Vol 75, No 4 (2024)
Guidelines / Expert consensus
Published online: 2024-08-19

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English

Polish diagnostic and therapeutic recommendations for adrenocortical carcinoma

Daria Handkiewicz-Junak1, Marek Dedecjus2, Urszula Ambroziak3, Marcin Barczyński4, Lucyna Bednarek-Papierska5, Ewa Chmielik1, Andrzej Cichocki2, Agnieszka Czarniecka1, Jarosław Ćwikła6, Kornelia Hasse-Lazar1, Alicja Hubalewska-Dydejczyk4, Joanna Januszkiewicz-Caulier2, Barbara Jarząb1, Grzegorz Kamiński7, Małgorzata Karbownik-Lewińska89, Agnieszka Kolasińska-Ćwikła2, Łukasz Koperski3, Beata Kos-Kudła10, Agnieszka Kotecka-Blicharz1, Aldona Kowalska1112, Jolanta Krajewska1, Leszek Królicki3, Andrzej Lewiński89, Iwona Michałowska13, Małgorzata Oczko-Wojciechowska1, Marek Ruchała14, Anhelli Syrenicz15, Andrzej Tysarowski2, Barbara Ziółkowska1, Agnieszka Żyłka2
Pubmed: 39279304
Endokrynol Pol 2024;75(4):339-358.

Abstract

Advances in the diagnosis and treatment of adrenocortical carcinoma (ACC), along with the development of new therapeutic and diagnostic methods, have prompted a team of experts to formulate the first Polish guidelines for managing ACC.

This article presents the diagnostic and therapeutic recommendations resulting from the discussion of specialists from various medical specialities, who participated in a series of online meetings aimed at developing consistent and effective recommendations under the National Oncology Strategy.

These guidelines aim to optimise ACC treatment in Poland through coordinated efforts of multidisciplinary specialist teams, ensuring an effective and modern approach. 

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References

  1. Benson III AB, Berman Z, Blaszkowsky LS, Brock P, Chan J, Center sCancer, et al. NCCN Guidelines Version 1.2024 Neuroendocrine and Adrenal Tumors NCCN Guidelines Panel Disclosures. 2024. https://www.nccn.org/home/member-.
  2. Fassnacht M, Assie G, Baudin E, et al. ESMO Guidelines Committee. Electronic address: clinicalguidelines@esmo.org. Adrenocortical carcinomas and malignant phaeochromocytomas: ESMO-EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2020; 31(11): 1476–1490.
  3. Fassnacht M, Arlt W, Bancos I, et al. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2016; 175(2): G1–G34.
  4. Fassnacht M, Dekkers O, 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–G46.
  5. Bednarczuk T, Bolanowski M, Sworczak K, et al. Adrenal incidentaloma in adults — management recommendations by the Polish Society of Endocrinology. Endokrynol Pol. 2016; 67(2): 234–258.
  6. Schwarte S, Brabant EG, Bastian L, et al. Cortisol as a possible marker of metastatic adrenocortical carcinoma: a case report with 3-year follow-up. Anticancer Res. 2007; 27(4A): 1917–1920.
  7. Januszewicz A, Januszewicz W, Jarząb B, et al. Grupa Robocza Polskiego Towarzystwa Nadciśnienia Tętniczego. Wytyczne dotyczące diagnostyki i leczenia chorych z guzem chromochłonnym [Diagnosis and management of patients with pheochromocytoma]. Nadciśnienie Tętn. 2006; 10(1): 1–19.
  8. Viëtor CL, Creemers SG, van Kemenade FJ, et al. How to Differentiate Benign from Malignant Adrenocortical Tumors? Cancers (Basel). 2021; 13(17).
  9. Fleseriu M. Salivary Cortisol in the Diagnosis of Cushing Syndrome, Always More Than One! J Endocr Soc. 2020; 4(10): bvaa109.
  10. Funder JW, Carey RM, Mantero F, et al. The Management of Primary Aldosteronism: Case Detection, Diagnosis, and Treatment: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2016; 101(5): 1889–1916.
  11. Sasano H, Suzuki T, Moriya T. Recent advances in histopathology and immunohistochemistry of adrenocortical carcinoma. Endocr Pathol. 2006; 17(4): 345–354.
  12. Suzuki S, Minamidate T, Shiga A, et al. Steroid metabolites for diagnosing and predicting clinicopathological features in cortisol-producing adrenocortical carcinoma. BMC Endocr Disord. 2020; 20(1): 173.
  13. Bancos I, Taylor AE, Chortis V, et al. ENSAT EURINE-ACT Investigators. Urine steroid metabolomics for the differential diagnosis of adrenal incidentalomas in the EURINE-ACT study: a prospective test validation study. Lancet Diabetes Endocrinol. 2020; 8(9): 773–781.
  14. Berke K, Constantinescu G, Masjkur J, et al. Plasma Steroid Profiling in Patients With Adrenal Incidentaloma. J Clin Endocrinol Metab. 2022; 107(3): e1181–e1192.
  15. Vogg N, Müller T, Floren A, et al. Simplified urinary steroid profiling by LC-MS as diagnostic tool for malignancy in adrenocortical tumors. Clin Chim Acta. 2023; 543: 117301.
  16. Chortis V, Bancos I, Nijman T, et al. Urine Steroid Metabolomics as a Novel Tool for Detection of Recurrent Adrenocortical Carcinoma. J Clin Endocrinol Metab. 2020; 105(3): e307–e318.
  17. Hamblin R, Coulden A, Fountas A, et al. The diagnosis and management of Cushing's syndrome in pregnancy. J Neuroendocrinol. 2022; 34(8): e13118.
  18. Ambroziak U, Kondracka A, Bartoszewicz Z, et al. The morning and late-night salivary cortisol ranges for healthy women may be used in pregnancy. Clin Endocrinol (Oxf). 2015; 83(6): 774–778.
  19. Savas M, Mehta S, Agrawal N, et al. Approach to the Patient: Diagnosis of Cushing Syndrome. J Clin Endocrinol Metab. 2022; 107(11): 3162–3174.
  20. Forestiero V, Sconfienza E, Mulatero P, et al. Primary aldosteronism in pregnancy. Rev Endocr Metab Disord. 2023; 24(1): 39–48.
  21. Else T. Association of adrenocortical carcinoma with familial cancer susceptibility syndromes. Mol Cell Endocrinol. 2012; 351(1): 66–70.
  22. Gonzalez KD, Noltner KA, Buzin CH, et al. Beyond Li Fraumeni Syndrome: clinical characteristics of families with p53 germline mutations. J Clin Oncol. 2009; 27(8): 1250–1256.
  23. Raymond VM, Else T, Everett JN, et al. Prevalence of germline TP53 mutations in a prospective series of unselected patients with adrenocortical carcinoma. J Clin Endocrinol Metab. 2013; 98(1): E119–E125.
  24. Renaux-Petel M, Charbonnier F, Théry JC, et al. Contribution of de novo and mosaic mutations to Li-Fraumeni syndrome. J Med Genet. 2018; 55(3): 173–180.
  25. Domènech M, Grau E, Solanes A, et al. Characteristics of Adrenocortical Carcinoma Associated With Lynch Syndrome. J Clin Endocrinol Metab. 2021; 106(2): 318–325.
  26. Gatta-Cherifi B, Chabre O, Murat A, et al. Adrenal involvement in MEN1. Analysis of 715 cases from the Groupe d'etude des Tumeurs Endocrines database. Eur J Endocrinol. 2012; 166(2): 269–279.
  27. Peppercorn PD, Grossman AB, Reznek RH. Imaging of incidentally discovered adrenal masses. Clin Endocrinol (Oxf). 1998; 48(4): 379–388.
  28. Caoili EM, Korobkin M, Francis IR, et al. Adrenal masses: characterization with combined unenhanced and delayed enhanced CT. Radiology. 2002; 222(3): 629–633.
  29. Ilias I, Sahdev A, Reznek RH, et al. The optimal imaging of adrenal tumours: a comparison of different methods. Endocr Relat Cancer. 2007; 14(3): 587–599.
  30. Mackie GC, Shulkin BL, Ribeiro RC, et al. Use of [18F]fluorodeoxyglucose positron emission tomography in evaluating locally recurrent and metastatic adrenocortical carcinoma. J Clin Endocrinol Metab. 2006; 91(7): 2665–2671.
  31. Groussin L, Bonardel G, Silvéra S, et al. 18F-Fluorodeoxyglucose positron emission tomography for the diagnosis of adrenocortical tumors: a prospective study in 77 operated patients. J Clin Endocrinol Metab. 2009; 94(5): 1713–1722.
  32. Deandreis D, Leboulleux S, Caramella C, et al. FDG PET in the management of patients with adrenal masses and adrenocortical carcinoma. Horm Cancer. 2011; 2(6): 354–362.
  33. Petersenn S, Richter PA, Broemel T, et al. German ACC Study Group. Computed tomography criteria for discrimination of adrenal adenomas and adrenocortical carcinomas: analysis of the German ACC registry. Eur J Endocrinol. 2015; 172(4): 415–422.
  34. Cistaro A, Niccoli Asabella A, Coppolino P, et al. Diagnostic and prognostic value of 18F-FDG PET/CT in comparison with morphological imaging in primary adrenal gland malignancies - a multicenter experience. Hell J Nucl Med. 2015; 18(2): 97–102.
  35. Nakajo M, Jinguji M, Nakajo M, et al. Texture analysis of FDG PET/CT for differentiating between FDG-avid benign and metastatic adrenal tumors: efficacy of combining SUV and texture parameters. Abdom Radiol (NY). 2017; 42(12): 2882–2889.
  36. Guerin C, Pattou F, Brunaud L, et al. Performance of 18F-FDG PET/CT in the Characterization of Adrenal Masses in Noncancer Patients: A Prospective Study. J Clin Endocrinol Metab. 2017; 102(7): 2465–2472.
  37. Corwin MT, Navarro SM, Malik DG, et al. Differences in Growth Rate on CT of Adrenal Adenomas and Malignant Adrenal Nodules. AJR Am J Roentgenol. 2019; 213(3): 632–636.
  38. Ahmed AA, Thomas AJ, Ganeshan DM, et al. Adrenal cortical carcinoma: pathology, genomics, prognosis, imaging features, and mimics with impact on management. Abdom Radiol (NY). 2020; 45(4): 945–963.
  39. Mayo-Smith WW, Song JH, Boland GL, et al. Management of Incidental Adrenal Masses: A White Paper of the ACR Incidental Findings Committee. J Am Coll Radiol. 2017; 14(8): 1038–1044.
  40. 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): 1612–1619.
  41. Bisceglia M, Ludovico O, Di Mattia A, et al. Adrenocortical oncocytic tumors: report of 10 cases and review of the literature. Int J Surg Pathol. 2004; 12(3): 231–243.
  42. Sinclair TJ, Gillis A, Alobuia WM, et al. Surgery for adrenocortical carcinoma: When and how? Best Pract Res Clin Endocrinol Metab. 2020; 34(3): 101408.
  43. Nilubol N, Patel D, Kebebew E. Does Lymphadenectomy Improve Survival in Patients with Adrenocortical Carcinoma? A Population-Based Study. World J Surg. 2016; 40(3): 697–705.
  44. Reibetanz J, Jurowich C, Erdogan I, et al. German ACC study group. Impact of lymphadenectomy on the oncologic outcome of patients with adrenocortical carcinoma. Ann Surg. 2012; 255(2): 363–369.
  45. Gerry JM, Tran TB, Postlewait LM, et al. Lymphadenectomy for Adrenocortical Carcinoma: Is There a Therapeutic Benefit? Ann Surg Oncol. 2016; 23(Suppl 5): 708–713.
  46. Reibetanz J, Rinn B, Kunz AS, et al. Patterns of Lymph Node Recurrence in Adrenocortical Carcinoma: Possible Implications for Primary Surgical Treatment. Ann Surg Oncol. 2019; 26(2): 531–538.
  47. Zsippai A, Szabó DR, Tömböl Z, et al. Effects of mitotane on gene expression in the adrenocortical cell line NCI-H295R: a microarray study. Pharmacogenomics. 2012; 13(12): 1351–1361.
  48. Puglisi S, Calabrese A, Basile V, et al. New perspectives for mitotane treatment of adrenocortical carcinoma. Best Pract Res Clin Endocrinol Metab. 2020; 34(3): 101415.
  49. Baudin E, Pellegriti G, Bonnay M, et al. Impact of monitoring plasma 1,1-dichlorodiphenildichloroethane (o,p'DDD) levels on the treatment of patients with adrenocortical carcinoma. Cancer. 2001; 92(6): 1385–1392, doi: 10.1002/1097-0142(20010915)92:6<1385::aid-cncr1461>3.0.co;2-2.
  50. Russo M, Scollo C, Pellegriti G, et al. Mitotane treatment in patients with adrenocortical cancer causes central hypothyroidism. Clin Endocrinol (Oxf). 2016; 84(4): 614–619.
  51. Chortis V, Taylor AE, Schneider P, et al. Mitotane therapy in adrenocortical cancer induces CYP3A4 and inhibits 5α-reductase, explaining the need for personalized glucocorticoid and androgen replacement. J Clin Endocrinol Metab. 2013; 98(1): 161–171.
  52. Kroiss M, Quinkler M, Lutz WK, et al. Drug interactions with mitotane by induction of CYP3A4 metabolism in the clinical management of adrenocortical carcinoma. Clin Endocrinol (Oxf). 2011; 75(5): 585–591.
  53. Fassnacht M, Libé R, Kroiss M, et al. Adrenocortical carcinoma: a clinician's update. Nat Rev Endocrinol. 2011; 7(6): 323–335.
  54. Terzolo M, Angeli A, Fassnacht M, et al. Adjuvant mitotane treatment for adrenocortical carcinoma. N Engl J Med. 2007; 356(23): 2372–2380.
  55. Berruti A, Grisanti S, Pulzer A, et al. Long-Term Outcomes of Adjuvant Mitotane Therapy in Patients With Radically Resected Adrenocortical Carcinoma. J Clin Endocrinol Metab. 2017; 102(4): 1358–1365.
  56. Terzolo M, Fassnacht M, Perotti P, 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. Lancet Diabetes Endocrinol. 2023; 11(10): 720–730.
  57. Nelson DW, Chang SC, Bandera BC, et al. Adjuvant Radiation is Associated with Improved Survival for Select Patients with Non-metastatic Adrenocortical Carcinoma. Ann Surg Oncol. 2018; 25(7): 2060–2066.
  58. Wu K, Liu Xu, Liu Z, et al. Benefit of Postoperative Radiotherapy for Patients With Nonmetastatic Adrenocortical Carcinoma: A Population-Based Analysis. J Natl Compr Canc Netw. 2021; 19(12): 1425–1432.
  59. Baudin E, Leboulleux S, Al Ghuzlan A, et al. Therapeutic management of advanced adrenocortical carcinoma: what do we know in 2011? Horm Cancer. 2011; 2(6): 363–371.
  60. Hermsen IGC, Gelderblom H, Kievit J, et al. Extremely long survival in six patients despite recurrent and metastatic adrenal carcinoma. Eur J Endocrinol. 2008; 158(6): 911–919.
  61. Libé R, Borget I, Ronchi CL, et al. ENSAT network. Prognostic factors in stage III-IV adrenocortical carcinomas (ACC): an European Network for the Study of Adrenal Tumor (ENSAT) study. Ann Oncol. 2015; 26(10): 2119–2125.
  62. Megerle F, Herrmann W, Schloetelburg W, et al. German ACC Study Group. Mitotane Monotherapy in Patients With Advanced Adrenocortical Carcinoma. J Clin Endocrinol Metab. 2018; 103(4): 1686–1695.
  63. Reidy-Lagunes DL, Lung B, Untch BR, et al. Complete Responses to Mitotane in Metastatic Adrenocortical Carcinoma-A New Look at an Old Drug. Oncologist. 2017; 22(9): 1102–1106.
  64. Vezzosi D, Do Cao C, Hescot S, et al. For Comete-Cancer Network. Time Until Partial Response in Metastatic Adrenocortical Carcinoma Long-Term Survivors. Horm Cancer. 2018; 9(1): 62–69.
  65. Fassnacht M, Terzolo M, Allolio B, et al. FIRM-ACT Study Group. Combination chemotherapy in advanced adrenocortical carcinoma. N Engl J Med. 2012; 366(23): 2189–2197.
  66. Bukowski RM, Wolfe M, Levine HS, et al. Phase II trial of mitotane and cisplatin in patients with adrenal carcinoma: a Southwest Oncology Group study. J Clin Oncol. 1993; 11(1): 161–165.
  67. Williamson SK, Lew D, Miller GJ, et al. Phase II evaluation of cisplatin and etoposide followed by mitotane at disease progression in patients with locally advanced or metastatic adrenocortical carcinoma: a Southwest Oncology Group Study. Cancer. 2000; 88(5): 1159–1165.
  68. Kimpel O, Schindler P, Schmidt-Pennington L, et al. Efficacy and safety of radiation therapy in advanced adrenocortical carcinoma. Br J Cancer. 2023; 128(4): 586–593.
  69. Sperone P, Ferrero A, Daffara F, et al. Gemcitabine plus metronomic 5-fluorouracil or capecitabine as a second-/third-line chemotherapy in advanced adrenocortical carcinoma: a multicenter phase II study. Endocr Relat Cancer. 2010; 17(2): 445–453.
  70. Henning JEK, Deutschbein T, Altieri B, et al. Gemcitabine-Based Chemotherapy in Adrenocortical Carcinoma: A Multicenter Study of Efficacy and Predictive Factors. J Clin Endocrinol Metab. 2017; 102(11): 4323–4332.
  71. Cosentini D, Badalamenti G, Grisanti S, et al. Activity and safety of temozolomide in advanced adrenocortical carcinoma patients. Eur J Endocrinol. 2019; 181(6): 681–689.
  72. Campbell MT, Jimenez C, Long JP, et al. 1MO An open-label, phase II trial of cabozantinib for advanced adrenocortical carcinoma. Ann Oncol. 2022; 33: S545.
  73. Kroiss M, Megerle F, Kurlbaum M, et al. Objective Response and Prolonged Disease Control of Advanced Adrenocortical Carcinoma with Cabozantinib. J Clin Endocrinol Metab. 2020; 105(5): 1461–1468.
  74. Burotto M, Tageja N, Rosenberg A, et al. Brain metastasis in patients with adrenocortical carcinoma: a clinical series. J Clin Endocrinol Metab. 2015; 100(2): 331–336.