R., R.M .; formal analysis, project administration, writing - original draft, and visualization: S.R. and S.M .; investigation: S.M. and R.M .; approval of final manuscript: all authors.
Data Availability Statement
All data generated or analysed during this study are included in this article and its online supplementary material. Further inquiries can be directed to the corresponding author.
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