Abstract
Objective: Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis. ACC was reported in 3.2% patients with Lynch syndrome (LS), however no particular case-detection strategies have been recommended. Participants: We report a case of a 65-year-old woman who was incidentally discovered with a large adrenal mass during work-up of postmenopausal uterine bleeding. She was recently diagnosed with MSH6 germline mutation after her sister presented with uterine carcinoma in the setting of LS. Results: Whereas the patient was asymptomatic for overt hormonal excess, biochemical work-up confirmed glucocorticoid autonomy and androgen and estrogen excess. Urine steroid profiling was suggestive of ACC. Adrenalectomy confirmed an oncocytic ACC with focal extracapsular extension into the periadrenal adipose tissue with a Ki-67 of 15% and a peak mitotic count of 40/50 high-power fields. Conclusion: ACC can be the only manifestation of LS. A best case-detection approach for ACC in the asymptomatic patient with LS is unclear, however urine steroid profiling could be considered.
Original language | English (US) |
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Pages (from-to) | 784-790 |
Number of pages | 7 |
Journal | Journal of the Endocrine Society |
Volume | 3 |
Issue number | 4 |
DOIs | |
State | Published - Jan 1 2019 |
Keywords
- adrenocortical carcinoma
- adrenocorticotropic hormone
- diagnosis
- Lynch syndrome
- steroid profiling
ASJC Scopus subject areas
- Endocrinology, Diabetes and Metabolism