ACTH-independent Cushing syndrome related to retroperitoneal liposarcoma: A case report

Liposarcoma is a rare malignant mesenchymal tumor, preferentially located in the retroperitoneum (15% of cases). It must be suspected in the presence of a fatty tumor, which is not related to an intra-abdominal solid organ. It is a non-secreting tumor. Only two cases of adrenal liposarcoma secreting glucocorticoids have been reported in the literature [1] . This is a 45-year-old female patient, with no notable personal history, referred for exploration of hypertension with hypokalemia. On examination she had a clinical Cushing's syndrome (CS). Baseline urinary free cortisol was1.5 times normal range and a lack of cortisol suppression during dexamethasone supression tests. ACTH independent CS was suspected on a low ACTH level at 10 pg/mL. An abdominal and pelvic MRI revealed a retroperitoneal mass measuring11 cm, independent of the adrenal gland and pushing the left kidney upwards. The patient underwent surgical resection of the tumor with the left adrenal gland and kidney. Anatomopathological study concluded on a sclerosing variant of well-differentiated liposarcoma with a hypotrophic kidney and an intact adrenal gland. The evolution was favorable with clinicobiological remission. Chemotherapy was not indicated as adjuvant treatment. Due to its rarity and the retroperitoneal location, retroperitoneal liposarcoma may pose a differential diagnosis with adrenocorticaloma and indicate an unnecessary adrenalectomy, hence the importance of a meticulous interpretation of radiological images [2] .