Journal of Yeungnam Medical Science

영남대 의대 학술지 편집위원회 (Yeungnam University College of Medicine, Yeungnam University Institute Medical Science)
권호 표지
DOI QR코드

DOI QR Code

Ruptured triple hormone-secreting adrenal cortical carcinoma with hyperaldosteronism, hypercortisolism, and elevated normetanephrine: a case report

  • Sin Yung Woo (Division of Endocrinology, Department of Internal Medicine, Konyang University College of Medicine) ;
  • Seongji Park (Division of Endocrinology, Department of Internal Medicine, Konyang University College of Medicine) ;
  • Kun Young Kwon (Division of Endocrinology, Department of Internal Medicine, Konyang University College of Medicine) ;
  • Dong-Mee Lim (Division of Endocrinology, Department of Internal Medicine, Konyang University College of Medicine) ;
  • Keun-Young Park (Division of Endocrinology, Department of Internal Medicine, Konyang University College of Medicine) ;
  • Jong-Dai Kim (Division of Endocrinology, Department of Internal Medicine, Konyang University College of Medicine)
  • 투고 : 2024.06.20
  • 심사 : 2024.08.02
  • 발행 : 2024.10.31
PDF 다운로드
⟨ 이전 논문 다음 논문 ⟩

초록

We report a case of a ruptured triple hormone-secreting adrenal mass with hyperaldosteronism, hypercortisolism, and elevated normetanephrine levels, diagnosed as adrenal cortical carcinoma (ACC) by histology. A 53-year-old male patient who initially presented with abdominal pain was referred to our hospital for angiocoagulation of an adrenal mass rupture. Abdominal computed tomography revealed a heterogeneous 19×11×15 cm right adrenal mass with invasion into the right lobe of the liver, inferior vena cava, retrocaval lymph nodes, and aortocaval lymph nodes. Angiocoagulation was performed. Laboratory evaluation revealed excess cortisol via a positive 1-mg overnight dexamethasone suppression test, primary hyperaldosteronism via a positive saline infusion test, and plasma normetanephrine levels three times higher than normal. An adrenal mass biopsy was performed for pathological confirmation to commence palliative chemotherapy because surgical management was not deemed appropriate considering the extent of the tumor. Pathological examination revealed stage T4N1M1 ACC. The patient started the first cycle of adjuvant mitotane therapy along with adjuvant treatment with doxorubicin, cisplatin, and etoposide, and was discharged. Clinical cases of dual cortisol- and aldosterone-secreting ACCs or ACCs presenting as pheochromocytomas have occasionally been reported; however, both are rare. Moreover, to the best of our knowledge, a triple hormone-secreting ACC has not yet been reported. Here, we report a rare case and its management. This case report underscores the necessity of performing comprehensive clinical and biochemical hormone evaluations in patients with adrenal masses because ACC can present with multiple hormone elevations.

키워드

참고문헌

  1. Fassnacht M, Kroiss M, Allolio B. Update in adrenocortical carcinoma. J Clin Endocrinol Metab 2013;98:4551-64.  https://doi.org/10.1210/jc.2013-3020
  2. Sharma E, Dahal S, Sharma P, Bhandari A, Gupta V, Amgai B, et al. The characteristics and trends in adrenocortical carcinoma: a United States population based study. J Clin Med Res 2018;10:636-40.  https://doi.org/10.14740/jocmr3503w
  3. Lerario AM, Mohan DR, Hammer GD. Update on biology and genomics of adrenocortical carcinomas: rationale for emerging therapies. Endocr Rev 2022;43:1051-73.  https://doi.org/10.1210/endrev/bnac012
  4. Bancos I, Prete A. Approach to the patient with adrenal incidentaloma. J Clin Endocrinol Metab 2021;106:3331-53.  https://doi.org/10.1210/clinem/dgab512
  5. Mansmann G, Lau J, Balk E, Rothberg M, Miyachi Y, Bornstein SR. The clinically inapparent adrenal mass: update in diagnosis and management. Endocr Rev 2004;25:309-40.  https://doi.org/10.1210/er.2002-0031
  6. Else T, Kim AC, Sabolch A, Raymond VM, Kandathil A, Caoili EM, et al. Adrenocortical carcinoma. Endocr Rev 2014;35:282-326.  https://doi.org/10.1210/er.2013-1029
  7. Zern NK, Eaton KD, Roth MY. Aldosterone-secreting adrenocortical carcinoma presenting with cardiac arrest. J Endocr Soc 2019;3:1678-81.  https://doi.org/10.1210/js.2019-00092
  8. Griffin AC, Kelz R, LiVolsi VA. Aldosterone-secreting adrenal cortical carcinoma. A case report and review of the literature. Endocr Pathol 2014;25:344-9.  https://doi.org/10.1007/s12022-014-9307-x
  9. Libe R, Fratticci A, Bertherat J. Adrenocortical cancer: pathophysiology and clinical management. Endocr Relat Cancer 2007;14:13-28.  https://doi.org/10.1677/erc.1.01130
  10. Korzeniowska D, Kostecka-Matyja M, Polak E, et al. Coincidence of pheochromocytoma and adrenocortical carcinoma at the same adrenal gland in a patient with neurofibromatosis. Endocrine Abstracts 2008;16:14. Poster presented at the 10th European Congress of Endocrinology [Internet]. European Society of Endocrinology; 2004 [cited 2024 Jun 20]. https://www.endocrine-abstracts.org/ea/0016/abstracts/poster-presentations/adrenal/ea0016p14/. 
  11. Fassnacht M, Dekkers OM, Else T, Baudin E, Berruti A, de Krijger R, 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:G1-46.  https://doi.org/10.1530/EJE-18-0608
  12. Allolio B, Fassnacht M. Clinical review: adrenocortical carcinoma. Clinical update. J Clin Endocrinol Metab 2006;91:2027-37.  https://doi.org/10.1210/jc.2005-2639
  13. Sada A, Asaad M, Bews KA, Thompson GB, Young WF, Bancos I, et al. Comparison between functional and non-functional adrenocortical carcinoma. Surgery 2020;167:216-23.  https://doi.org/10.1016/j.surg.2019.04.066
  14. Rosenberg R, Raeburn CD, Clay MR, Wierman ME. Dual aldosterone- and cortisol-secreting adrenal cortical carcinoma: pre- and perioperative evaluation and management. JCEM Case Rep 2023;1:luad073. 
  15. Funder JW, Carey RM, Fardella C, Gomez-Sanchez CE, Mantero F, Stowasser M, et al. Case detection, diagnosis, and treatment of patients with primary aldosteronism: an endocrine society clinical practice guideline. J Clin Endocrinol Metab 2008;93:3266-81.  https://doi.org/10.1210/jc.2008-0104
  16. Fassnacht M, Tsagarakis S, Terzolo M, Tabarin A, Sahdev A, Newell-Price J, et al. European Society of Endocrinology clinical practice guidelines on the management of adrenal incidentalomas, in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol 2023;189:G1-42.  https://doi.org/10.1093/ejendo/lvad066
  17. Gonzalez LAR, Rivas A, Moreno D, Percovich J. Metanephrine-producing adrenocortical carcinoma: a case report. Rev Latinoam Hipertens 2021;16:173-5. 
  18. Bagchi PK, Bora SJ, Barua SK, Thekumpadam Puthenveetil R. Giant adrenal tumor presenting as Cushing's syndrome and pheochromocytoma: a case report. Asian J Urol 2015;2:182-4.  https://doi.org/10.1016/j.ajur.2015.06.007
  19. Ni H, Htet A. Adrenal cortical carcinoma masquerading as pheochromocytoma: a case report. Ecancermedicalscience 2012;6:277. 
  20. Alsabek MB, Alhmaidi R, Ghazzawi B, Hamed G, Alseoudi A. Mixed corticomedullary adrenal carcinoma: case report. Comparison in features, treatment and prognosis with the other two reported cases. Int J Surg Case Rep 2017;31:254-61. https://doi.org/10.1016/j.ijscr.2017.01.010