潘柏勳¹、林子平^1,2、張延驊^1,2、黃志賢^1,2

¹台北榮民總醫院 泌尿部；²國立陽明大學醫學院 泌尿學科 書田泌尿科學研究中心

Po-Hsun Pan¹, Tzu-Ping Lin^1,2, Yen-Hwa Chang^1,2, William J. Huang^1,2

¹Department of Urology, Taipei Veterans General Hospital, Taipei, Taiwan

² Department of Urology, School of Medicine and Shu-Tien Urological Institute, National Yang-Ming University, Taipei, Taiwan

 

Adrenocortical carcinoma(ACC) is an uncommon malignant disease derived from adrenal gland. It is usually aggressive and may be functional that caused by hormone-secreting. Here we going to present a case of functional adrenocortical carcinoma and the following treatment.

A 46-year-old female has past history of hypertension and diabetes mellitus under oral hypoglycemic agents treatment. She found that she has face swelling at first. So she went to Metabolism and Endocrinology outpatient department for survey where Cushing syndrome was suspected. After image survey, a left adrenal tumor was disclosed. So she was referred to urology department for further survey and treatment.

Lab data for endocrine function was checked which showed elevated urine free cortisol level– 152.6 ug/day (normal range 20-80 ug/day), Dehydroepiandrosterone sulfate (DHEA-S) >40.7 umol/L (normal range 0.2-16.1 umol/L), and low adrenocorticotropic hormone (ACTH) level <5.0 pg/ml (normal range <46.0 pg/ml). Renin, aldosterone and catecholamine level were in normal range. Abdominal CT was arranged which revealed a aypervascular mass with necrosis in left suprarenal space about 10x10x12.5cm in size, tumor thrombi in left renal vein, infrahepatic, intrahepatic and suprahepatic inferior vena cava. Besides, 0.9cm enhancing nodule at S4b of liver and multiple nodules in bilateral lower lungs were also seen, suspected metastasis. Due to IVC thrombus, transesophageal echocardiography was preformed and a long linear mass lesion originated from IVC and protruded into right atrium was seen, compatible with tumor invasion. Then, after we consulted general surgeon and cardiac surgeon, she received radical left nephrectomy, adrenalectomy, IVC thrombectomy and right atrium cardiotomy. Pathology showed left adrenal adrenocortical carcinoma with IVC and right atrium tumor thrombi and liver metastasis, pT4NxM1.

After operation, cortisone treatment was prescribed under endocrinologist suggestion and gradually tapered down. Drainage tube was also removed due to reduce drainage amount. Mitotane was also used for tumor treatment. She was discharged smoothly on post-operative day 20. Follow up abdominal CT showed no locally recurrent but progression lung metastasis and newly liver metastasis at lateral segment were found.

This is a rare case of adrenocortical carcinoma with multiple metastases. Surgery was performed combined with multidisciplinary specialist. We will keep follow up the prognosis of the patient.