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Endocrinology116 papers

Adrenocortical hemorrhage

Last edited: 4/14/2026

Overview

Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy arising from the adrenal cortex, often characterized by hormone overproduction and poor prognosis. Diagnosis and management remain challenging due to its rarity and heterogeneity.

Diagnosis

  • Clinical Presentation: Symptoms may include hormonal excess (Cushingoid, virilization, feminization) and nonspecific signs like abdominal pain or mass 162932.
  • Imaging: CT and MRI are essential for tumor characterization, staging, and detection of metastases 1711.
  • Biopsy and Histopathology: Core needle biopsy or surgical resection with histopathological examination using Weiss score for malignancy assessment 424.
  • Markers: Elevated serum levels of adrenal hormones (cortisol, aldosterone, androgens) and tumor markers like Ki67 for prognostic stratification 1722.
  • Staging: Utilize the European Network for the Study of Adrenal Tumors (ENSAT) staging system for accurate clinical staging 17.

    • Management

  • Surgical Resection: Complete surgical removal is the primary treatment for localized disease 118.
  • Adjuvant Therapy: Mitotane is recommended as adjuvant therapy for low-grade, localized ACC to reduce recurrence risk 31113.
  • Targeted Therapy: Emerging evidence suggests potential benefits of EZH2 inhibitors for aggressive forms 5.
  • Systemic Therapy: Limited options include chemotherapy regimens like EMA-CO, with ongoing research into novel treatments 418.
  • Local Ablation: Image-guided ablation for oligometastatic disease, particularly in liver and lung metastases 15.
  • Supportive Care: Management of adrenal insufficiency and hormonal imbalances post-treatment 617.

    • Special Populations

  • Pediatrics: Higher incidence in certain populations due to genetic mutations (e.g., TP53); tailored management strategies are evolving 814.
  • Comorbidities: Increased risk of venous thromboembolism; prophylactic measures recommended 7.

    • Key Recommendations

  • Surgical resection for localized ACC is the cornerstone of treatment (Evidence: Strong 118).
  • Adjuvant mitotane should be considered for low-grade, localized ACC to improve recurrence-free survival (Evidence: Moderate 311).
  • Use of ENSAT staging system for accurate clinical staging and guiding treatment decisions (Evidence: Expert opinion 17).
  • Consider image-guided ablation for patients with limited metastatic disease (Evidence: Moderate 15).
  • Proactive management of adrenal insufficiency post-treatment to address complications (Evidence: Moderate 617).

    • References

    1 Handkiewicz-Junak D, Dedecjus M, Ambroziak U, Barczyński M, Bednarek-Papierska L, Chmielik E et al.. Polish diagnostic and therapeutic recommendations for adrenocortical carcinoma. Endokrynologia Polska 2024. link 2 de Ponthaud C, Bekada S, Buffet C, Roy M, Bachelot A, Ayed A et al.. Which lymphadenectomy for adrenocortical carcinoma?. Surgery 2024. link 3 Terzolo M, Fassnacht M, Perotti P, Libé R, Kastelan D, Lacroix A et al.. Adjuvant mitotane versus surveillance in low-grade, localised adrenocortical carcinoma (ADIUVO): an international, multicentre, open-label, randomised, phase 3 trial and observational study. The lancet. Diabetes & endocrinology 2023. link00193-6) 4 Padua TC, Marandino L, Raggi D, Hallanger-Johnson J, Kutikov A, Spiess PE et al.. A Systematic Review of Published Clinical Trials in the Systemic Treatment of Adrenocortical Carcinoma: An Initiative Led on Behalf of the Global Society of Rare Genitourinary Tumors. Clinical genitourinary cancer 2023. link 5 Ferrarelli LK. Exploiting an epigenetic switch in adrenal cancer. Science signaling 2023. link 6 Yeoh P, Czuber-Dochan W, Aylwin S, Sturt J. Lived experience of people with adrenocortical carcinoma and associated adrenal insufficiency. Endocrinology, diabetes & metabolism 2022. link 7 Badesha AS, Khan T, Abdellatif E. Iliac vein deep vein thrombosis as an atypical presentation of an adrenocortical carcinoma. BMJ case reports 2022. link 8 Zambaiti E, Duci M, De Corti F, Gamba P, Dall'Igna P, Ghidini F et al.. Clinical prognostic factors in pediatric adrenocortical tumors: A meta-analysis. Pediatric blood & cancer 2021. link 9 Skrzypiec-Spring M, Kuliczkowska-Płaksej J, Stachowska B, Szeląg A, Kustrzycki W, Bolanowski M. Metastasis of adrenocortical carcinoma to the heart. Endokrynologia Polska 2021. link 10 Crona J, Baudin E, Terzolo M, Chrisoulidou A, Angelousi A, Ronchi CL et al.. ENSAT registry-based randomized clinical trials for adrenocortical carcinoma. European journal of endocrinology 2021. link 11 Amodru V, Garcia ME, Libe R, Brue T, Reznik Y, Castinetti F. Medical management of adrenocortical carcinoma: Current recommendations, new therapeutic options and future perspectives. Annales d'endocrinologie 2021. link 12 Bansari A, Radhakrishnan NS, Kattan JN. A rare case of right shoulder pain. BMJ case reports 2021. link 13 Puglisi S, Calabrese A, Basile V, Pia A, Reimondo G, Perotti P et al.. New perspectives for mitotane treatment of adrenocortical carcinoma. Best practice & research. Clinical endocrinology & metabolism 2020. link 14 Brondani VB, Fragoso MCBV. Pediatric adrenocortical tumor - review and management update. Current opinion in endocrinology, diabetes, and obesity 2020. link 15 Veltri A, Basile D, Calandri M, Bertaggia C, Volante M, Porpiglia F et al.. Oligometastatic adrenocortical carcinoma: the role of image-guided thermal ablation. European radiology 2020. link 16 Gupta A, Elfiky A. Novel findings from determination of common expressed plasma exosomal microRNAs in patients with psoriatic arthritis, psoriasis vulgaris, rheumatoid arthritis, and gouty arthritis. Discovery medicine 2019. link 17 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. European journal of endocrinology 2018. link 18 Varghese J, Habra MA. Update on adrenocortical carcinoma management and future directions. Current opinion in endocrinology, diabetes, and obesity 2017. link 19 Pilla M, Gilbert J, Moore L, Byard RW. Occult Adrenocortical Carcinoma and Unexpected Early Childhood Death. Journal of forensic sciences 2017. link 20 . Piecing Together the Adrenal Cancer Puzzle. Cancer discovery 2016. link 21 Park JJ, Park BK, Kim JH, Jeong BC, Kim CK. Salvage computed tomography-guided transhepatic radiofrequency ablation for unresected aldosteronoma of adrenohepatic fusion after adrenalectomy. International journal of urology : official journal of the Japanese Urological Association 2016. link 22 Papathomas TG, Pucci E, Giordano TJ, Lu H, Duregon E, Volante M et al.. An International Ki67 Reproducibility Study in Adrenal Cortical Carcinoma. The American journal of surgical pathology 2016. link 23 Lafemina J, Brennan MF. Adrenocortical carcinoma: past, present, and future. Journal of surgical oncology 2012. link 24 Tissier F, Aubert S, Leteurtre E, Al Ghuzlan A, Patey M, Decaussin M et al.. Adrenocortical tumors: improving the practice of the Weiss system through virtual microscopy: a National Program of the French Network INCa-COMETE. The American journal of surgical pathology 2012. link 25 Subbiah S, Walia R, Kumar S, Nahar U, Bhansali A. An unusual cause of gynaecomastia. BMJ case reports 2011. link 26 Fassnacht M, Libé R, Kroiss M, Allolio B. Adrenocortical carcinoma: a clinician's update. Nature reviews. Endocrinology 2011. link 27 Juliano JJ, Cody RL, Suh JH. Metastatic adrenocortical oncocytoma: a case report. Urologic oncology 2008. link 28 Castellano JJ, Warren MW, Arroyo MR, Cendan JC. Laparoscopic resection of a virilizing adrenocortical tumor. JSLS : Journal of the Society of Laparoendoscopic Surgeons 2008. link 29 Mann MW, Ellis SS, Mallory SB. Infantile acne as the initial sign of an adrenocortical tumor. Journal of the American Academy of Dermatology 2007. link 30 Ghazi AA, Mofid D, Rahimi F, Sadeghi-Nejad A. Cortisol and estradiol secretion by a benign virilizing adrenocortical tumor in a prepubertal girl. Journal of pediatric endocrinology & metabolism : JPEM 2004. link 31 Nakata A, Yagi S, Oyama K, Kida H, Sugioka G. Adrenocortical carcinoma with a giant pericardial mass. Internal medicine (Tokyo, Japan) 1993. link 32 Lanigan D, Choa RG, Evans J. A feminizing adrenocortical carcinoma presenting with gynaecomastia. Postgraduate medical journal 1993. link 33 Karagiannis A, Pikramenos D, Vlasssopoulos G, Sofras F, Dimopoulos C. Non functioning adrenocortical carcinoma. A rare case. Acta urologica Belgica 1992. link 34 Saha PK, Ura T, Suzu H, Yamashita S, Yushita Y, Kanetake H et al.. A case of deoxycorticosterone-producing benign adrenocortical tumor. Urologia internationalis 1990. link 35 Kitazawa M, Kaneko H, Toshima M, Ishikawa H, Watanabe H, Takagi A et al.. Crystalloid structure in adrenocortical adenoma. Acta pathologica japonica 1985. link 36 Klein FA, Miller NL, Hackler RH. Flow cytometry in feminizing adrenocortical carcinoma. The Journal of urology 1985. link47533-1) 37 Touitou Y, Bogdan A, Auzeby A. Experimental evidence for biosynthesis of steroids in metastatic tissue originating from a primitive adrenocortical carcinoma. The International journal of biochemistry 1983. link90133-7) 38 Smith JM, Keane FB, O'Flynn JD, Collins PG. Primary nonfunctioning carcinoma of adrenal cortex. Urology 1979. link90413-8) 39 Marsden HB, Jones PM, Lees PD, Hann IM. Late functioning adrenocortical carcinoma in a 5-year-old girl. Archives of disease in childhood 1978. link

    Original source

    1. [1]
      Polish diagnostic and therapeutic recommendations for adrenocortical carcinoma. — Handkiewicz-Junak D, Dedecjus M, Ambroziak U, Barczyński M, Bednarek-Papierska L, Chmielik E et al. Endokrynologia Polska (2024)
    2. [2]
      Which lymphadenectomy for adrenocortical carcinoma? — de Ponthaud C, Bekada S, Buffet C, Roy M, Bachelot A, Ayed A et al. Surgery (2024)
    3. [3]
      Adjuvant mitotane versus surveillance in low-grade, localised adrenocortical carcinoma (ADIUVO): an international, multicentre, open-label, randomised, phase 3 trial and observational study. — Terzolo M, Fassnacht M, Perotti P, Libé R, Kastelan D, Lacroix A et al. The lancet. Diabetes & endocrinology (2023)
    4. [4]
      A Systematic Review of Published Clinical Trials in the Systemic Treatment of Adrenocortical Carcinoma: An Initiative Led on Behalf of the Global Society of Rare Genitourinary Tumors. — Padua TC, Marandino L, Raggi D, Hallanger-Johnson J, Kutikov A, Spiess PE et al. Clinical genitourinary cancer (2023)
    5. [5]
      Exploiting an epigenetic switch in adrenal cancer. — Ferrarelli LK Science signaling (2023)
    6. [6]
      Lived experience of people with adrenocortical carcinoma and associated adrenal insufficiency. — Yeoh P, Czuber-Dochan W, Aylwin S, Sturt J Endocrinology, diabetes & metabolism (2022)
    7. [7]
      Iliac vein deep vein thrombosis as an atypical presentation of an adrenocortical carcinoma. — Badesha AS, Khan T, Abdellatif E BMJ case reports (2022)
    8. [8]
      Clinical prognostic factors in pediatric adrenocortical tumors: A meta-analysis. — Zambaiti E, Duci M, De Corti F, Gamba P, Dall'Igna P, Ghidini F et al. Pediatric blood & cancer (2021)
    9. [9]
      Metastasis of adrenocortical carcinoma to the heart. — Skrzypiec-Spring M, Kuliczkowska-Płaksej J, Stachowska B, Szeląg A, Kustrzycki W, Bolanowski M Endokrynologia Polska (2021)
    10. [10]
      ENSAT registry-based randomized clinical trials for adrenocortical carcinoma. — Crona J, Baudin E, Terzolo M, Chrisoulidou A, Angelousi A, Ronchi CL et al. European journal of endocrinology (2021)
    11. [11]
      Medical management of adrenocortical carcinoma: Current recommendations, new therapeutic options and future perspectives. — Amodru V, Garcia ME, Libe R, Brue T, Reznik Y, Castinetti F Annales d'endocrinologie (2021)
    12. [12]
      A rare case of right shoulder pain. — Bansari A, Radhakrishnan NS, Kattan JN BMJ case reports (2021)
    13. [13]
      New perspectives for mitotane treatment of adrenocortical carcinoma. — Puglisi S, Calabrese A, Basile V, Pia A, Reimondo G, Perotti P et al. Best practice & research. Clinical endocrinology & metabolism (2020)
    14. [14]
      Pediatric adrenocortical tumor - review and management update. — Brondani VB, Fragoso MCBV Current opinion in endocrinology, diabetes, and obesity (2020)
    15. [15]
      Oligometastatic adrenocortical carcinoma: the role of image-guided thermal ablation. — Veltri A, Basile D, Calandri M, Bertaggia C, Volante M, Porpiglia F et al. European radiology (2020)
    16. [16]
      Novel findings from determination of common expressed plasma exosomal microRNAs in patients with psoriatic arthritis, psoriasis vulgaris, rheumatoid arthritis, and gouty arthritis. — Gupta A, Elfiky A Discovery medicine (2019)
    17. [17]
      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. — Fassnacht M, Dekkers OM, Else T, Baudin E, Berruti A, de Krijger R et al. European journal of endocrinology (2018)
    18. [18]
      Update on adrenocortical carcinoma management and future directions. — Varghese J, Habra MA Current opinion in endocrinology, diabetes, and obesity (2017)
    19. [19]
      Occult Adrenocortical Carcinoma and Unexpected Early Childhood Death. — Pilla M, Gilbert J, Moore L, Byard RW Journal of forensic sciences (2017)
    20. [20]
      Piecing Together the Adrenal Cancer Puzzle. Cancer discovery (2016)
    21. [21]
      Salvage computed tomography-guided transhepatic radiofrequency ablation for unresected aldosteronoma of adrenohepatic fusion after adrenalectomy. — Park JJ, Park BK, Kim JH, Jeong BC, Kim CK International journal of urology : official journal of the Japanese Urological Association (2016)
    22. [22]
      An International Ki67 Reproducibility Study in Adrenal Cortical Carcinoma. — Papathomas TG, Pucci E, Giordano TJ, Lu H, Duregon E, Volante M et al. The American journal of surgical pathology (2016)
    23. [23]
      Adrenocortical carcinoma: past, present, and future. — Lafemina J, Brennan MF Journal of surgical oncology (2012)
    24. [24]
      Adrenocortical tumors: improving the practice of the Weiss system through virtual microscopy: a National Program of the French Network INCa-COMETE. — Tissier F, Aubert S, Leteurtre E, Al Ghuzlan A, Patey M, Decaussin M et al. The American journal of surgical pathology (2012)
    25. [25]
      An unusual cause of gynaecomastia. — Subbiah S, Walia R, Kumar S, Nahar U, Bhansali A BMJ case reports (2011)
    26. [26]
      Adrenocortical carcinoma: a clinician's update. — Fassnacht M, Libé R, Kroiss M, Allolio B Nature reviews. Endocrinology (2011)
    27. [27]
      Metastatic adrenocortical oncocytoma: a case report. — Juliano JJ, Cody RL, Suh JH Urologic oncology (2008)
    28. [28]
      Laparoscopic resection of a virilizing adrenocortical tumor. — Castellano JJ, Warren MW, Arroyo MR, Cendan JC JSLS : Journal of the Society of Laparoendoscopic Surgeons (2008)
    29. [29]
      Infantile acne as the initial sign of an adrenocortical tumor. — Mann MW, Ellis SS, Mallory SB Journal of the American Academy of Dermatology (2007)
    30. [30]
      Cortisol and estradiol secretion by a benign virilizing adrenocortical tumor in a prepubertal girl. — Ghazi AA, Mofid D, Rahimi F, Sadeghi-Nejad A Journal of pediatric endocrinology & metabolism : JPEM (2004)
    31. [31]
      Adrenocortical carcinoma with a giant pericardial mass. — Nakata A, Yagi S, Oyama K, Kida H, Sugioka G Internal medicine (Tokyo, Japan) (1993)
    32. [32]
      A feminizing adrenocortical carcinoma presenting with gynaecomastia. — Lanigan D, Choa RG, Evans J Postgraduate medical journal (1993)
    33. [33]
      Non functioning adrenocortical carcinoma. A rare case. — Karagiannis A, Pikramenos D, Vlasssopoulos G, Sofras F, Dimopoulos C Acta urologica Belgica (1992)
    34. [34]
      A case of deoxycorticosterone-producing benign adrenocortical tumor. — Saha PK, Ura T, Suzu H, Yamashita S, Yushita Y, Kanetake H et al. Urologia internationalis (1990)
    35. [35]
      Crystalloid structure in adrenocortical adenoma. — Kitazawa M, Kaneko H, Toshima M, Ishikawa H, Watanabe H, Takagi A et al. Acta pathologica japonica (1985)
    36. [36]
      Flow cytometry in feminizing adrenocortical carcinoma. — Klein FA, Miller NL, Hackler RH The Journal of urology (1985)
    37. [37]
      Experimental evidence for biosynthesis of steroids in metastatic tissue originating from a primitive adrenocortical carcinoma. — Touitou Y, Bogdan A, Auzeby A The International journal of biochemistry (1983)
    38. [38]
      Primary nonfunctioning carcinoma of adrenal cortex. — Smith JM, Keane FB, O'Flynn JD, Collins PG Urology (1979)
    39. [39]
      Late functioning adrenocortical carcinoma in a 5-year-old girl. — Marsden HB, Jones PM, Lees PD, Hann IM Archives of disease in childhood (1978)
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