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Localised adrenocortical carcinoma is adrenal cortex cancer still confined to the adrenal gland and its surroundings that surgery can remove, the only treatment that cures it. Afterwards the adrenal-specific drug mitotane is given to patients whose tumour has a high risk of returning, judged by the Ki-67 index and whether it was completely removed, while low-risk patients are watched.
Adrenocortical carcinoma is a rare cancer of the steroid-producing adrenal cortex. About six in ten tumours secrete hormones, most often cortisol (Cushing's syndrome) or androgens (virilisation in women), and the rest are found as incidental or symptomatic masses; a full hormone work-up before surgery both establishes the diagnosis and prepares the patient for adrenal insufficiency afterwards. The Weiss score confirms malignancy on pathology, the Ki-67 index grades it, and the ENSAT system stages it: stage I and II tumours are confined to the adrenal, stage III has invaded surrounding tissue or nodes, and stage IV has metastasised. Children with adrenocortical carcinoma nearly always carry a germline TP53 mutation, including the R337H founder mutation of southern Brazil, and do better than adults when the tumour is resected; adults are tested for Lynch syndrome and Li-Fraumeni syndrome.
Open adrenalectomy with en bloc removal of adherent structures and regional nodes by an experienced surgeon is the standard for suspected carcinoma; laparoscopic surgery is reserved for small tumours and tumour rupture must be avoided because it seeds the peritoneum. Even after complete resection the disease often returns, so the 2018 ESE/ENSAT guideline recommends adjuvant mitotane, the adrenolytic drug approved in 1970, for patients at high risk of recurrence (Ki-67 above 10 percent, stage III or incomplete resection), titrated to plasma levels of 14 to 20 mg/L and given for at least two years alongside hydrocortisone replacement. The ADIUVO trial (Lancet Diabetes and Endocrinology 2023) randomised low-risk patients (stage I to III, complete resection, Ki-67 of 10 percent or less) to mitotane or observation and found no benefit, so observation is now the standard for that group. Postoperative radiotherapy to the tumour bed is considered for incomplete resection, and platinum-based chemotherapy is added to mitotane for very high-risk tumours in some centres, although the randomised ADIUVO-2 trial addressing that question is still recruiting. Follow-up imaging every three months in the first years catches recurrences that can sometimes be resected again.
Roughly half to two thirds of adrenocortical carcinomas are found without distant metastases, often as a large adrenal mass causing Cushing's syndrome or virilisation, or incidentally on a scan; recurrence after surgery is nonetheless common.
Renal cell carcinoma comes from the kidney's filtering cortex, urothelial cancer from the lining of the collecting system and bladder, and the adrenal on top hosts cortical and medullary (neuroblastoma) tumours.
Same organ: Collecting duct carcinoma of the kidney, Renal medullary carcinoma (SMARCB1-deficient), TFE3-rearranged (translocation) renal cell carcinoma, Fumarate hydratase-deficient renal cell carcinoma (HLRCC-associated), Succinate dehydrogenase-deficient renal cell carcinoma, Mucinous tubular and spindle cell carcinoma of the kidney, Eosinophilic solid and cystic renal cell carcinoma, Clear cell papillary renal cell tumour, Urothelial carcinoma of the urethra, Squamous cell carcinoma of the urethra, Adenocarcinoma of the urethra (including clear cell adenocarcinoma), Melanoma of the urethra, Non-muscle-invasive bladder cancer, Muscle-invasive and advanced bladder cancer, Bladder & urothelial cancer, Clear cell renal cell carcinoma, Papillary renal cell carcinoma, Chromophobe renal cell carcinoma, Renal cell carcinoma, Wilms tumour (nephroblastoma), Neuroblastoma (paediatric), Low-risk neuroblastoma (INRG very low and low risk, including stage MS), Intermediate-risk neuroblastoma, High-risk neuroblastoma, Adrenocortical carcinoma, Pheochromocytoma and paraganglioma (PPGL), Urethral cancer, Penile cancer, Localised penile cancer (organ-confined, node-negative), Node-positive and metastatic penile cancer, Advanced and metastatic adrenocortical carcinoma (ENSAT stage IV or unresectable), Hereditary pheochromocytoma and paraganglioma (SDHx, VHL, RET, NF1, MAX and TMEM127), Metastatic pheochromocytoma and paraganglioma
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Also on OnCo: Symptoms and red flags · Early detection roadmap.
Hormone work-up, contrast CT or MRI of the adrenal, chest CT and FDG-PET; no biopsy of a resectable adrenal mass; germline testing.
Open adrenalectomy with en bloc resection of adherent structures and locoregional lymphadenectomy by an experienced surgeon; laparoscopic surgery only for small tumours; perioperative hydrocortisone for cortisol-secreting tumours.
Observation with imaging every three months (ADIUVO showed no benefit from mitotane).
Adjuvant mitotane titrated to 14 to 20 mg/L for at least two years with glucocorticoid replacement; tumour-bed radiotherapy after incomplete resection; platinum-based chemotherapy considered for very high-risk tumours (ADIUVO-2).
Repeat resection when feasible after a disease-free interval of a year or more, with mitotane; ablation or radiotherapy for small unresectable recurrences.
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Adjuvant mitotane may be withheld in low-grade localised adrenocortical carcinoma, given the good prognosis and the drug's toxicity.
The standard-of-care rows on both adrenocortical carcinoma pages, from who gets mitotane after surgery to the EDP-M regimen, follow this guideline.
Query for this cancer: (TITLE:"Localised adrenocortical carcinoma" OR ABSTRACT:"Localised adrenocortical carcinoma" OR TITLE:"ENSAT stage I to III, resectable" OR ABSTRACT:"ENSAT stage I to III, resectable" OR TITLE:"Resectable adrenocortical carcinoma" OR ABSTRACT:"Resectable adrenocortical carcinoma" OR TITLE:"Early-stage ACC" OR ABSTRACT:"Early-stage ACC" OR TITLE:"Non-metastatic adrenal cortical carcinoma" OR ABSTRACT:"Non-metastatic adrenal cortical carcinoma" OR TITLE:"Adrenocortical carcinoma after complete resection" OR ABSTRACT:"Adrenocortical carcinoma after complete resection") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Localised adrenocortical carcinoma (ENSAT stage I to III, resectable), not a curated reading list.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Dose reduce or avoid for CrCl below 60 (carboplatin is the alternative).
Reduce to 75% for CrCl 15-50.
The leukaemia risk after chemotherapy was described in the era of mustards and etoposide, and it did not stay there. Platinum drugs carry it, PARP inhibitors raise it about two and a half times against placebo, and lenalidomide with oral melphalan raises it nearly fivefold against melphalan alone. The absolute numbers are small, but the choice of partner drug is sometimes a real decision.
Etoposide and the anthracyclines can cause a leukaemia too, but a different one: it arrives after about two years rather than six, it starts as acute leukaemia without a myelodysplastic phase, and it carries a balanced break in a chromosome rather than a missing piece. So the first two or three years after this chemotherapy are when a blood count matters most.
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