Endocrinology, Diabetes & Metabolism CASE REPORTS
A nonfunctional adrenal incidentaloma revealed by intestinal obstruction: metastatic adrenocortical carcinoma diagnosed by surgery
Yasemin Keskin®, Pınar Dağ® and Elif Buse Güngör®
Department of General Surgery, Dr Abdurrahman Yurtaslan Ankara Oncology Training and Research Hospital, Ankara, Turkey
Correpondence should be addressed to Y Keskin: konuk.yasemin@gmail.com, yasemin.keskin7@saglik.gov.tr
Summary
This case report describes an 84-year-old male patient who presented with symptoms of intestinal obstruction and was subsequently diagnosed with metastatic adrenocortical carcinoma (ACC), a rare and aggressive endocrine malignancy. The patient arrived at the emergency department with abdominal pain, nausea, vomiting, and bloating. Imaging studies revealed a segmental ileal mass suggestive of obstruction and a large (12 x 8 cm) adrenal mass. Hormonal assays indicated a nonfunctional adrenal incidentaloma. Due to persistent obstruction, the patient underwent surgical resection of the terminal ileum and a left adrenalectomy. Histopathological examination confirmed ACC with metastasis to the small intestine. This rare presentation is notable because ACC typically manifests with hormone excess or mass effect symptoms, and intestinal metastasis causing mechanical obstruction is exceedingly uncommon. Radiologically, the adrenal mass lacked classic features of ACC, contributing to initial diagnostic uncertainty. Postoperatively, the patient recovered uneventfully and was started on mitotane therapy after PET imaging confirmed residual disease and para- aortic lymph node metastasis. This report underscores the importance of thorough evaluation of large adrenal incidentalomas, even if nonfunctional, and highlights intestinal obstruction as a rare but possible initial presentation of metastatic ACC. It contributes to the limited literature documenting atypical clinical manifestations of this rare tumor.
Learning points
· Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy that is frequently diagnosed at an advanced or metastatic stage.
· Nonfunctional adrenal incidentalomas ≥4 cm in size warrant comprehensive hormonal evaluation and imaging, followed by multidisciplinary assessment due to the potential risk of malignancy.
· In this case, despite benign-appearing radiological features, histopathological examination confirmed that the adrenal mass was a metastatic adrenocortical carcinoma.
· RO resection remains the only potentially curative treatment for ACC; in selected cases, synchronous resection of metastatic lesions may be necessary.
· This case highlights a previously undocumented presentation of ACC manifesting as small bowel obstruction due to intestinal metastasis, thereby expanding the clinical spectrum of this rare entity.
Keywords: adrenocortical carcinoma; incidentaloma; metastasis; intestinal obstruction; adrenal tumor
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Background
Adrenocortical carcinoma (ACC) is a rare and aggressive endocrine malignancy with a poor prognosis. It has an incidence of 200 cases per year and a 5-year survival rate of 15-44% (1). Complete resection (RO) through adrenalectomy is the curative treatment option for patients without distant metastases (1). Despite its rarity, ACC often presents with a poor prognosis due to locoregional or metastatic disease at the time of diagnosis.
Due to its rarity, data from studies involving large populations are limited. This case report describes a patient who presented with intestinal obstruction due to ACC metastasis to the terminal ileum. A review of the existing literature revealed no other similar cases of intestinal obstruction. This case report aims to highlight a rare clinical presentation of ACC manifesting as intestinal obstruction at admission to the emergency department, due to intestinal metastasis - a finding not previously documented in the literature.
Case presentation
An 84-year-old male patient presented to the emergency department complaining of abdominal pain and bloating over the previous week, followed by nausea and vomiting. The patient reported a small amount of watery stool discharge over the past three days. In addition to these complaints, the patient reported weakness and loss of appetite. The patient mentioned that he had experienced abdominal bloating and pain one week before presenting to the emergency department.
The patient had a 10-year history of hypertension and had been treated with metoprolol, amlodipine, and acetylsalicylic acid. After being diagnosed with hypertension, the patient underwent coronary angiography, which revealed no pathology. Seven years prior, the patient developed a raised, dark-colored lesion in the left zygomatic region and underwent a wide excisional biopsy. Biopsy results indicated basal cell carcinomas. Due to negative surgical margins in the pathology material after excision, the patient was followed up.
There were no significant features in the patient’s family history.
The patient presented with abdominal distension and hypoactive bowel sounds. Widespread abdominal tenderness was observed; however, no guarding or rebound tenderness was noted. A rectal examination revealed an empty ampulla with minimal stool contamination.
Investigation
The laboratory tests conducted at the time of the patient’s admission showed a white blood cell count of
13.45 × 103/uL (reference range (RR): 4-11 × 103/µL), hemoglobin level of 10.7 g/dL (RR: 13-17.5 g/dL), neutrophil count of 9.66 x 103/uL (RR: 2-7 x 103/uL), creatinine level of 0.4 mg/dL (RR: 0.55-1.25 mg/dL), and C-reactive protein level of 69.7 mg/L (RR: 0-5 mg/L). A complete urinalysis was also performed, which did not reveal any pathology.
A standing abdominal X-ray obtained at the time of admission revealed fluid levels measuring 3.5 cm at the widest part of the base, indicative of a small bowel type (Fig. 1). Based on this finding and the suspicion of intestinal obstruction, abdominopelvic computed tomography (CT) with intravenous contrast was performed. CT showed concentric wall thickening measuring 18 mm at the thickest part over a segment of approximately 6 cm in the ileal loops, along with adjacent fat stranding and several lymphadenopathies measuring up to 2 cm in diameter (Fig. 2). In addition, there was an increase in caliber and minimal fluid volume in the proximal ileal segments adjacent to the aforementioned area. Abdominopelvic CT revealed a suspicious mass in the ileal area.
Furthermore, the CT scan report revealed a contrast- enhanced septated cystic lesion measuring 12 × 8 cm in the medial aspect of the spleen, with borders that were not distinguishable from the left adrenal gland (Fig. 3). In addition, a 42 mm peripherally calcified lesion was observed in the spleen, which was suspected to be a hydatid cyst.
Treatment
Due to the patient’s intestinal obstruction diagnosis, treatment was initiated with the placement of a
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nasogastric decompression tube (NG tube) and a urinary catheter. An incidental mass suspected to be of adrenal origin was considered potentially functional. Due to the risk of possible complications following anesthesia induction and the absence of guarding and rebound tenderness in the patient, emergency surgery was not prioritized.
The patient was referred to the Department of Endocrinology and Metabolic Diseases for investigation of whether the adrenal mass was functional. Blood tests were conducted to assess the levels of adrenocorticotropic hormone (ACTH), cortisol, dehydroepiandrosterone sulfate (DHEA-S), total testosterone, 11-deoxycortisol, and 17-OH-progesterone. Simultaneously, a 24-h urine collection was performed to measure the metanephrine/normetanephrine levels. Except for a low total testosterone level of 78.81 ng/dL (RR: 187-684 ng/dL), no pathological findings were detected on other tests. A dynamic adrenal CT scan was performed to differentiate the adrenal mass that was initially suspected to originate from the adrenal gland. The scan revealed a cystic lesion with fine septations, primarily of adrenal origin, measuring approximately 12 × 8 cm at the level of the left suprarenal recess, with solid components enhanced after intravenous contrast injection, similar to the previous CT findings.
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During the investigations, there were no changes in the patient’s acute abdominal symptoms, and the distension persisted. The nasogastric tube continued to drain approximately 200 cc of gastric content daily. The patient passed gas but did not have significant stool output. Vital signs remained stable, and uncontrolled hypertension was not observed. Upon reassessment with the Department of Endocrinology and Metabolic Diseases, it was determined that the adrenal mass was a non-functional incidentaloma, and the decision was made to proceed with surgery due to the intestinal obstruction.
The patient was scheduled for diagnostic laparotomy due to intestinal obstruction. Given the size of the adrenal mass (12 cm), a decision was made to perform a left adrenalectomy in addition to the planned surgery. The patient was positioned in the supine position, as both ileal resection and adrenalectomy were planned via a transperitoneal approach. During exploration in the operating room, a mass measuring approximately 4 x 4 cm near the ileocecal valve and approximately 60 cm proximal to it was found obstructing the lumen of the small intestine. To maintain surgical field sterility and adhere to the clean-to-contaminated sequence, adrenalectomy was performed first, followed by ileal resection. The adrenal mass was found to not invade any nearby structures and was dissected free from
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surrounding tissues using sharp and blunt dissections before the adrenalectomy was completed. Subsequently, the mass in the terminal ileum was resected along with at least 5 cm margins proximally and distally, including the mesentery of the small intestine, followed by an end-to-end anastomosis (Fig. 4).
Outcome and follow-up
The patient was closely monitored in the intensive care unit during the first 24 h postoperatively. As no complications occurred during monitoring, the patient was transferred to a regular ward for further observation. The patient’s vital signs remained stable, and on the third postoperative day, oral intake gradually increased as stool passage resumed. The patient was discharged on the 10th postoperative day.
The pathology results of the adrenalectomy specimen indicated widespread positivity for vimentin, focal positivity for epithelial membrane antigen (EMA) and calretinin, and negativity for synaptophysin, inhibin, and SF-1, consistent with adrenocortical carcinoma. The resection material from the small intestine was reported as ‘compatible with adrenocortical carcinoma metastasis, with surgical margins containing 29 reactive lymph nodes’.
Following staging, the patient underwent F-18 fluorodeoxyglucose (FDG) positron emission tomography (PET), which revealed findings supporting the presence of residual primary malignancy in the left adrenal gland and metastasis to the para-aortic lymph node. Considering the pathology report and F-18 FDG PET results, the patient was referred to the Medical Oncology Department for the evaluation of adjuvant treatment needs. Due to the patient’s Eastern Cooperative Oncology Group (ECOG) performance status (2) of 2 and the presence of metastatic disease, mitotane therapy was initiated. Adjuvant treatment has recently begun and is being monitored.
Discussion
Intestinal obstruction is defined as a mechanical blockage of intestinal contents caused by physical factors, such as a mass, adhesion, or hernia. In its differential diagnosis, conditions that mimic ileus - such as intra-abdominal abscess or perforation - must be carefully excluded (3). This case report presents a rare instance of intestinal metastasis from ACC leading to mechanical small bowel obstruction, thereby contributing to the limited literature on this uncommon clinical manifestation.
ACC is a rare malignancy with an incidence of 0.7 to 2 cases per million per year (4). ACC can occur at any age, but its peak incidence is between 40 and 60 years of age, with women being the most affected (55-60%). The age-adjusted incidence of ACC has been determined to be 1 case per million population per year (4). Most cases of ACC in adults occur sporadically. However, ACC can sometimes be a part of hereditary syndromes such as Li-Fraumeni syndrome, Lynch syndrome, multiple endocrine neoplasia (MEN) 1, and familial adenomatous polyposis syndrome (5).
ACC at the time of diagnosis is often associated with locally advanced or systemic disease and is defined as a malignancy with a poor prognosis characterized by hormonal hypersecretion, which is considered one of the factors increasing morbidity. Hormonal hypersecretion occurs in 40-74% of ACC cases at diagnosis, tumor syndrome occurs in 40-60%, incidentaloma occurs in 10-20%, and rare paraneoplastic conditions may also occur (6).
The clinical diagnosis of ACC is made by measuring adrenocortical hormone levels and using cross-sectional imaging techniques. In every suspected case, the levels of glucocorticoids, sex hormones, mineralocorticoids, and adrenocortical steroid hormone precursors should be assessed and pheochromocytomas should be specifically excluded (5). Tissue diagnosis by biopsy carries the risk of false-negative results due to the heterogeneous nature of ACC. In addition, there is a risk of tumor seeding along the biopsy tract. Therefore, in suspected ACC based on
imaging studies, surgical treatment is recommended for both diagnosis and therapy (7).
ACC can present with symptoms of excess autonomous adrenal hormones or those related to an intra-abdominal mass. Less frequently, it can manifest with non-specific symptoms, such as weight loss, fever, night sweats, back or flank pain, and a feeling of abdominal fullness. However, the number of incidentally detected cases has been increasing worldwide. Despite this, the likelihood of adrenal incidentalomas with ACC is low (5). In this case report, the patient’s existing adrenal mass, as mentioned, did not exhibit hormonal function. Although the adrenal mass had a long axis of 12 cm, it did not cause compression symptoms and became symptomatic after metastasis. In addition, the current literature states that the probability of incidentalomas being ACC is low, and the patient in this case report falls into this low- probability group.
The radiological characteristics of ACC typically include large adrenal masses that are heterogeneous with calcifications, hemorrhage/necrosis, and a poor washout pattern on delayed CT series (7). In 90% of ACC cases, there is a unilateral mass >6 cm with necrosis (8). In this case report, the dynamic adrenal CT described a ‘cystic lesion with fine septations, and solid components enhancing after intravenous contrast injection’. These findings do not align with the expected radiological features of ACC. Consequently, the mass was initially considered a non-functional benign adrenal lesion.
According to the recommendations of the European Society of Endocrinology, adrenal masses ≥4 cm have an increased risk of malignancy (9). Therefore, the masses should be evaluated by a multidisciplinary team. Surgery is recommended for most patients, and additional investigations are required for staging. Patient staging is recommended before surgery. In light of this information, an adrenalectomy was planned for the patient mentioned in the case report. However, because the patient presented with intestinal obstruction, a preoperative staging evaluation could not be performed.
Due to its rarity and short life expectancy, few prospective or randomized controlled trials have been conducted on ACC. Consequently, there is a lack of high-quality evidence for guidance in follow-up and treatment processes, leading to a lack of consensus. Surgery is considered the most important treatment regimen and is generally accepted as the only curative treatment. Adjuvant systemic therapy is usually administered based on the stage of the disease and the size of the tumor to improve disease-free and overall survival (10).
R0 resection is the only curative treatment for ACC. For patients suspected of having ACC, en bloc resection, including a suspicious mass and peritumoral/periadrenal retroperitoneal fat tissue, is recommended (5).
Incomplete resection results in better long-term outcomes and survival compared with cases in which no resection is performed (11). In addition, lymph node metastases in ACC are associated with a poor prognosis, although the role of lymphadenectomy remains unclear (12). Tseng et al. showed that lymphadenectomy not only allows for extensive staging but also improves overall survival (1). However, in some cases, due to the lack of a preoperative diagnosis of ACC, the decision for lymphadenectomy cannot be made before surgery.
ACC is a rare malignancy with a short survival period. Therefore, there is no consensus regarding a definitive treatment or follow-up approach. Despite its rarity, an atypical presentation, as mentioned in this case report, can occasionally occur. Hence, incidentally detected adrenal masses >4 cm should be considered alarming and warrant a more detailed evaluation for malignancy.
Declaration of interest
The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the work reported.
Funding
This work did not receive any specific grant from any funding agency in the public, commercial, or not-for-profit sector.
Patient consent
Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Author contribution statement
YK conceived the study; YK, PD, and EG performed data curation; PD and EG performed formal analysis; YK and PD investigated the results; YK and EG designed the methodology; YK supervised the study; YK, PD, and EG wrote the original draft; YK wrote, reviewed, and edited the manuscript.
References
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