Dai Jun, He Hong-Chao, Huang Xin, Sun Fu-Kang, Zhu Yu, Xu Dan-Feng
Department of Urology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai 200025, China.
World J Clin Cases. 2019 Feb 6;7(3):340-346. doi: 10.12998/wjcc.v7.i3.340.
Adrenal primitive neuroectodermal tumor (PNET) is an extremely rare malignant tumor with poor prognosis and of neural crest origin. Herein, we report a case of adrenal PNET and summarized its clinical and pathological characteristics on the basis of 16 patients reported recently.
A female patient aged 25 years presented with right lumbago for 12 mo, and pre-operative computed tomography showed a huge right adrenal mass. She received tumorectomy, and post-operative pathological examination showed adrenal PNET. After surgery, she underwent adjuvant chemotherapy and was followed up 31 mo after surgery. She received brachytherapy for right paracolic and hepatic metastases. She was alive and followed up for 60 mo. In available studies, only 57.14% (4/7) and 44.44% (4/9) were positive for the expression of neuron-specific enolase and synaptophysin, respectively, although CD99 expression was found in all the patients (100%; 10/10).
It is concluded that adrenal PNET is very rare and highly malignant, and histology is a golden standard in its diagnosis. Surgery and adjuvant therapy is the main treatment.
肾上腺原始神经外胚层肿瘤（PNET）是一种极为罕见的恶性肿瘤，预后较差，起源于神经嵴。在此，我们报告一例肾上腺PNET病例，并在最近报道的16例患者的基础上总结其临床和病理特征。
一名25岁女性患者因右腰痛12个月就诊，术前计算机断层扫描显示右侧肾上腺有巨大肿块。她接受了肿瘤切除术，术后病理检查显示为肾上腺PNET。术后，她接受了辅助化疗，并在术后31个月进行随访。她因右侧结肠旁和肝脏转移接受了近距离放射治疗。她存活并随访了60个月。在现有研究中，尽管所有患者（100%；10/10）均发现CD99表达，但分别只有57.14%（4/7）和44.44%（4/9）的神经元特异性烯醇化酶和突触素表达呈阳性。
得出结论，肾上腺PNET非常罕见且恶性程度高，组织学是其诊断的金标准。手术和辅助治疗是主要治疗方法。
