Endocrine Abstracts

Two female patients, A – 61-year-old, B – 68-year-old, were admitted to hospital with symptoms of hypercortisolemia. Both of them were diagnosed with neuroendocrine tumor (NET), patient A with pancreatic NET G3 (Ki67 – 30%), and patient B with a disease with a probable primary origin in lungs - NET G2 (Ki67 - 15%). Both women had metastases to the liver. While laboratory results were similar in both cases (high level of cortisol – up to 1750 nmol/l and 1459 nmol/l (normal value: <497 nmol/l), ACTH – up to 184.40 pg/ml and 214.90 pg/ml (normal value: <63.30 pg/ml), hypokalemia, hyperglycemia, high BP), the clinical manifestation of the disease was quite different. Examination of patient A revealed signs of hirsutism, muscular atrophy, edema of the lower limbs, easy bruising. Patient B presented psychotic symptoms, such as delusions, aggressive and inadequate behavior, confusion. MRI scan of the pituitary gland in both cases revealed no tumor. In both patients, their liver biopsy samples from metastatic foci were tested for the ACTH expression and were positive. Patient A underwent left adrenalectomy, while resection of the right adrenal gland was impossible because of the tumor infiltration. The pathomorphological examination of the removed tissues revealed lipid-poor adenoma. Patient B did not agree for surgery. In both cases, metyrapone was administered as a treatment for Cushing syndrome. Due to the limited access to the drug and its temporary withdrawal, in both patients, cortisol level fluctuations accompanied by a transient aggravation of the general condition were observed. Patient A was treated with octreotide, as well as capecitabine and temozolomide (CAPTEM) and patient B was qualified for treatment with everolimus due to lack of somatostatin receptor expression in HYNIC-TOC. Patient B died 6 months after the diagnosis, while patient A achieved partial remission of the disease and her general condition improved. Ectopic adrenocorticotropic hormone (ACTH) secretion (EAS) may be a form of ACTH-dependent Cushing syndrome in patients diagnosed with neuroendocrine tumors. The treatment of underlying disease together with the treatment of hypercortisolemia should be considered and may constitute a challenge. Metopirone might be a useful drug to reduce concentration of cortisol. However, adrenalectomy may remain basis for the treatment of EAS affecting patient’s quality of life and prognosis. The assessment of ACTH expression in the neuroendocrine tumor metastases may guide the management of patients diagnosed with Cushing syndrome due to EAS.