Endocrine Pathology

, Volume 28, Issue 2, pp 165–170 | Cite as

Adrenocortical Carcinoma Arising in an Adrenal Rest: a Case Report and Review of the Literature

  • Kristine M. Cornejo
  • Henrietta A. Afari
  • Peter M. Sadow


Carcinomas arising from embryonic adrenal rests are rare with only a handful of reported cases. We report a case of an adrenocortical carcinoma arising from an adrenal rest located between the bladder and prostate in a 51 year-old man. The patient presented following a year of rectal pain and constipation. Computed tomography (CT) scan revealed a 9 cm pelvic mass that appeared to arise from the soft tissue between the bladder and prostate, with displacement of the organs and narrowing of the rectal lumen, suspected to be a sarcoma. The surgically resected specimen showed a well-circumscribed, partially encapsulated tumor measuring 10.0 cm in greatest dimension. Both adrenal glands were identified intraoperatively. Grossly, the lesion was heterogeneous tan-brown to yellow, hemorrhagic and necrotic. Histology revealed sheets and nests of high-grade pleomorphic tumor cells with abundant clear to vacuolated cytoplasm with areas of necrosis, a high mitotic index (>10 mitoses/10 HPF) and foci suspicious for lymphovascular invasion. Adjacent adrenal cortical-type tissue was identified. Immunohistochemical stains revealed the tumor cells were weakly and focally positive for MiTF, Melan-A, inhibin and synaptophysin, and negative for CKAE1/AE3, HMB-45, calretinin, EMA, SMA, chromogranin, PAX8, MDM2 and CDK4. Based upon the morphologic and immunohistochemical profile, this was diagnosed as an adrenocortical carcinoma, arising in an adrenal rest. To our knowledge, no such tumor has been previously described in this location.


Adrenal rest Adrenocortical carcinoma 


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Conflict of Interest



  1. 1.
    Morgagni, G.B.: Cited by Wiesel, quod vidae.Google Scholar
  2. 2.
    Abell I. Primary Hypernephroma of the Liver. Ann Surg. 1928;87:829–836.CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Schechter DC. Aberrant adrenal tissue. Ann Surg. 1968;167:421–426.CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Pepere, A.: Tumeur primitive du foie originaire des germes aberrants de la capsule serrenale. Arch. De med. Exper. Et d’anat. Path., Par., 1902, vol. xiv, pp.765–808.Google Scholar
  5. 5.
    Keene MF. Observations on the Development of the Human Suprarenal Gland. J Anat. 1927;61:302–324.PubMedPubMedCentralGoogle Scholar
  6. 6.
    Anderson JR, Ross AH. Ectopic adrenal tissue in adults. Postgrad Med J. 1980;56:806–808.CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Barwick TD, Malhotra A, Webb JA, et al. Embryology of the adrenal glands and its relevance to diagnostic imaging. Clin Radiol. 2005;60:953–959.CrossRefPubMedGoogle Scholar
  8. 8.
    Wiener MF, Dallgaard SA. Intracranial adrenal gland; a case report. AMA archives of pathology. 1959;67:228–233.PubMedGoogle Scholar
  9. 9.
    Wallace EZ, Leonidas JR, Stanek AE, et al. Endocrine studies in a patient with functioning adrenal rest tumor of the liver. The American journal of medicine. 1981;70:1122–1125.CrossRefPubMedGoogle Scholar
  10. 10.
    Cassarino DS, Santi M, Arruda A, et al. Spinal adrenal cortical adenoma with oncocytic features: report of the first intramedullary case and review of the literature. International journal of surgical pathology. 2004;12:259–264.CrossRefPubMedGoogle Scholar
  11. 11.
    Goren E, Engelberg IS, Eidelman A. Adrenal rest carcinoma in hilum of kidney. Urology. 1991;38:187–190.CrossRefPubMedGoogle Scholar
  12. 12.
    Yokoyama H, Adachi T, Tsubouchi K, et al. Non-functioning adrenocortical carcinoma arising in an adrenal rest: immunohistochemical study of an adult patient. Tohoku J Exp Med. 2013;229:267–270.CrossRefPubMedGoogle Scholar
  13. 13.
    Akishima-Fukasawa Y, Yoshihara A, Ishikawa Y, et al. Malignant adrenal rest tumor of the retroperitoneum producing adrenocortical steroids. Endocr Pathol. 2011;22:112–117.CrossRefPubMedGoogle Scholar
  14. 14.
    Rodriguez FJ, Scheithauer BW, Erickson LA, et al. Ectopic low-grade adrenocortical carcinoma in the spinal region: immunohistochemical and molecular cytogenetic study of a pediatric case. The American journal of surgical pathology. 2009;33:142–148.CrossRefPubMedPubMedCentralGoogle Scholar
  15. 15.
    Engel FL, McPherson HT, Fetter BF, et al. Clinical, Morphological and Biochemical Studies on a Malignant Testicular Tumor. The Journal of clinical endocrinology and metabolism. 1964;24:528–542.CrossRefPubMedGoogle Scholar
  16. 16.
    Morimoto Y, Hiwada K, Nanahoshi M, et al. Cushing's syndrome caused by malignant tumor in the scrotum: clinical, pathologic and biochemical studies. The Journal of clinical endocrinology and metabolism. 1971;32:201–210.CrossRefPubMedGoogle Scholar
  17. 17.
    Contreras P, Altieri E, Liberman C, et al. Adrenal rest tumor of the liver causing Cushing's syndrome: treatment with ketoconazole preceding an apparent surgical cure. The Journal of clinical endocrinology and metabolism. 1985;60:21–28.CrossRefPubMedGoogle Scholar
  18. 18.
    Ney RL, Hammond W, Wright L, et al. Studies in a patient with an ectopic adrenocortical tumor. The Journal of clinical endocrinology and metabolism. 1966;26:299–304.CrossRefPubMedGoogle Scholar
  19. 19.
    Raith L, Karl HJ. Pregnancy in ectopic adrenal carcinoma. Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et metabolisme. 1969;1:149–150.CrossRefPubMedGoogle Scholar
  20. 20.
    Bani-Hani KE. Primary non-functional extra-adrenal adrenocortical carcinoma. Saudi Med J. 2003;24:301–304.PubMedGoogle Scholar
  21. 21.
    Transactions of the Philadelphia Academy of Surgery-Stated Meeting March 7, 1932. Annals of surgery. 1933;97:146–155.Google Scholar
  22. 22.
    Jain SH, Sadow PM, Nose V, et al. A patient with ectopic cortisol production derived from malignant testicular masses. Nat Clin Pract Endocrinol Metab. 2008;4:695–700.CrossRefPubMedGoogle Scholar
  23. 23.
    Tran TB, Postlewait LM, Maithel SK, et al. Actual 10-year survivors following resection of adrenocortical carcinoma. Journal of surgical oncology. 2016.Google Scholar
  24. 24.
    Tritos NA, Cushing GW, Heatley G, et al. Clinical features and prognostic factors associated with adrenocortical carcinoma: Lahey Clinic Medical Center experience. The American surgeon. 2000;66:73–79.PubMedGoogle Scholar
  25. 25.
    Icard P, Goudet P, Charpenay C, et al. Adrenocortical carcinomas: surgical trends and results of a 253-patient series from the French Association of Endocrine Surgeons study group. World journal of surgery. 2001;25:891–897.CrossRefPubMedGoogle Scholar
  26. 26.
    Terzolo M, Angeli A, Fassnacht M, et al. Adjuvant mitotane treatment for adrenocortical carcinoma. The New England journal of medicine. 2007;356:2372–2380.CrossRefPubMedGoogle Scholar
  27. 27.
    Habra MA, Ejaz S, Feng L, et al. A retrospective cohort analysis of the efficacy of adjuvant radiotherapy after primary surgical resection in patients with adrenocortical carcinoma. The Journal of clinical endocrinology and metabolism. 2013;98:192–197.CrossRefPubMedGoogle Scholar
  28. 28.
    Fassnacht M, Hahner S, Polat B, et al. Efficacy of adjuvant radiotherapy of the tumor bed on local recurrence of adrenocortical carcinoma. The Journal of clinical endocrinology and metabolism. 2006;91:4501–4504.CrossRefPubMedGoogle Scholar
  29. 29.
    Sabolch A, Feng M, Griffith K, et al. Adjuvant and definitive radiotherapy for adrenocortical carcinoma. International journal of radiation oncology, biology, physics. 2011;80:1477–1484.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2017

Authors and Affiliations

  • Kristine M. Cornejo
    • 1
    • 2
    • 3
  • Henrietta A. Afari
    • 4
  • Peter M. Sadow
    • 1
    • 2
  1. 1.James Homer Wright Pathology LaboratoriesMassachusetts General HospitalBostonUSA
  2. 2.Department of Pathology, Harvard Medical SchoolBostonUSA
  3. 3.Department of PathologyUMass Memorial HealthcareWorcesterUSA
  4. 4.Harvard Medical SchoolBostonUSA

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