SAT-479 - A Rare Case of Bilateral ACC in A Patient with NF1

Introduction:
Adrenocortical Carcinoma (ACC) is a rare and highly aggressive malignancy arising from the adrenal cortex. ACC is associated with many familial cancer susceptibility syndromes, such as Li-Fraumeni and adenomatous polyposis coli syndromes. We present a patient with NF1 who comes in with bilateral ACC.

Case Description:
A 50-year-old female with chronic medical conditions including invasive estrogen-positive breast cancer, type 1 neurofibromatosis (NF1) was attending her oncology/radiation oncology appointment for her breast cancer. At the time, she had clinical signs of hirsutism. An MRI breast was done which revealed an 18 cm mass in the right hepatic lobe. MRI liver revealed the hepatic mass, a 20 cm right adrenal mass with mass effect on liver and kidneys as well as a 5.5 cm left adrenal mass. A PET CT Dotatate revealed increased activity in bilateral adrenal masses. Laboratory testing was also done including DHEA-S which was elevated. A Dexamethasone suppression test was also negative. Metanephrine testing was done which was negative. Patient underwent bilateral adrenalectomy and was started on hydrocortisone and fludrocortisone. Pathology revealed bilateral ACC. Initially, management was with surveillance however subsequent abdominal MRI revealed worsening hepatic metastasis with increased uptake noted on repeat PET CT Dotatate scan. Needle biopsy was done of the liver with pathology revealing poorly differentiated carcinoma. Patient was started on chemotherapy with Mitotane.

Discussion:
This case highlights the rarity of having bilateral ACC especially in patients with NF1. Most adrenal incidentalomas are unilateral with the remaining being bilateral. Amongst the bileral incidentalomas, only a small percentage represent bilateral ACC. When bilateral adrenal lesions are seen, the most common etiologies include metastases and cortical adenomas. One study done in Olmsted County, Minnesota looked at the epidemiology of adrenal tumors and the frequency of different adrenal tumors. The study revealed that only 0.3% of adrenal tumors were adrenocortical carcinomas. This rarity also highlights the importance of elucidating the etiology of ACC development. In this case, the NF1 gene mutations led to the development of ACC. Ultimately, while bilateral ACC is rare, it is not absent, and it is crucial to figure out the etiology that can help determine management.

Reference:
Ebbehoj, A., Li, D., Kaur, R. J., Zhang, C., Singh, S., Li, T., Atkinson, E., Achenbach, S., Khosla, S., Arlt, W., Young, W. F., Rocca, W. A., & Bancos, I. (2020). Epidemiology of adrenal tumours in Olmsted County, Minnesota, USA: a population-based cohort study. The Lancet. Diabetes & Endocrinology, 8(11), 894–902. https://doi.org/10.1016/S2213-8587(20)30314-4

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