Ectopic Adrenocortical Cancer Originating From the Pancreas: A Case Report With Literature Review
International Journal of Surgical Pathology
1-8
@ The Author(s) 2026
CC 1. BY NC
Article reuse guidelines: sagepub.com/journals-permissions DOI: 10.1177/10668969251407230 journals.sagepub.com/home/ijs S Sage
Alperen Onur İşler, MD1,2 (D, Nezih Akkapulu, MD3 (D, Altan Kavuncuoğlu, MD4 (D, Büşra Fırlatan, MD2 İD, and Uğur Ünlütürk, MD1,2 İD
Abstract
Malignancies arising from adrenal rests are extremely rare. They may occur in locations such as the retroperitoneum, testes/scrotum, ovaries, kidneys, anterior abdominal wall, spinal cord, and liver, in association with embryological devel- opment. In this article, we present a patient with ectopic adrenocortical cancer (ACC) located in the pancreas, along with a review of the relevant literature. A 57-year-old female patient was diagnosed with mixed-type breast cancer (lobular and ductal carcinoma). During further diagnostic evaluations, in addition to a malignant-appearing mass in the left breast, imaging revealed a second tumor in the left paraaortic region, located in the pancreas. This mass, measuring 6 cm, was well-circumscribed, partially necrotic, heterogeneous in appearance, and displayed no significant invasion of surrounding tissues. The patient exhibited no signs of hormonal hyperfunction, such as hypertension, electrolyte imbalances, cushin- goid appearance, or hyperandrogenism. Following lumpectomy, the mass adjacent to the pancreas was surgically excised. Pathological examination revealed an oncocytic type of ACC, characterized by nuclear abnormalities, a high mitotic index, and high nuclear grade, with positivity for steroidogenic factor 1 (SF-1) (NR5A1) in immunohistochemical staining. Mitotane and glucocorticoid replacement therapy were added to the patient’s treatment plan for ACC. At present, there are no signs of recurrence or metastasis. To our knowledge, this is the first reported example of ectopic ACC arising in the pancreas, diagnosed in a 57-year-old patient. ACCs should be considered in the differential diagnosis of tumors arising in nonadrenal regions.
Keywords
adrenocortical cancer, ectopic adrenocortical cancer, pancreas, pancreas tumors, ectopic
Introduction
Adrenocortical cancer (ACC) is an uncommon malignant tumor with a low incidence rate of 0.7 to 2.0 patients per mil- lion people per year. It is more frequently diagnosed in women, comprising about 55% to 60% of all patients. Although it typically peaks in the first decade of life and in individuals aged 40 to 50, ACC can be diagnosed at any age.
The adrenal medulla originates in neural crest tissue, while the adrenal cortex originates from the coelomic mesoderm of the urogenital process.2 Ectopic adrenal rests can sometimes be found in various locations, including the celiac plexus, ova- ries, broad ligament, spermatic cord, testicles, bilateral lung, liver, spleen, pancreas, duodenum, and colon, due to the adre- nal cortex’s embryological development.3-7 While malignan- cies arising from adrenal rests are rare, when they do occur, the gender distribution is equal. These tumors have been reported in various locations such as the retroperitoneum, tes- tis/scrotum, ovary, kidney, anterior abdominal wall, spinal
cord, and liver.8-10 This article presents a unique tumor of ectopic ACC located in the pancreas, which, to our knowl- edge, has not been previously reported. Additionally, this patient is distinguished by the simultaneous presence of breast cancer, making it an intriguing clinical finding.
1Division of Endocrinology and Metabolism, Hacettepe University School of Medicine, Ankara, Turkey
2Department of Internal Medicine, Hacettepe University School of Medicine, Ankara, Turkey
3Department of General Surgery, Hacettepe University School of Medicine, Ankara, Turkey
4Department of Pathology, Hacettepe University School of Medicine, Ankara, Turkey
Corresponding Author:
Uğur Ünlütürk, Division of Endocrinology and Metabolism; Department of Internal Medicine, Hacettepe University School of Medicine, Hacettepe 06100, Ankara, Turkey.
Email: ugurunluturk@gmail.com
Patient
A 57-year-old female patient presented to the general surgery department with swelling, redness, and stiffness in her left breast. She did not have any prior medical conditions but had a family history of thyroid and lung cancer. No significant findings were observed during the physical examination except for the mass in her left breast. Mammography revealed multifocal masses in the lower half of the left breast, with a BI-RADS 6. The true-cut biopsy from the breast was compat- ible with a mixed type of infiltrative carcinoma comprising ductal and lobular components.
The thoracoabdominal computed tomography (CT) and FDG PET CT scans revealed a malignant mass in the left breast, as well as a second tumor in the left paraaortic area adjacent to the pancreas. The latter was 5.5 x 6 cm, well-circumscribed, locally necrotic, and had a heterogeneous appearance (Figure 1), with an SUVmax of 37.7 on the FDG PET CT scan. The adrenal glands appeared normal, except for a 1.5 to 2 cm nodule on the left side. A subsequent abdominal magnetic resonance imaging (MRI) revealed a mass with cystic and solid components originating from the pancreatic body and tail junc- tion and growing toward the posterior and inferior regions. Notably, the patient did not exhibit any signs of hormonal hyperfunction, such as hypertension, electrolyte disturbance, cushingoid appearance, or hyperandrogenism.
As an initial intervention, the patient underwent a lumpec- tomy and sentinel lymph node biopsy for a breast tumor. The final diagnosis revealed that the patient had a grade 2, ER-positive, PR- and HER2-negative mixed (ductal + lobular) infiltrative carcinoma with a tumor stage of T2N0M0. Subsequently, the patient underwent surgery to remove the mass adjacent to the pancreas and the left adrenal gland. A mar- ginal excision of the mass was performed adjacent to the distal pancreas, yet the excision did not include pancreatic tissue. Radiologically and intraoperatively tumor had no macroscopic relation to the left adrenal gland. Ectopic adrenal tissue was inci- dentally found in the pathological examination and diagnosed through a clinical, radiological, and a pathological correlation. A histopathological examination of the mass revealed a cellular oncocytic neoplasm growing in diffuse sheets and a well- demarcated, expansile manner adjacent to a rim of adrenal tissue (Figure 2A). The tumor cells were large polygonal cells with sig- nificant nuclear atypia and lacked cytoplasmic inclusions (Figure 2B and C). Immunohistochemical analysis showed that the neoplasm was positive for melan-A (MLANA) (Figure 2D), SF-1 (NR5A1) (Figure 2E), calretinin (CALB2) (Figure 2F), and vimentin (VIM), which indicated an adrenocor- tical origin. However, it was negative for inhibin-« (INHA), synaptophysin (SYP), keratin AE1/AE3, EMA, chromogranin A (CHGA), PAX8, S100, HMB45, desmin (DES), Hep Par-1 (CPS1), and GATA3. The mitotic index was 6/50 in high-power fields (HPFs) (Figure 2C), while the Ki-67 proliferation index was 5%. No additional worrisome features were observed in the patient, such as venous, capsular, sinusoidal invasion,
necrosis, or a tumor diameter >10 cm or weight>200 g. The sur- gical margins were found to be intact. A concomitant nodular neoplasm was also detected in the patient’s left adrenal gland, but it exhibited benign features consistent with an adrenocortical adenoma. We employed the Lin-Weiss-Bisceglia (LWB) sys- tem, as recommended by the current World Health Organization (WHO) classification, for the evaluation of onco- cytic ACCs. According to this system, the tumor was classified as malignant based on the presence of one major criterion: high mitotic activity (6 mitoses per 10 mm2 or 50 HPFs at 0.2 mm2 field size). Morphologically, the tumor exhibited features consis- tent with an oncocytic phenotype, including a diffuse growth pat- tern, nuclear atypia up to grade 3, and abundant eosinophilic granular cytoplasm in nearly all tumor cells. The Weiss score for the tumor was 4. The diagnosis of ectopic adrenocortical car- cinoma was established through the correlation of radiological evidence showing no anatomical connection to the adrenal glands, intraoperative findings demonstrating no macroscopic relation to the left adrenal gland-indicating that the tumor was neither an extension nor a metastasis of an adrenal pri- mary-and pathological confirmation of ectopic adrenal tissue displaying an adrenocortical immunophenotype.
The patient underwent radiotherapy as a treatment for breast cancer following 4 adjuvant cycles of docetaxel- cyclophosphamide. After that, she was treated with letro- zole treatment. Mitotane and glucocorticoid replacement were included in her follow-up therapy for adrenocortical cancer. The subsequent follow-ups did not show any signs of recurrence or metastasis.
Discussion
This is the first case report of adrenocortical cancer local- ized in the pancreas, diagnosed at age 57. Another remark- able feature of the patient is the presence of concurrent breast cancer. Tumors of adrenal rest are typically not can- cerous and are nonfunctional. They are often identified acci- dentally or after undergoing inguinal hernia surgery. They generally are detected incidentally or after inguinal hernia surgery.11,12 Occasionally, these tumors can be functional, producing glucocorticoids, mineralocorticoids, or andro- gens.13,14 It is important to note that a negative or low tumor marker result does not necessarily rule out malignancy, especially in cortisol-secreting tumors. This is because excess glucocorticoids can inhibit tumor markers.15
ACCs that are associated with high levels of steroid hor- mones tend to grow at a faster rate and are more extensive. Typically, these tumors are larger, ranging from 10 to 13 cm.16 Conversely, nonfunctional tumors are less common and typically smaller in size.17 In our patient, faint clinical signs, a tumor diameter of 5.5 x 6 cm, and a Ki-67 prolifera- tion index of 5% suggest a relatively less aggressive cancer.
Imaging, specifically MRI or CT, is necessary to evaluate ACCs. These tumors are generally heterogeneous with irregu- lar borders and can contain areas of hemorrhage or necrosis.18
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C
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20 um
E
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Pathological diagnosis of our patient was ultimately challeng- ing due to the tumor’s unusual clinical presentation, variant his- tology, and subtle atypical features. Ectopic ACCs have been previously reported in the retroperitoneum, but a central rela- tion to the pancreas is highly unexpected. Due to this abnormal localization, the oncocytic morphology, history of additional
malignancy, and limited specificity of ancillary markers, an extensive workup was necessary to rule out other potential diagnoses, such as tumors of gonadal stromal lineage or non- steroidogenic oncocytic malignancies. However, an accompa- nying adrenal rest was an important clue that aided in the diagnosis.
| Study | Age and Gender | Ectopic Location | Size (cm) | Hormonal Function | Weiss Score | Positive Pathological Staining | Ki-67 Index | Establishment of Malignant Diagnosis | Distant Metastasis at Diagnosis |
|---|---|---|---|---|---|---|---|---|---|
| Tsai et al9 | 59, F | Ovary | 23× 21 | Not detected | 8 | Synaptophysin (SYP), inhibin-a (INHA), TFE3, calretinin (CALB2), CD56 | >80% | Necrosis, diffuse architecture, high mitotic activity, and lymphovascular invasion | Lymph node, peritoneal, and pulmonary |
| Chentli et al 12 | 34, F | Ovary | 14.5× 13.7 | Yes | >5 | Inhibin-a (INHA), melan-A (MLANA), and SF-1 | Unknown | Vascular embolism, necrosis, high nuclear grade, and atypical mitosis | Lymph node and peritoneal |
| Salle et al 22 | 4, F | Ovary | 20 | Yes | Unknown | Unknown | Unknown | Revision of the diagnosis of "steroid cell tumor of the ovary" upon lung metastasis | Unknown |
| Wright et al23 | 68, M | Retroperitoneal | 4.1×4 | No | Unknown | Synaptophysin (SYP), keratin AE1/AE3, and INI1 (SMARCB1) | 30% | Necrosis, high nuclear grade, extension through the diaphragm in an otherwise well-defined mass | No |
| Yokoyama | 34, F | Retroperitoneal | 6.5×5.6× | No | 4 | SF-1 (NR5A1), | <1% | Nuclear atypia, atypical | No |
| et al24 | 4.5 | 17a-hydroxylase, dehydroepiandrosterone sulfotransferase, and 3ß-hydroxysteroid dehydrogenase | mitosis, sinusoidal invasion | ||||||
| Zhou et al 10 | 77, M | Anterior abdominal wall | 30× 15 | No | 6 | Synaptophysin (SYP), melan A (MLANA), vimentin (VIM), keratin 20 (KRT20), IGF2, and EMA | >%80 | Necrosis, high mitotic count, and high nuclear grade | No |
| Park et al6 | 75, M | Liver | 6×4.5 | Unknown | 7 | Inhibin-a (INHA) and melan-A (MLANA) | Unknown | Nuclear atypia, necrosis, venous invasion, and capsular invasion | No |
| Permana et al25 | 21, F | Liver | 11.6× 14.2 × 15.6 | Yes | Unknown | CD56, Hep Par-1 (CPS1), and neuron specific enolaz (ENO2) | Unknown | NA | No |
| Study | Age and Gender | Ectopic Location | Size (cm) | Hormonal Function | Weiss Score | Positive Pathological Staining | Ki-67 Index | Establishment of Malignant Diagnosis | Distant Metastasis at Diagnosis |
|---|---|---|---|---|---|---|---|---|---|
| Nie et al26 Rodriguez et al27 Cornejo et al® | 71, M 5*, F 51, M | Lung Spinal region Between the bladder and prostate | 6.7×6.5×4 6× 1.5 10 | No Unknown No | 5 Unknown Unknown | Inhibin-a (INHA), melan-A (MLANA), synaptophysin (SYP), keratin AE1/AE3, cromagranin A (CHGA), and CD10 (MME) Synaptophysin (SYP), inhibin-a (INHA), melan-A (MLANA), keratin AE1/AE3, and CAM 5.2 Synaptophysin (SYP), inhibin-a (INHA), melan-A (MLANA), and MITF | Unknown Unknown Unknown | Upon prompt High nuclear grade, necrosis, and capsular invasion recurrence after the initial diagnosis of "adrenocortical tumor of indeterminate malignant potential": Mitosis 5/10 HPF, and no frankly malignant feature High nuclear grade, high mitotic activity, necrosis, and lymphovascular invasion | No No No |
| Jain et al28 Present patient | 65, M 57, F | Left testis Pancreas | 8.5×6.5 5.5×6 | Yes No | Unknown N/A (oncocytic) | Inhibin-a (INHA) Vimentin (VIM), melan-A (MLANA), calretinin (CALB2), and SF-1 | Unknown 5% | Necrosis, extension through the tunica albuginea, and metastases concomitant High mitotic activity | Lymph node and retroperitoneum No |
Abbreviations: F, female; M, male, *, months; TFE3, transcription factor enhancer-3; SF-1 (NR5A1), steroidogenic factor-1; IGF2: insulin-like growth factor-2; N/A: not applicable.
Determining the biological potential of oncocytic ACCs based on morphological criteria has proven challenging. However, the major criteria established by LWB indicate that a high mitotic index supports a carcinoma diagnosis.19 In our patient, although only one major LWB criterion was met, the Weiss score for the tumor with its features listed above was 4, a threshold at which ectopic ACC has previ- ously been reported. Additionally, the Helsinki score, pro- posed for oncocytic ACCs, was calculated as 8 (based on mitotic count: 3 and Ki-67 index: 5%). This value falls below the established malignancy threshold of 8.5. However, the applicability of this score in our patient is lim- ited, as the Ki-67 index was not assessed using the recom- mended computer-assisted image analysis, which may affect reproducibility.20 The sampling protocol followed for this specimen involved the standard approach of one tis- sue block per centimeter of tumor. Given that this diagnosis predated the widespread adoption of the Helsinki system, more extensive capsular sampling-as is sometimes employed in other endocrine tumors-might have provided additional diagnostic clarity, particularly in resolving the apparent discordance between the LWB scoring and Helsinki system. Reticulin staining, while considered by some as a supportive diagnostic tool, was not performed due to limited interobserver reliability in our experience and the absence of published data on its utility in ectopic ACCs. It is important to highlight that no standardized diag- nostic criteria currently exist for ectopic ACCs. Several patients in the literature have also been diagnosed retrospec- tively, based on atypical clinical progression. Despite their location, ectopic ACCs have conventionally been assessed using the Weiss criteria, even when demonstrating oncocy- tic features. In some instances, infiltrative growth across natural anatomical boundaries has also been emphasized as an indicator of malignancy (see Table 1). Collectively, these observations point to the need for pathological stan- dardization in the assessment of these rare presentations, encompassing aspects ranging from macroscopic sampling protocols to ancillary diagnostic techniques.
Recent updates to adrenocortical tumor classifications, published after the patient’s diagnosis, have shown that ACCs with a lower Ki-67 index ≤20%, as observed in our patient with a Ki-67 index of 5%, are considered low-grade malignancies with a better prognosis.21 Unfortunately, due to the rarity of these occurrences, there is limited data and experience with unusual presentations. Table 1 provides a review of reported patients of adrenocortical carcinoma aris- ing from ectopic adrenal remnants, based on the published literature.
The primary treatment of adrenocortical cancers is sur- gery,29 while mitotane can be used as medical therapy alone or in combination with chemotherapeutic drugs.3º Although our patient involved a nonmetastatic, hormonally inactive, histologically low-grade ACC, adjuvant mitotane therapy was administered following multidisciplinary evaluation.
This decision was made considering both the rarity of ectopic ACC and the limitations of existing prognostic tools.
Conclusion
This report describes an ectopic presentation of adrenocor- tical cancer in a 57-year-old female patient. The cancer was localized in the pancreas and was treated with surgical resection. Adrenocortical cancers should also be considered in nonadrenal tumors.
ORCID iDs
Alperen Onur İşler İD https://orcid.org/0009-0004-4738-9807 Nezih Akkapulu İD https://orcid.org/0000-0001-7392-961X Altan Kavuncuoğlu İD https://orcid.org/0000-0003-0708-2291 Büşra Fırlatan İD https://orcid.org/0000-0002-9072-5683 Uğur Ünlütürk İD https://orcid.org/0000-0002-5054-1396
Ethical Approval
Hacettepe University does not require IRB approval for a case report.
Author Contributions
AOİ, AK, and UÜ: study design, data collection, drafting, and crit- ical review of the manuscript. NA and BF: data collection and crit- ical review of the manuscript. All authors approved the final version of the paper.
Funding
The authors received no financial support for the research, author- ship, and/or publication of this article.
Declaration of Conflicting Interests
The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Data Availability Statement
The dataset regarding the patient’s evaluation may be obtained from the corresponding author upon reasonable request.
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