Ectopic Adrenocortical Cancer Originating From the Pancreas: A Case Report With Literature Review
Alperen Onur İşler1,2, Nezih Akkapulu3, Altan Kavuncuoğlu4
1Division of Endocrinology and Metabolism, Hacettepe University School of Medicine, Ankara, Turkey.
International Journal of Surgical Pathology
|January 22, 2026
Summary
Ectopic adrenocortical cancer (ACC) is rare, with this case detailing the first instance of ACC originating in the pancreas. This finding highlights the importance of considering ACC in non-adrenal tumors.
Area of Science:
- Endocrinology
- Oncology
- Pathology
Background:
- Malignancies from adrenal rests are exceptionally rare and can occur in ectopic locations due to embryological development.
- Ectopic adrenocortical cancer (ACC) is a rare entity, with documented occurrences in various non-adrenal sites.
Purpose of the Study:
- To report the first diagnosed case of ectopic adrenocortical cancer (ACC) arising in the pancreas.
- To review the literature on ectopic ACC and emphasize its consideration in differential diagnoses of non-adrenal tumors.
Main Methods:
- A case study of a 57-year-old female patient diagnosed with ectopic pancreatic ACC.
- Diagnostic evaluation including imaging and pathological examination with immunohistochemical staining (SF-1).
- Surgical excision of the pancreatic mass followed by adjuvant therapy (mitotane and glucocorticoid replacement).
Main Results:
- A 6 cm, well-circumscribed, heterogeneous mass in the left paraaortic region, located in the pancreas, was identified.
- Pathological examination confirmed an oncocytic type of ACC with high nuclear grade and SF-1 positivity.
- The patient showed no signs of hormonal hyperfunction and no recurrence or metastasis at present.
Conclusions:
- This is the first reported case of ectopic adrenocortical cancer (ACC) originating in the pancreas.
- Ectopic ACC should be included in the differential diagnosis for tumors found in non-adrenal locations.
- Early diagnosis and appropriate management are crucial for patients with ectopic ACC.
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