Endocrine Abstracts

Adrenocortical carcinoma (ACC) is a rare (incidence 1–2 per 1 million population) and heterogeneous malignancy with incompletely understood pathogenesis, unknown etiology and poor prognosis. ACC tends to grow and metastasize rapidly if untreated. The majority of cases are incidental diagnoses of advanced stage disease that metastasizes precociously due to its tendency to invade vascular structures. Among adults presenting with hormonal syndromes due to tumor excess secretion, Cushing’s syndrome alone is most common, followed by mixed Cushing’s and virilization (glucocorticoid and androgen overproduction). Conn syndrome (mineralocorticoid excess) occur in <10% of cases. Our patients shows all symptoms of Cushing and Conn syndrome.

Case report: Patient is a 37-year-old woman with oncologic genetic load. Patient presented with spontaneous fracture of the right femur and transferred to our clinic because of suspicion of ACC. Patient has one year progression of high blood pressure controlled with angiotensin-converting enzyme inhibitor. She complained also of diffuse postprandial abdominal pain with peaks of intensity accompanied with vomiting, aside from high blood pressure manifestations of muscle cramps, muscle weakness and headaches. Our patients is also presented with signs and symptoms of hormonal syndromes (Cushing syndrome, virilism and Conn syndrome).

Laboratory work-up including full blood count, blood chemistry, serum electrolytes, liver function tests, cortisol, aldosterone, normetanephrines, total metanephrines, and urine metanephrines. Ultrasound imaging revealed 90×83×88 mm left heterogeneous adrenal mass that was confirmed with computer tomography and magnetic resonance. Histopathological result of adrenal mass reported adrenal carcinoma\. Local invasion and tumor extension into the inferior vena cava as well as lymph node and left kidney or other metastases (lung and liver, peritoneum, colon, femur, mandible) are found. Palliative cisplatin-based chemotherapy and radiotherapy are alternative to surgical debulking. Ketoconazole is used to control hypercortisolemic symptoms and the therapy with i.v. bisphosphonate (Pamidronat 60 mg) is also given to prevent further pathological fractures.