Ectopic Adrenocortical Cancer Originating From the Pancreas: A Case Report With Literature Review

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 development. 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, cushingoid 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.