endocrine

Adrenocortical carcinoma is a rare, aggressive cancer of the adrenal gland that often over-produces hormones. Surgery is the only cure, mitotane is the one drug specific to it (with real toxicity), and chemotherapy or immunotherapy help only a minority.

Advanced adrenocortical carcinoma is adrenal cortex cancer that has spread to distant organs or cannot be removed, one of the hardest endocrine cancers to treat. The standard is mitotane with etoposide, doxorubicin and cisplatin, established by the FIRM-ACT trial; limited spread is treated locally, hormone excess with steroid-blocking drugs, and immunotherapy helps a minority.

Extrapulmonary neuroendocrine carcinoma is the fast-growing, poorly differentiated form of neuroendocrine cancer arising outside the lung, most often in the bowel, oesophagus, stomach or pancreas. It behaves like small-cell lung cancer and is treated the same way, with platinum and etoposide chemotherapy, and drugs against the DLL3 protein are now in phase 3 trials.

Grade 3 well-differentiated neuroendocrine tumours divide fast enough to be called grade 3 yet still look and behave like their slower relatives rather than like neuroendocrine carcinoma. Recognised as separate since 2017, they keep the somatostatin receptor, respond less well to platinum chemotherapy, and in the NETTER-2 trial were among the first treated with lutetium-177 dotatate up front.

Hereditary pheochromocytoma and paraganglioma is the inherited form of these adrenaline-producing tumours, caused by a fault in one of more than a dozen genes, most often SDHB, SDHD, VHL and RET. Knowing the gene changes care: SDHB carriers have the highest risk of spread, VHL and MEN2 patients get adrenal-sparing surgery because tumours arise on both sides, and relatives are screened.

Leads the next wave of endocrine therapy: oral SERDs like imlunestrant and the PI3K inhibitor inavolisib.

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.

Lung neuroendocrine tumours, called typical and atypical carcinoids, are slow-growing tumours of the airways that are usually cured by surgery. When they spread, everolimus is the one drug tested in a randomised trial for this site, cabozantinib was approved in 2025, and somatostatin analogues and lutetium radioligand therapy are borrowed from gut tumours.

Metastatic pheochromocytoma and paraganglioma is disease that has spread to bone, lymph nodes, liver or lungs, the only way these adrenaline-producing tumours are called malignant. It is often slow, so treatment starts with blood pressure control and watching, then moves through radioactive drugs that home to the tumour, the kinase inhibitor sunitinib, chemotherapy and, since 2025, belzutifan.

The MEN syndromes are inherited faults in a single gene that cause tumours in several hormone glands over a lifetime. Because the gene can be found in childhood, at-risk relatives can be tested, watched and in MEN2 have the thyroid removed before cancer develops; and for MEN2 thyroid cancer that does spread there is now a precise pill, selpercatinib, that blocks the faulty RET protein.

A family of usually slow-growing tumours that start in hormone-producing cells of the gut, pancreas and lungs. They pioneered the idea of using the same molecule to see a tumour on a scan and then to treat it with radiation.

Pancreatic neuroendocrine tumours arise from the hormone-producing islet cells of the pancreas and behave very differently from ordinary pancreatic cancer, often growing for years. Surgery cures localised tumours; advanced disease is treated in sequence with somatostatin analogues, lutetium-177 dotatate, targeted tablets and oral chemotherapy, and a minority secrete insulin or gastrin.

Parathyroid carcinoma is a cancer of the glands that control blood calcium, seen in a few people per ten million a year; most of its harm comes from dangerously high calcium rather than spread. Removing the tumour intact at the first operation gives the best chance of cure, and cinacalcet and bone-protecting drugs control calcium when it cannot be removed.

Pheochromocytomas and paragangliomas are tumours of adrenaline-producing tissue that cause dangerous blood pressure surges. Surgery after careful blood-pressure blockade cures most, genetic testing finds an inherited cause in nearly half, and for the minority that spread there are now radioactive drugs that home to the tumour and, since 2025, the first oral targeted pill, belzutifan.

Pituitary tumours are usually benign growths of the hormone gland at the base of the brain that cause trouble by overproducing hormones or pressing on the optic nerves. Prolactin-producing tumours melt away with a tablet, most others are cured by surgery through the nose, and the rare aggressive ones respond to the chemotherapy drug temozolomide.

Small intestinal neuroendocrine tumours are slow-growing hormone-producing tumours of the ileum and jejunum, often found only after they have spread to lymph nodes and the liver. Monthly somatostatin analogue injections control symptoms and growth, lutetium-177 dotatate is the main second treatment, and everolimus, cabozantinib and surgery fill in.

Thyroid cancer is usually curable with surgery and radioactive iodine, the original theranostic. Rare aggressive forms respond to RET and BRAF inhibitors.