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Case Reports
. 2019 Mar 5;3(4):784-790.
doi: 10.1210/js.2019-00050. eCollection 2019 Apr 1.

Adrenal Cortical Carcinoma Associated With Lynch Syndrome: A Case Report and Review of Literature

Ravinder Jeet Kaur  1 , Pavel N Pichurin  2 , Jolaine M Hines  3 , Ravinder J Singh  4 , Stefan K Grebe  4 , Irina Bancos  1
Affiliations
Case Reports

Adrenal Cortical Carcinoma Associated With Lynch Syndrome: A Case Report and Review of Literature

Ravinder Jeet Kaur et al. J Endocr Soc. .

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.

Keywords: Lynch syndrome; adrenocortical carcinoma; adrenocorticotropic hormone; diagnosis; steroid profiling.

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Figures

Figure 1.
Figure 1.
(a, left) Axial CT image and (right) coronal CT image showing a 6.0 × 5.1 × 7.8-cm right adrenal mass (arrows). (b) Gross pathology serial cut sections of a 9.2-cm right ACC.
Figure 2.
Figure 2.
Urine steroid profiling. HRAM, high resolution, accurate mass.
See this image and copyright information in PMC

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