A 44-year-old woman was admitted for diabetic ketoacidosis and severe hypertension. She had aggressive clinical features related to Cushings syndrome (CS). ACTH-independent CS was diagnosed based on undetectable ACTH (0.65 pmol/L) and unsuppressed cortisol levels by dexamethasone (6363 nmol/L). Adrenal contrast-enhanced computed tomography (CT) scans showed a 15x12x20 cm heterogeneous mass of the left adrenal that pushed back the spleen and the left kidney with thrombosis of the left ovarian and renal veins. The unenhanced density of the mass was >10 Hounsfield Units with a <50% absolute percentage washout. Multiple liver nodules (from 35 to 80 mm) were identified with a hyper vascularized pancreatic nodule compatible with a neuroendocrine tumor (NET). A well differentiated Grade 1 NET was diagnosed after liver biopsy. Laboratory testing found increased levels of pancreatic polypeptide >2000 pmol/L. Somatostatin receptor scintigraphy showed positive findings only in one liver metastasis. (18)F-FDG PET/CT showed an increased FDG uptake by the left adrenal mass (SUV max: 12.3) and the liver metastases (SUV max from 4 to 11.4) and also by thoracic and lumbar vertebras. As she had hypercalcemia, we looked for a primary hyperparathyroidism that was biologically and morphologically confirmed. Because of the occurrence of tumors involving several endocrine glands, we suspected a Multiple endocrine neoplasia type 1 (MEN1). A pituitary prolactin adenoma was also found. Genetic diagnosis allowed us to identify a germline MEN1 mutation. Given the liver discrepancy of morphologic and functional imaging about the origin of the liver metastases, we performed a liver MRI which characteristics confirmed a double origin of liver metastases: hyperintense lesions on T2-weighted and diffusion-weighted imaging with a strong hypervascularization were rather for the diagnosis of neuroendocrine liver metastases, while hyperintense lesions on T2-weighted associated with a heterogeneous enhancement were rather for the diagnosis of metastases of adrenal carcinoma. Adrenal steroidogenesis blockade was started using ketoconazole and mitotane, then metyrapone. Chemotherapy with carboplatin and etoposide was performed because of disease progression. After 2 months, the size of some liver metastases was decreased by 10%. Unfortunately, this woman returned to her country and she has not been seen since her departure. In conclusion, this case allowed us to diagnose a MEN1 from an association of liver metastases with a double primary tumors origin: adrenal carcinoma and pancreatic neuroendocrine tumors. Morphological (CT, MRI) and functional (PET-CT) imagings were complementary to help us to better manage this patient.
18 - 21 May 2019
European Society of Endocrinology