Endocrine Abstracts

Background: Ectopic malignancies arising from adrenal rests are extremely rare. Localization has been reported in the retroperitoneum, testis/scrotum, ovary, kidney, anterior abdominal wall, spinal cord, and liver associated with embryological development ^1-3.

Objective: This report presents a case of ectopic adrenocortical cancer (ACC) localized to the pancreas. An exciting feature of the case was the simultaneous presence of breast cancer.

Case: A 57 years old female patient was diagnosed with mixed-type breast cancer (lobular and ductal carcinoma) in 2019. After the diagnosis, in addition to the malignant mass in the left breast on CT and FDG PET CT images, a second tumor with well-circumscribed, necrotic, heterogeneous malignant appearance was seen adjacent to the pancreas, in the left paraaortic area, without significant invasion to the environment (SUVmax: 37.7 on FDG PET CT). On abdominal MRI, A 5.2 cm diameter mass with cystic, solid components originating from the pancreatic body and tail junction, growing to posterior and inferior, was observed. The patient did not have features suggestive of hormonal hyperfunction, such as hypertension, electrolyte imbalance, cushingoid appearance, and signs of hyperandrogenism. After the lumpectomy, the mass adjacent to the pancreas was surgically removed. Positive staining with Steroidogenic factor 1 (SF1) was observed in pathology. Ki67 proliferation index was determined as 5%. Neither venous invasion nor capsular invasion was observed. This neoplasm had a diffuse pattern, increased mitosis, and marked nuclear pleomorphism. Regarding p53 mutation in tumor, 5% weak-moderate staining was observed. The tumor was diagnosed with oncocytic-type adrenocortical carcinoma. Mitotane and glucocorticoid replacement were added to her follow-up treatment. There was no evidence of recurrence or metastasis in the follow-ups.

Conclusion: This rare case is the first whose ectopic adrenocortical cancer is located in the pancreas. Adrenocortical cancers should also be considered in tumors of non-adrenal areas.

References: 1. Cornejo, K.M., H.A. Afari, and P.M. Sadow, Adrenocortical carcinoma arising in an adrenal rest: a case report and review of the literature. Endocrine Pathology, 2017. 28(2): p. 165-170.

2. Tsai, W.-H., et al., Case Report: Ectopic Adrenocortical Carcinoma in the Ovary. Frontiers in endocrinology, 2021. 12: p. 662377.

3. Zhou, D.-K., et al., Giant nonfunctional ectopic adrenocortical carcinoma on the anterior abdominal wall: A case report. World Journal of Clinical Cases, 2019. 7(15): p. 2075.