Autopsy and Case Reports
https://app.periodikos.com.br/journal/autopsy/article/doi/10.4322/acr.2021.259
Autopsy and Case Reports
Clinical Case Report and Review

Giant virilising adrenal cortical carcinoma

Shruti Dogra; Arvind Ahuja; Minakshi Bhardwaj; Rohan Sardana; Hemant Goel

Downloads: 8
Views: 1105

Abstract

Androgen secreting adrenocortical carcinoma (ACC) is a very rare disease with a poor prognosis. Approximately 80% of tumors are functional, most commonly secreting glucocorticoids. We herewith report a case of a huge functional ACC of the right adrenal gland in a 33-year-old female who presented with complaints of hirsutism, amenorrhea and an abdominal lump. On abdominal examination a large lump was palpable in the right hypochondrium reaching up to the umbilicus. Contrast-enhance computed tomography (CECT) revealed a mass in the right suprarenal region. The tumor measured 29 cm × 20 cm × 12 cm and weighed 7.8 kg, the largest reported case of ACC in the world to the best of our knowledge.

Keywords

Adrenocortical carcinoma, Adrenal Gland, Functional, Giant, Largest

References

1 Lloyd R, Osamura R, Kloppel G, Rosai J. WHO Classification of tumours of endocrine organs. 4th ed. Lyon: International Agency for Research on Cancer; 2017. p. 163-4.

2 Else T, Kim AC, Sabolch A, et al. Adrenocortical carcinoma. Endocr Rev. 2014;35(2):282-326. http://dx.doi.org/10.1210/er.2013-1029. PMid:24423978.

3 Sternberg SS, Mills SE, Carter D. Sternberg’s diagnostic surgical pathology. 6th ed. Philadelphia: Wolters Kluwer; 2015.

4 Almarzouq A, Asfar S, Hussain S, Al-Hunayan A, Aldousari S. Giant nonfunctioning adrenocortical carcinoma: a case report and review of the literature. BMC Res Notes. 2014;7(1):769. http://dx.doi.org/10.1186/1756-0500-7-769. PMid:25361816.

5 Didolkar MS, Bescher RA, Elias EG, Moore RH. Natural history of adrenal cortical carcinoma: a clinicopathologic study of 42 patients. Cancer. 1981;47(9):2153-61. http://dx.doi.org/10.1002/1097-0142(19810501)47:9<2153::AID-CNCR2820470908>3.0.CO;2-6. PMid:7226109.

6 Stojadinovic A, Ghossein RA, Hoos A, et al. Adrenocortical carcinoma: clinical, morphologic, and molecular characterization. J Clin Oncol. 2002;20(4):941-50. http://dx.doi.org/10.1200/JCO.2002.20.4.941. PMid:11844815.

7 Kunieda K, Saji S, Mori S, et al. Recurrence of giant adrenocortical carcinoma in the contralateral adrenal gland 6 years after surgery: report of a case. Surg Today. 2000;30(3):294-7. http://dx.doi.org/10.1007/s005950050063. PMid:10752787.

8 Straka M, Soumarova R, Bulejcik J, Banik M, Pura M, Skrovina M. Giant adrenocortical carcinoma with 27-month disease-free survival by surgical resection alone: a case report. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2014;158(3):474-8. http://dx.doi.org/10.5507/bp.2013.040. PMid:23783027.

9 Uruc F, Urkmez A, Yuksel O, Sahin A, Verit A. Androgen secreting giant adrenocortical carcinoma with no metastases: a case report and review of the literature. Can Urol Assoc J. 2015;9(9-10):E644. http://dx.doi.org/10.5489/cuaj.2867. PMid:26425231.

10 Patel V, Shah D, Raychaudhari C, Patel K. Giant non-functioning adrenocortical carcinoma: a rare childhood tumor. Indian J Med Paediatr Oncol. 2010;31(2):65-8. http://dx.doi.org/10.4103/0971-5851.71659. PMid:21209768.

11 Aubert S, Wacrenier A, Leroy X, et al. Weiss system revisited: a clinicopathologic and immunohistochemical study of 49 adrenocortical tumors. Am J Surg Pathol. 2002;26(12):1612-9. http://dx.doi.org/10.1097/00000478-200212000-00009. PMid:12459628.

12 Ayala-Ramirez M, Jasim S, Feng L, et al. Adrenocortical carcinoma: clinical outcomes and prognosis of 330 patients at a tertiary care center. Eur J Endocrinol. 2013;169(6):891-9. http://dx.doi.org/10.1530/EJE-13-0519. PMid:24086089.

13 Fassnacht M, Dekkers OM, Else T, et al. European Society of Endocrinology Clinical Practice guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2018;179(4):G1-46. http://dx.doi.org/10.1530/EJE-18-0608. PMid:30299884.
 


Submitted date:
01/01/2021

Accepted date:
02/01/2021

Publication date:
05/06/2021

609442a7a9539517bc7fd9d2 autopsy Articles
Links & Downloads

Autops Case Rep

Share this page
Page Sections