Peppa Melpomeni, Pikounis Vasilios, Papaxoinis Georgios, Macheras Anastasios, Economopoulos Theofanis, Raptis Sotirios A, Hadjidakis Dimitrios
Endocrine Unit, Second Department of Internal Medicine-Propaedeutic, Research Institute and Diabetes Center, Athens University Medical School, 'Attikon' University Hospital, 1 Rimini Str, Athens, 12462, Greece.
Cases J. 2009 Sep 10;2:8951. doi: 10.1186/1757-1626-0002-0000008951.
Adrenocortical carcinoma is a rare malignancy with a poor prognosis and presents with mass effects and less often with signs of hormone excess (approximately 60% of all adrenocortical carcinoma's). Hormonally active adrenocortical carcinomas most commonly secrete cortisol while the co-secretion of multiple steroid hormones is rare.
We report the case of a 59 year-old woman with a medical history of hyperaldosteronism due to a right adrenal adenoma. During follow up, she showed symptoms of hypercortisolism and hyperandrogenemia and a rapid growth of the adrenal mass. She underwent right adrenalectomy and the histology revealed the presence of an adrenocortical carcinoma. Six months post-operatively being on treatment with mitotane, she was diagnosed of metastatic disease to the liver. She underwent right hepatectomy and was started on systemic chemotherapy, with no signs of tumour recurrence during the following six months.
The hormonal status should be carefully investigated in all cases of suspected adrenocortical carcinoma, as the pattern of hormone secretion may be a clue to the malignancy of an adrenal lesion. In addition, more data are needed to clarify the clinical and prognostic significance of the combined production of all adrenocortical hormones affecting either the survival or the quality of life of adrenocortical carcinoma patients.
肾上腺皮质癌是一种罕见的恶性肿瘤，预后较差，表现为占位效应，较少出现激素过多的体征（约占所有肾上腺皮质癌的60%）。具有激素活性的肾上腺皮质癌最常分泌皮质醇，而多种甾体激素共同分泌的情况罕见。
我们报告一例59岁女性，有因右肾上腺腺瘤导致醛固酮增多症的病史。在随访期间，她出现了皮质醇增多症和高雄激素血症的症状，且肾上腺肿块迅速增大。她接受了右肾上腺切除术，组织学检查显示为肾上腺皮质癌。术后6个月，她接受米托坦治疗，被诊断为肝转移。她接受了右肝切除术，并开始全身化疗，在接下来的6个月中没有肿瘤复发的迹象。
在所有疑似肾上腺皮质癌的病例中，都应仔细调查激素状态，因为激素分泌模式可能是肾上腺病变恶性程度的线索。此外，需要更多数据来阐明所有肾上腺皮质激素联合产生对肾上腺皮质癌患者生存或生活质量的临床和预后意义。
