CASE REPORT
Primary cholangiocarcinoma of the adrenal gland: a surgical and diagnostic challenge - a case report
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Merary Z. Nazario-Perez1*, Krystell E. Ceballos-Alvarez3, Guillermo Gonzalez-Figueroa2,4, Itzamar Pastrana-Echevarria1, Liza Vazquez5 and Gilberto Ruiz-Deya2,4
Abstract
Background Malignancies of the adrenal gland account for a minority of adrenal incidentalomas, with metastases being more common than primary tumors. Cholangiocarcinoma, an aggressive malignancy of the bile ducts, rarely metastasizes to the adrenal gland, and to date, no cases of primary cholangiocarcinoma originating in the adrenal gland have been reported. This case presents a novel and unprecedented tumor origin, offering valuable insights into diagnostic challenges and the utility of molecular profiling in rare adrenal neoplasms.
Case presentation A 44-year-old female with a history of hypertension, obstructive sleep apnea, and bariatric surgery presented with progressive voiding dysfunction. Imaging revealed a right adrenal mass with radiologic features suspicious of adrenocortical carcinoma. Biochemical evaluation for a functional tumor was unremarkable. She underwent laparoscopic adrenalectomy. Histopathology revealed metastatic adenocarcinoma with an immunoprofile initially suggestive of a pancreatic primary. However, further molecular analysis using AI-driven genomic profiling indicated a 91% probability of cholangiocarcinoma. Despite comprehensive post-operative imaging, including PET-CT and MRCP, no primary hepatic, pancreatic, or biliary tumor was identified, supporting the diagnosis of a primary adrenal cholangiocarcinoma. The patient was treated with six cycles of gemcitabine, cisplatin, and durvalumab, which was later discontinued due to thyroiditis. Follow-up imaging revealed no residual or metastatic disease.
Conclusions This case represents the first known report of cholangiocarcinoma arising in the adrenal gland, broadening the differential diagnosis for adrenal incidentalomas. It underscores the critical role of advanced histopathologic and genomic profiling in evaluating atypical adrenal lesions, especially in patients without a known primary malignancy. Comprehensive diagnostic workup and a multidisciplinary approach are essential for accurate diagnosis and appropriate management of rare adrenal tumors.
Keywords Adrenal incidentaloma, Cholangiocarcinoma, Adrenal metastasis, Primary adrenal tumor, Molecular profiling
*Correspondence: Merary Z. Nazario-Perez merarynazarioperezmd@gmail.com
1Department of Graduate Medical Education, St. Luke’s Hospital, Research Fellow, 917 Av. Tito Castro, Ponce 00733, Puerto Rico
2St. Luke’s Hospital, Urology Residency , 917 Av. Tito Castro, Ponce 00733, Puerto Rico
3Ponce Health Sciences University, School of Medicine, 388 Calle Luis F, Ponce 00716, Puerto Rico
4Department of Surgery, Ponce Health Sciences University, 388 Calle Luis F, Ponce 00716, Puerto Rico 5Hospital Damas, 2213 Ponce By Pass, Ponce PR 00717-1318, Puerto Rico
☒ BMC
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Background
Adrenal incidentalomas are frequently detected in imag- ing studies, with an estimated prevalence of 1% to 6% in the general population. This prevalence increases with age due to the widespread use of imaging and advance- ments in diagnostic technology [1, 2]. Most adrenal incidentalomas are benign and nonfunctioning, with nonfunctioning cortical adenomas comprising approxi- mately 75% of cases [2]. However, adrenal metastases from cholangiocarcinoma (CCA) are exceptionally rare, posing significant diagnostic challenges.
The initial evaluation of an adrenal incidentaloma requires a comprehensive clinical assessment, biochemi- cal testing, and imaging studies. Computed tomography (CT) and magnetic resonance imaging (MRI) play a cru- cial role in differentiating benign from malignant adrenal masses based on lesion size, density, and contrast wash- out characteristics [3]. Additionally, biochemical test- ing is essential for identifying hormonally active tumors, including plasma or urinary catecholamines for pheo- chromocytoma, the aldosterone-renin ratio for primary aldosteronism, and the dexamethasone suppression test for cortisol excess [4, 5].
Management decisions for adrenal incidentalomas depend on these evaluations. According to the 2023 European Society of Endocrinology and ENSAT guide- lines, routine imaging or biochemical follow-up is not recommended for adrenal incidentalomas that are ≤ 4 cm, nonfunctioning, and have benign imaging charac- teristics, unless there are clinical or imaging uncertain- ties [Eur J Endocrinol. 2023;189:G1-G42]. In contrast, hormonally active or suspicious masses may necessitate surgical removal [2, 6]. CCA is an aggressive malignancy of the bile ducts, classified into intrahepatic, perihilar, and distal subtypes [7]. CCA metastasizing to the adre- nal gland is exceedingly uncommon, and primary adre- nal CCA has not been previously reported, making this case particularly unique. While the liver is the most com- mon site of metastasis, the disease can also spread to the lungs, peritoneum, and bones [8]. However, adrenal involvement remains exceedingly uncommon, further complicating diagnosis.
Distinguishing between a primary adrenal tumor and metastatic disease is challenging using standard imag- ing alone. Advanced diagnostic tools, such as positron emission tomography-computed tomography (PET- CT), histopathology, and immunohistochemistry, are often necessary for a definitive diagnosis. However, limited access to these tests may contribute to delayed diagnosis or misclassification [9]. A case reported by Nugroho et al. (2020) described a patient with com- bined hepatocellular-cholangiocarcinoma (cHCC-CC) who initially presented with adrenal metastasis, high- lighting the uncommon metastatic pattern of CCA
[10]. Expanding on this rare occurrence, our case report describes a patient with an adrenal inciden- taloma ultimately diagnosed as CCA. This case under- scores the importance of a thorough evaluation of incidental adrenal lesions, particularly in patients without a known history of malignancy. Recognizing these uncommon metastatic pathways is critical for timely diagnosis and optimal patient management.
Case presentation
A 44-year-old female with a medical history of arterial hypertension, obstructive sleep apnea, and morbid obe- sity status post bariatric surgery three years prior pre- sented to the emergency department with right flank pain and progressive voiding dysfunction over the past year. She denied any personal history of malignancy. Her social history was unremarkable. Family history was notable for her father’s diagnosis of colonic adenocarci- noma at age 68.
During the workup, cross-sectional imaging inciden- tally revealed a right adrenal mass. The patient was ini- tially referred to General Surgery for further assessment, and subsequently to Urology for comprehensive evalua- tion of the adrenal lesion. An abdominopelvic MRI was obtained to further characterize the adrenal mass and guide management based on current risk stratification criteria.
An abdominopelvic computed tomography (CT) scan performed in September 2023 revealed a well-circum- scribed, heterogeneous mass arising from the right adre- nal gland, measuring 4.9×4.4 cm, with a density of 33 Hounsfield units (HU) (Fig. 1). The lesion demonstrated areas of internal density variation consistent with its het- erogeneous nature. There was no evidence of gross cal- cifications, intralesional fat, or hemorrhage. Additionally, the mass maintained clear fat planes with surrounding retroperitoneal structures, and no invasion into adjacent tissues was observed.
Repeat magnetic resonance imaging (MRI) 60 days later showed interval enlargement of the mass to 7×4.1×6.9 cm, with lobulated contours, heterogeneous enhancement, and mixed T2 hyperintensity and T1 hypointensity-features concerning adrenocortical carci- noma (ACC) (Fig. 2).
In accordance with NCCN guidelines and current rec- ommendations for the evaluation of adrenal incidentalo- mas, metabolic assessment was performed to exclude the presence of a hormonally active neoplasm, particu- larly given the lesion’s heterogeneous appearance and size exceeding 4 cm, both of which are considered radio- logic criteria warranting further biochemical investiga- tion. The endocrine workup revealed a morning serum cortisol level of 1.1 ug/dL following the 1-mg overnight dexamethasone suppression test, which is below the
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widely accepted threshold of 1.8 µg/dL (50 nmol/L). This result effectively excludes autonomous cortisol secre- tion, cortisol excess, and overt Cushing’s syndrome. Screening for primary aldosteronism, performed due to clinical suspicion and lesion characteristics, revealed a serum aldosterone concentration of 13.3 ng/dL (refer- ence: 7-30 ng/dL) and an upright plasma renin activity of 2.2ng/ml/hr (reference: 0.5-4.0.5.0 ng/ml/hr), yielding an aldosterone-to-renin ratio (ARR) of 6.68. This value falls below the commonly accepted diagnostic threshold
(ARR>20-30), thereby excluding primary aldosteronism. Additionally, evaluation of androgen secretion showed normal total testosterone (14.4 ng/dL) (reference: 15-70 ng/dL) and free testosterone (0.346 pg/mL) (reference: 0.1-6.4 pg/mL) levels, with no biochemical evidence of androgen excess. Moreover, the DHEA-S level (50 µg/ dL) (reference: 32-240 µg/dL) is within normal limits, arguing against a significant androgen-secreting adrenal tumor. The estradiol level (60 pg/mL) (reference: 0-30 pg/mL) is mildly elevated above the expected range for
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a postmenopausal female. However, in the context of the patient’s morbid obesity, this finding is likely due to increased peripheral aromatization of androgens to estrogens in adipose tissue-a known physiological effect in obese individuals. Finally, a 24-hour urine collec- tion for fractionated metanephrines and catecholamines demonstrated values of 196 µg/24 hr and <6 µg/24 hr, respectively. Both results were within normal lim- its (metanephrine upper limit 220 µg/24 hr; catechol- amines: norepinephrine as <90 µg/24 hr, epinephrine as
<20 µg/24 hr, dopamine <500 µg/24 hr in healthy adults). These findings excluded catecholamine hypersecretion consistent with pheochromocytoma. Collectively, these findings support a non-functioning adrenal inciden- taloma in accordance with current guideline-based. criteria.
Moreover, laboratory studies showed mild microcytic anemia, and tumor markers-including lactate dehydro- genase (LDH), cancer antigen (CA) 19-9, and carcino- embryonic antigen (CEA)-were within normal ranges, except for a mildly elevated cancer antigen (CA)125 at 108 U/mL.
Given the suspicion of malignancy, a laparoscopic right adrenalectomy was performed via a transperitoneal approach. The surgery was uneventful, and the patient was discharged on postoperative day two without complications. At her 2-week follow-up, she reported a complete resolu- tion of symptoms and no postoperative concerns.
Intraoperatively, the mass was well-encapsulated and clearly separated from surrounding structures, with no anatomical or vascular connection to the liver or adja- cent organs. There was no evidence of infiltration into the liver, and the adrenal gland was completely excised with an intact capsule. The mass was encased within the adre- nal gland, and no macroscopically abnormal tissue was seen in adjacent structures.
Histopathologic examination confirmed that the tumor was confined to the adrenal gland and sur- rounded by an intact capsule. The adjacent adrenal tis- sue was partially preserved, with identifiable normal adrenal cortex tissue at the periphery. There was no evidence of ectopic liver tissue or bile ducts within the tumor or surrounding areas. The absence of hepatic or biliary structures, along with the presence of adrenal tissue, supports the exclusion of a metastatic exten- sion from a nearby hepatobiliary tumor. The surgical margins were clear, indicating a complete (R0) resec- tion. Despite this, the tumor demonstrated glandular features on microscopic evaluation, initially suggestive of a pancreatic origin (Fig. 3).
Immunohistochemistry demonstrated positivity for CK7, CK19, CA 19-9, and Villin, with negativity for CK20, SATB2, and PAX8-markers that helped rule out renal or pancreatic primaries. Genomic profiling using CARIS analysis showed a tumor mutational burden (TMB) of 3, microsatellite stability, and an artificial intel- ligence-driven genomic profile (GPSai) indicating a 91% probability for cholangiocarcinoma.
Further investigations, including PET-CT and mag- netic resonance cholangiopancreatography (MRCP), did not reveal a primary tumor elsewhere, supporting the diagnosis of a primary adrenal cholangiocarcinoma. Furthermore, given the absence of any biliary or gallblad- der abnormalities on cross-sectional imaging (CT, MRI,
Adrenal Tissue
Suspected Adenocarcinomatous Tissue
MRCP, PET-CT) and the intraoperative finding of a well- encapsulated adrenal mass without macroscopic involve- ment of the liver of biliary tree, an ERCP with biopsy was not pursue, as it was not considered clinically indicated. These findings, along with histopathology demonstrating preserved adrenal cortex at the tumor periphery and no ectopic bile ducts, further supported exclusion of a bili- ary primary.
The patient was started on gemcitabine and cisplatin chemotherapy in combination with durvalumab, follow- ing National Comprehensive Cancer Network (NCCN) guidelines [8]. After six cycles, treatment was discon- tinued due to immune-related thyroiditis, which mani- fested as severe, overt hypothyroidism unresponsive to interval titration and eventual maximal dosing of thyroid hormone replacement therapy. Given the persistence of debilitating symptoms despite optimized endocrine management, and in the absence of primary disease, metastatic involvement, or recurrence on imaging and laboratory evaluation, the multidisciplinary team elected to discontinue immunotherapy. Follow-up imaging showed no evidence of recurrence or metastasis, and the patient remains clinically well. The patient is currently maintained on surveillance with clinical evaluation and imaging every 6 months.
Discussion
This case highlights an exceptionally rare presentation of cholangiocarcinoma (CCA) as an adrenal incidentaloma. To date, there have been no documented cases of chol- angiocarcinoma originating within the adrenal gland, making this a remarkable and novel finding. While CCA is known to metastasize primarily to the liver, lungs, peri- toneum, and bones, adrenal involvement is exceedingly rare.
The literature includes a single case of liver cholangio- carcinoma metastasizing to the adrenal gland, but no reports of a primary tumor arising in this location have been published. Adrenal incidentalomas are frequently encountered in clinical practice, with an estimated preva- lence of 2% in the general population, increasing to over 7% in individuals older than 70 years. Most of these lesions are benign, though approximately 7.2% are malig- nant, including both primary adrenal tumors and meta- static disease [11]. Among malignant adrenal tumors, metastases are second only to benign adenomas in fre- quency and often originate from lung, breast, kidney, or gastrointestinal malignancies.
In this case, the patient initially presented with pro- gressive voiding dysfunction, and imaging revealed a large adrenal mass lacking typical benign features. CT and MRI raised suspicion for a lipid-poor adenoma or adrenocortical carcinoma (ACC), prompting fur- ther evaluation (Figs. 1 and 2). A negative biochemical
workup ruled out functional adrenal tumors, and tumor markers-including CA-125, LDH, CA 19-9, and CEA-were essentially within normal limits, except for a mildly elevated CA-125. Given the uncertain diagnosis, surgical excision was necessary for definitive pathological assessment.
Histopathological examination initially suggested met- astatic adenocarcinoma, with features favoring a pan- creatic primary (Fig. 3). However, immunohistochemical analysis provided crucial findings: the tumor was posi- tive for CK7, CK19, CA 19 - 9, and Villin-markers commonly associated with cholangiocarcinoma-while CK20, PAX8, and SATB2 were negative, ruling out pan- creatic and renal origins. Although steroidogenic factor- 1(SF-1) and synaptophysin, markers commonly used to evaluate adrenocortical origin, were not included in the immunohistochemical panel, the staining profile strongly supported a pancreatobiliary phenotype [12]. Addition- ally, p53 demonstrated a wild-type pattern, and although B-catenin immunostaining was not included in the panel, its absence limits further assessment of Wnt/B-catenin pathway involvement [13]. This is relevant, as nuclear accumulation of ß-catenin is a hallmark finding in a sub- set of ACCs due to CTNNB1 mutations. Moreover, TERT immunostaining was also not performed; however, TERT promoter mutations are more frequently associated with ACC and have also been implicated in aggressive hepato- biliary malignancies [14].
To further confirm the tumor’s origin, the tissue under- went genomic profiling through AI-driven analysis, which estimated a 91% probability that the malignancy arose from cholangiocarcinoma, supporting the diagno- sis of primary adrenal CCA. Although imaging failed to identify a primary tumor elsewhere, and immunopro- filing supported CCA, it remains possible that a small, undetectable primary site existed, given the known meta- static behavior of cholangiocarcinoma.
Another differential diagnosis worth to mention although rare is ectopic liver tissue. While most ectopic liver rests remain benign, a significant fraction undergo malignant transformation, most commonly to hepatocel- lular carcinoma (HCC), particularly in tissue lacking nor- mal vascular or biliary architecture. Several cases have also reported carcinogenesis arising in ectopic hepatic tissue mimicking primary adrenal tumors [15]. In our case, there was no histological evidence of hepatocytes or bile ducts within the adrenal mass, helping exclude this rare but important differential diagnosis.
This case underscores the importance of maintaining a broad differential diagnosis when evaluating adrenal incidentalomas, particularly in patients without a known history of malignancy. While CT and MRI are valuable first-line tools in assessing adrenal masses, they may not always reliably distinguish between benign and malignant
lesions. As demonstrated in this case, histopathological analysis, immunohistochemistry, and molecular diag- nostics are critical for accurate tumor characterization and should be considered when imaging findings are inconclusive.
In terms of management, the patient was treated in accordance with guidelines for metastatic cholangiocar- cinoma and was initiated on gemcitabine and cisplatin chemotherapy in combination with durvalumab immu- notherapy. This regimen has been shown to improve overall survival, progression-free survival, and response rates in patients with advanced biliary tract cancers [16]. Given the aggressive nature of CCA, early recognition and appropriate treatment selection remain crucial to optimizing patient outcomes.
Patient perspective
“When I first started having trouble with urination, I never imagined it could be anything serious. After the doctors found a mass on my adrenal gland, I was scared and anxious about what it could mean. The thought of having cancer was overwhelming, but my medical team explained everything clearly and sup- ported me through each step.
I’m grateful that the surgery went smoothly and the recovery was quicker than expected. Finding out that the tumor was likely a rare type of cancer was shock- ing but knowing that the team used advanced test- ing to find the correct diagnosis gave me confidence. Starting chemotherapy was difficult, but I’m thank- ful for the ongoing support from my doctors. I now feel hopeful about my future and trust that I’m in good hands.”
Conclusion
This case represents the first documented instance of primary cholangiocarcinoma originating in the adrenal gland. This underscores the importance of comprehen- sive diagnostic workup, including genomic profiling, in evaluating adrenal incidentalomas. Surgical removal allowed fordefinitive diagnosis and oncologic control, highlighting the importance of considering molecular profiling in atypical adrenal tumors. Further research into the carcinogenic mechanisms underlying adrenal CCA is warranted.
Abbreviations
| ACC AI | Adrenocortical carcinoma Artificial intelligence |
|---|---|
| CA 19-9Cancer antigen 19-9 | |
| CA-125 | Cancer antigen 125 |
| CCA | Cholangiocarcinoma |
| CEA | Carcinoembryonic antigen |
| CK | Cytokeratin |
| CT | Computed tomography |
FDG Fluorodeoxyglucose
GPSai Genomic profile similarity artificial intelligence
HU Hounsfield unit
LDH Lactate dehydrogenase
MRI Magnetic resonance imaging
MRCP Magnetic resonance cholangiopancreatography
NCCN National Comprehensive Cancer Network
PET-CT Positron emission tomography-computed tomography
TMB Tumor mutational burden
Acknowledgements
Not applicable.
Use of Large Language Models (LLMs)
A large language model (ChatGPT, OpenAI) was used to assist with grammar checking. The authors reviewed and approved all content.
Authors’ contributions
M.N.P. contributed to the abstract, introduction, discussion, conclusion, and case history section. M.N.P. created, labeled, and described the figures; organized the references and ensured proper in-text citation; prepared the manuscript according to journal guidelines; and finalized the manuscript for submission.K.C.A. contributed to the introduction and discussion, and reformatted the references according to journal requirements.G.G.F. contributed to the abstract and conclusion, and provided key input to the case presentation and discussion.G.R.D. reviewed and edited all sections of the manuscript, added the SCARE guideline statement, and composed the Patient Perspective section.L.V. contributed to the case presentation and discussion sections, and reviewed the final manuscript.All authors reviewed and approved the final manuscript and agree to be accountable for the accuracy and integrity of the work.
Funding
The authors received no specific funding for this work.
Data availability
All data generated or analyzed during this study are included in this published article.
Declarations
Ethics approval and consent to participate
This case report was reviewed and deemed exempt by the Institutional Review Board of Ponce Health Sciences University (IRB Reference Number: 2501235062). All procedures were conducted in accordance with the ethical standards of the Institutional Review Board.
Consent for publication
Written informed consent was obtained from the patient for publication of this case report and any accompanying images.
Competing interests
The authors declare no competing interests.
Received: 22 March 2025 / Accepted: 7 October 2025
Published online: 22 November 2025
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