Endocrine Abstracts

Introduction: Ectopic Cushing’s syndrome (ECS) is a rare disease caused by ACTH secretion by extrapituitary neuroendocrine tumors, characterized by high mortality. Despite the clinical sings of Cushing’s syndrome, difficult access to reliable clinical examinations, leads to a delay in diagnosis and the choice of optimal treatment tactics. Small-cell lung cancer patients with ECS have a very poor prognosis.

Case report: A 35-year-old woman was hospitalized in the endocrinology department with complaints of dry mouth, thirst, hypertension (up to 170/100 mmHg). The patient also had signs of heart failure due to concomitant congenital heart disease, diabetes mellitus type 2 (T2DM) and obesity. During the year, she was repeatedly examined due to the presence of clinical manifestations of Cushing’s syndrome. Physical examination: centrapetal obesity with fat deposits around the face, neck and upper back, BMI=41.8 kg/m², hyperpigmentation in the neck, axillary and inguinal region, reddish-blue stretch marks on the arms, abdomen, buttocks. Laboratory evaluation revealed high levels of ACTH in the blood – 229,3 pg/ml (reference 7,2-63,3), and cortisol in daily urine 3600.8 μg/24 h (normal 58 - 403), and a decrease in potassium levels to 1.1 mmol/l, fasting glucose - 12 - 21 mmol/l. CT scan of the thoracic segment revealed a dense focus of the X segment of the right lung, metastatic character is not excluded. Abdominal CT revealed hyperplasia of both adrenal glands, multiple liver formations. PET-CT (18F-fluorodeoxyglucose): metabolically inactive formation of the lower lobe of the right lung, multiple metabolically inactive hypodense liver formations, moderately pronounced splenomegaly. The tumor marker Pro Gastrin Releasing Peptide (ProGRP) in blood was significantly increased - up to 5000 pg/ml (reference 0-46), which can increase in small cell lung cancer. Somatostatin analogues and steroidogenesis blockers (Ketoconazole) were used to treat the disease, but cortisol levels did not decrease. The primary tumor during life could not be identified, death occurred due to lung and cardiovascular failure. The histological examination at post-mortem autopsy revealed the lung neuroendocrine tumor with signs of malignancy and multiple metastatic lesions of the liver.

Conclusion: Despite the availability of modern imaging diagnostic methods, the search for the primary focus and the treatment of ACTH-ectopic syndrome present considerable difficulties and often require long-term follow-up and an interdisciplinary approach. Although the prognosis for this type of tumor is unfavorable, timely diagnosis using lung tissue tumor markers and PET could help clarify the diagnosis at an earlier stage of the process.