Adrenal cortical carcinoma associated with lynch syndrome: A case report and review of literature

Ravinder Jeet Kaur, Pavel N. Pichurin, Jolaine M. Hines, Ravinder J. Singh, Stefan K. Grebe, Irina Bancos

Research output: Contribution to journalReview articlepeer-review

6 Scopus citations

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 languageEnglish (US)
Pages (from-to)784-790
Number of pages7
JournalJournal of the Endocrine Society
Volume3
Issue number4
DOIs
StatePublished - Jan 1 2019

Keywords

  • adrenocortical carcinoma
  • adrenocorticotropic hormone
  • diagnosis
  • Lynch syndrome
  • steroid profiling

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism

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