Agha Ayman, Hornung Matthias, Iesalnieks Igors, Schreyer Andreas, Jung Ernst Michael, Haneya Assad, Schlitt Hans J
Department of Surgery, University Hospital Regensburg, Franz-Josef-Strauss-Allee 11, 93053, Regensburg, Germany.
Langenbecks Arch Surg. 2014 Jan;399(1):93-8. doi: 10.1007/s00423-013-1121-2. Epub 2013 Sep 19.
Primary aldosteronism (PA, also Conn syndrome) is a benign disease in majority of cases. However, malignant transformation has been described. Present study reports on three cases of aldosterone producing adrenocortical carcinoma (APAC) in comparison to patients with benign PA.
Data of patients undergoing adrenalectomy for benign PA were compared to patients with APAC. Retrospective chart analysis was performed. All patients received spironolactone for 6-8 weeks preoperatively.
Seventy-four patients underwent adrenalectomy for PA between 1994 and 2011. Three of them revealed an APAC. Patients with APAC presented with a significantly lower serum potassium level (1.7 mmol/l vs. 3.4 mmol/l, p = 0.001) and significant larger tumors (5.2 vs. 1.8 cm, p = 0.002). In addition, aldosterone/renin (A/R) ratio 675 in patients with APAC as compared to 74 in patients with benign PA (p = 0.0001). Sixty-eight of 71 patients with benign PA underwent minimal invasive surgery, whereas all three patients with APAC were operated conventionally. All patients with APAC developed disease recurrence 6-18 months postoperatively.
Tumor size >4 cm and a very high A/R ratio seems to predictors of malignancy in patients with PA. If these criteria are present, open adrenalectomy should be performed instead of endoscopic procedure.
原发性醛固酮增多症(PA,亦称康恩综合征)在大多数情况下是一种良性疾病。然而,已有恶性转化的报道。本研究报告了3例醛固酮分泌性肾上腺皮质癌(APAC)病例,并与良性PA患者进行了比较。
将因良性PA接受肾上腺切除术的患者数据与APAC患者的数据进行比较。进行了回顾性病历分析。所有患者术前均接受螺内酯治疗6 - 8周。
1994年至2011年间,74例患者因PA接受了肾上腺切除术。其中3例为APAC。APAC患者的血清钾水平显著较低(1.7 mmol/L对3.4 mmol/L,p = 0.001),肿瘤显著更大(5.2对1.8 cm,p = 0.002)。此外,APAC患者的醛固酮/肾素(A/R)比值为675,而良性PA患者为74(p = 0.0001)。71例良性PA患者中有68例行微创手术,而3例APAC患者均行传统手术。所有APAC患者术后6 - 18个月疾病复发。
肿瘤大小>4 cm和极高的A/R比值似乎是PA患者恶性肿瘤的预测指标。如果存在这些标准,应行开放性肾上腺切除术而非内镜手术。