Nair Lekha Madhavan, Jagathnath Krishna K M, Kumar Aswin, Mathews Susan, Joseph John, James Francis Vadakkumparambil
Regional Cancer Centre, Genitourinary Clinic, Thiruvananthapuram, Kerala, India.
Department of Cancer Epidemiology and Biostatistics, Regional Cancer Centre, Thiruvananthapuram, Kerala, India.
Indian J Urol. 2019 Jul-Sep;35(3):213-217. doi: 10.4103/iju.IJU_19_19.
Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with aggressive behavior. Most of our knowledge about this rare tumor is based on retrospective case series. This study aimed at analyzing the clinicopathological features and outcomes of patients treated at a tertiary cancer center in India.
We retrospectively reviewed the data of patients with ACC registered from January 2006 to December 2015.
Thirty-seven patients were included in the study, 20 males and 17 females. Median age was 49 (18-78) years. Hormonal overproduction was noticed in 27% of patients. Median tumor size was 10 cm (2-22). Seventeen patients had metastatic disease and 20 patients were localised at diagnosis. Median follow-up was 22 months and median overall survival (OS) was 23.46 months. OS at 2 years and 5 years was 46.1% and 21%, respectively. The median disease-free survival (DFS) was 20 months. DFS at 2 years and 5 years was 45% and 24%, respectively. Age, sex, tumor size, hormonal overproduction, tumor laterality, and stage of the disease did not influence survival. However, advanced stage was associated with higher risk for recurrence. ( = 0.03).
ACC is a rare endocrine malignancy with very poor survival rates. Rate of recurrence is high even after complete surgery. Systemic treatment options are limited. Newer agents are needed to improve outcome.
肾上腺皮质癌（ACC）是一种具有侵袭性的罕见内分泌恶性肿瘤。我们对这种罕见肿瘤的了解大多基于回顾性病例系列研究。本研究旨在分析印度一家三级癌症中心治疗的患者的临床病理特征及预后情况。
我们回顾性分析了2006年1月至2015年12月登记的ACC患者的数据。
37例患者纳入研究，男性20例，女性17例。中位年龄为49岁（18 - 78岁）。27%的患者存在激素过度分泌。肿瘤中位大小为10厘米（2 - 22厘米）。17例患者有转移性疾病，20例患者诊断时为局限性病变。中位随访时间为22个月，中位总生存期（OS）为23.46个月。2年和5年的OS分别为46.1%和21%。无病生存期（DFS）的中位时间为20个月。2年和5年的DFS分别为45%和24%。年龄、性别、肿瘤大小、激素过度分泌、肿瘤部位及疾病分期均不影响生存。然而，晚期与更高的复发风险相关（P = 0.03）。
ACC是一种罕见的内分泌恶性肿瘤，生存率极低。即使完全手术切除后复发率仍很高。全身治疗选择有限。需要新型药物来改善预后。
