Endocrine Abstracts

Extraadrenal perirenal myelolipom has been reported since 1980. In recent years, it has been reported at an increasing rate in different sites of the body ranging from the nasal cavity, mediastinum to presacral, paravesical region. However, it was the first reported case that perirenal multiple myelolipomas, each myelolipomas was about 5 cm in diameter, in associated with cortisol hypersecretion. A patient was an emigrant. She was followed in her country and was prescribed ketokonazol. She did not take her medicine and she was brought to emergency service when her situation deteriorated. She was exposed to hypercortisolemia for a long time and had severe muscle weakness, fatigue, amenorrhea and weight loss at admitted-time. Physical examination revealed cushingoid appearance with trunk obesity, sebaceous moon face, hirsutism, muscle wasting, high blood pressure, acanthosis nigricans and abdominal striae. Laboratory tests were as following; glucose: 170 mg/dl (70–100), urea:42.3 mg/dl (17–43), creatinin: 0.82 mg/dl (0.51–0.95), sedimentation: 35 mm/h (0–30), HbA1c: 8.8%, FSH: 1.59 mIU/ml (4.54–22.51) LH: 0.31 mIU/ml (12–12.86 mIU/ml), estradiol: 16 pg/ml (49–291), progesterone: 1.75 ng/ml (5.16–18.56), total testesterone: 0.48 pg/ml (0.1–0.75), cortisol: 30.40 μg/dl (6.7–22.6), ACTH: <1 pg/ml (7.2–63.3). Eosinopenia, lymphocytopenia and neutrophilia was obtained in whole blood cell evaluation. Cortisol level was 27.60 μg/dl after 1 mg dexamethasone suppression test, result revealed failure of suppression. Pituitary MR was normal. A contrast enhanced abdominal MR showed multipl lobulated bilateral perirenal masses and a cystic mass at the caput of the pancreas without solid component. Majority of perirenal masses was located in the right perirenal area with 5×4 cm in diameter. The MR followed by a F-18 fluorodeoxyglucose tomography (FDG PET/CT). The increased FDG uptake in the right pararenal area. Myelolipoma was diagnosed in microfiber biopsy of the perirenal mass. In her hospitalisation period femur fracture occurred. After operation for femur fracture she could not be extubed because of the respiratory muscle weakness. Adrenal myelolipomas with Cushing syndrome have been reported previously. However, extra-adrenal perirenal myelolipomas presented with Cushing syndrome is the first case of the literature.