EP1744 - LBA_ECE_1037 - Adrenocortical carcinoma: a case series highlighting diagnostic challenges, hormonal heterogeneity and therapeutic complexity

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Abstract Introduction Adrenocortical carcinoma (ACC) is a rare malignancy (0.5-2 cases/million/year), characterised by heterogeneous clinical presentation, variable hormonal secretion and poor prognosis in advanced stages. Multidisciplinary management combining surgery, mitotane and cytotoxic chemotherapy remains the gold standard of treatment. We report three cases illustrating distinct clinical phenotypes of ACC managed at a tertiary endocrine centre. Case Presentations Case 1: A 39-year-old woman presented with hypertension, hirsutism and generalised oedema. Hormonal evaluation demonstrated cortisol and androgen secretion (suppressed adrenocorticotropic hormone, elevated dehydroepiandrosterone sulfate, testosterone, urinary free cortisol and no suppression of cortisol with 1 mg dexamethasone). Computed tomography (CT) identified a right retroperitoneal mass of 98 × 94 × 98 mm. Laparoscopic right adrenalectomy confirmed high-grade ACC (pT2NxMxL1V1, Ki67 15%-20%). Post-operative corticotropic insufficiency required glucocorticoid substitution. Adjuvant mitotane was initiated and therapeutic levels were achieved slowly. Serial imaging showed no recurrence, though mitotane side effects required dose adjustments. Case 2: A 52-year-old man presented with left lumbar pain. CT identified a left retroperitoneal mass of 133 × 90 × 136 mm, bilateral pulmonary nodules, locoregional adenopathy and a right adrenal nodule. Extended surgery included tumour resection with left nephrectomy, splenectomy, body and tail pancreatectomy and periaortic lymph node dissection. Histopathology confirmed non-functioning high-grade ACC (Ki67~60%). Post-operative staging revealed multiple hepatic metastases and therapy with mitotane combined with EDP (etoposide, doxorubicin, cisplatin) chemotherapy was initiated. Case 3: A 50-year-old male patient was incidentally diagnosed with a left adrenal mass of 12 × 7 × 8 cm on a CT performed for suspected disc herniation. Partial left adrenalectomy with splenectomy and distal pancreatectomy confirmed a low-grade oncocytic ACC (pT2L0V0R0M0, Ki67 10%). Subsequent imaging revealed local recurrence and hepatic metastases, requiring reoperation with resection of the pancreatic body and tail, residual left adrenal, atypical hepatic resection (segments II and V), partial gastrectomy and omentectomy. Adjuvant mitotane and EDP chemotherapy were initiated. Discussion The present case series illustrates the clinical, hormonal and histological heterogeneity of ACC: a functioning localised tumour with slow mitotane titration, a non-functioning metastatic high-grade ACC requiring systemic chemotherapy and a low-grade oncocytic ACC with metastatic relapse requiring repeated surgical interventions. These cases highlight the challenges of achieving therapeutic mitotane levels, managing adrenocortical insufficiency and coordinating multidisciplinary oncological care. Conclusion The cases presented confirm that no two ACC presentations are alike. Histological subtype, functional status and disease stage at diagnosis influence therapeutic strategy and long-term outcomes in this aggressive malignancy.

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