Familial Cancer

, Volume 17, Issue 3, pp 381–385 | Cite as

Ectopic, retroperitoneal adrenocortical carcinoma in the setting of Lynch syndrome

  • Jesse P. Wright
  • Kathleen W. Montgomery
  • Joshua Tierney
  • Jill Gilbert
  • Carmen C. Solórzano
  • Kamran Idrees
Short Communication


Adrenocortical carcinoma (ACC) is rare within the adult population. Ectopic ACC proves even rarer. This variant is formed by cortical fragments arrested during embryologic migration. ACC is also known to be associated with several genetic syndromes and has recently been linked to Lynch syndrome in 3% of cases. We present the case of a 68-year-old male with a confirmed diagnosis of Lynch syndrome secondary to a germline MSH2 mismatch-repair gene-mutation who presented with 2 months history of non-specific abdominal pain. After imaging work-up, the patient was found to have a right upper quadrant, retroperitoneal mass. Biochemical tests were without any evidence of a hormonally active process. Fine needle aspiration of the mass revealed a poorly differentiated carcinoma of unknown etiology. The lesion was resected and found to be consistent with ectopic ACC with an associated MSH2 mutation.


Adrenocortical carcinoma Lynch syndrome Ectopic ACC Adrenal rests 


Compliance with ethical standards

Conflict of interest

The authors report no conflicts of interest.


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Copyright information

© Springer Science+Business Media B.V. 2017

Authors and Affiliations

  • Jesse P. Wright
    • 1
  • Kathleen W. Montgomery
    • 2
  • Joshua Tierney
    • 3
  • Jill Gilbert
    • 4
  • Carmen C. Solórzano
    • 1
  • Kamran Idrees
    • 1
  1. 1.Division of Surgical Oncology, Department of SurgeryVanderbilt University Medical CenterNashvilleUSA
  2. 2.Department of PathologyVanderbilt University Medical CenterNashvilleUSA
  3. 3.Department of SurgeryUniversity of LouisvilleLouisvilleUSA
  4. 4.Division of Medical Oncology, Department of MedicineVanderbilt University Medical CenterNashvilleUSA

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