背景:膀胱原始神经外胚层肿瘤(PNET)是一种高度侵袭性的肿瘤,在不完全切除的情况下具有高的局部复发率和远处转移率。我们报道了一个年轻的女性病人,其中早期腹腔镜根治性膀胱切除术联合标准淋巴结清扫术和改良长春新碱,盐酸多柔比星,环磷酰胺(VAC)化疗方案存在争议。因为膀胱的PNET是一种罕见的恶性肿瘤,标准治疗方案尚未建立.目前尚不清楚手术联合术后化疗对PNET患者的长期生存率是否优于单独手术。
患者是一名45岁的中国女性,主诉下尿路症状,包括紧迫性,频率,排尿困难,两个月。
■PNET。
方法:患者行腹腔镜根治性膀胱切除术和标准淋巴结清扫术,联合改良VAC化疗方案。
结果:在2018年接受根治性手术后,患者完成了6个疗程的辅助化疗。在手术后3、6、9和12个月进行腹部和胸部计算机断层扫描。诊断后2年,患者仍然活着,没有疾病复发的迹象。
结论:根治性手术和标准淋巴结清扫联合辅助化疗可能是改善膀胱PNET预后的关键。
BACKGROUND: Primitive neuroectodermal tumor (PNET) of the urinary bladder is a highly aggressive tumor with high local recurrence and distant metastasis rates in cases of incomplete excision. We report a
case of a young female patient, in whom early laparoscopic radical cystectomy combined with standard lymph node dissection and a modified vincristine, doxorubicin hydrochloride, and cyclophosphamide (VAC) chemotherapy regimen was controversial. Because PNET of the urinary bladder is a rare malignancy, the standard treatment regimen has not yet been established. It is not clear whether surgery combined with postoperative chemotherapy for PNET patients may be superior to surgery alone on long term survival.
UNASSIGNED: The patient was a 45-year-old Chinese woman who complained of lower urinary tract symptoms, including urgency, frequency, and difficulty in urination, for 2 months.
UNASSIGNED: PNET.
METHODS: The patient underwent laparoscopic radical cystectomy and standard lymph node dissection, combined with modified VAC chemotherapy regimens.
RESULTS: After undergoing radical surgery in 2018, the patient completed 6 courses of adjuvant chemotherapy. Abdominal and thorax computed tomography scanning was performed 3, 6, 9, and 12 months after the surgery was completely free of tumor. The patient is still alive with no signs of recurrent disease 2 years after diagnosis.
CONCLUSIONS: Radical surgery and standard lymphadenectomy combined with adjuvant chemotherapy may be essential to improve the prognosis of PNET of the urinary bladder.