目的:本研究目的是明确泌尿生殖系统横纹肌肉瘤(rhabdomyosarcoma,RMS)患者的临床病理特征,同时制作预测泌尿生殖系统RMS患者的1、3、5年生存率的列线图.方法:对1975-2016年SEER数据库确诊的泌尿及生殖系统RMS患者进行筛选,最终有990例患者纳入本研究.采用单因素及多因素Cox回归分析筛选泌尿及生殖系统RMS的独立危险因素,并以此来构建预测泌尿及生殖系统RMS生存率的列线图.选取2012-2018年郑州大学第一附属医院确诊的26例泌尿生殖系统RMS患者作为外部验证队列,然后采用C指数和校准曲线对模型进行内部及外部验证.结果:在泌尿及生殖系统RMS患者中,多因素Cox回归分析结果显示,患者的年龄、肿瘤部位、病理类型、肿瘤大小、总分期、N分类、M分类、手术、化疗均具有独立预测价值(P＜0.05).根据上述变量构建预测模型,此列线图内部及外部验证的C指数分别为0.841、0.838,具有良好的区分度,同时内部及外部数据的校准曲线均显示出此预测模型具有较好的一致性.结论:本研究所构建的列线图可为泌尿及生殖系统RMS患者提供更为简洁的预后评估,为临床的个体化诊疗提供参考.

Objective:To investigate the factors related to recurrence and prognosis of retroperitoneal liposarcoma.Method:The clinical data of patients with primary retroperitoneal liposarcoma who underwent surgical treatment in the First Affiliated Hospital of Zhengzhou University from June 2011 to January 2020 were analyzed retrospectively. There were 42 males and 47 females and patients’median age was 53 (26-78). Sixty-five cases were treated by operation in our hospital, and 24 cases were primarily treated by the operation in another hospital. The clinical manifestations of the initial diagnosis included retroperitoneal mass in 41 cases, abdominal distension in 12 cases, abdominal pain in 10 cases, fever in 11 cases, nausea, vomiting and poor appetite in 8 cases, frequent urination and dysuria in 6 cases, and bilateral lower limb edema in 1 case. Preoperative CT imaging showed that the tumor body was located in the retroperitoneal kidney area in 58 cases, while in the retroperitoneal space or the pelvic extraperitoneal space in 31 cases. There were 55 single cases and 34 multiple cases. The median tumor length was 20(3-52) cm. Among the primarily treated 65 patients, 47(72.3%) were considered as primary retroperitoneal liposarcoma by preoperative imaging examination. Among the 89 patients treated by surgery, 78 underwent endoscopic surgery, among which 21 underwent laparoscopic surgery, 38 cases of retroperitoneal laparoscopic surgery, 19 cases of Da Vinci robot-assisted laparoscopic surgery. Open operation was performed in 11 cases. There were 87 patients undergoing radical resection and 2 patients undergoing palliative resection. Forty-two patients underwent intraoperative combined resection of the adjacent organs. The recurrence and survival status of patients were followed up.Results:All the 89 patients underwent the operation successfully, with the median operative blood loss of 200 (10-2000) ml. There were 23 cases being diagnosed of well differentiated liposarcoma, 40 cases of dedifferentiated, 20 cases of myxoid/round, 5 cases of myxoid liposarcoma, and 1 cases of mixed type. Pathologically, there 42 cases with low grade histology and 47 cases with high grade histology. In this study, 89 patients were followed up for 3 to 108 months, and the median follow-up time was 28 months. The 5-year recurrence free survival rate, disease-free survival rate and overall survival rate of the patients were 16.7%, 16.1% and 52.6%, respectively. There were 57 patients presenting local recurrence, 1 patient of lung metastasis, and 1 patient of liver metastasis, and the median disease-free survival time was 24 months. There were 42 patients died of the disease, with a median survival time of 64 months. Univariate analysis showed that intraoperative blood loss( P<0.01), whether multiple cases( P<0.01), pathologic types( P<0.01), and histological grades ( P<0.01) were related to disease-free survival.The intraoperative blood loss( P<0.01), multiple cases( P<0.05), pathologic types ( P<0.05), and recurrence ( P<0.01)were related with overall survival. Gender, age, tumor size, tumor location, whether primary surgery, radical resection or combined resection of adjacent organ had no effect on the prognosis of patients ( P>0.05). Cox regression model multivariate analysis revealed that surgical bleeding ( RR=2.360, 95% CI 1.313-4.241, P=0.004), multiple tumor ( RR=1.899, 95% CI 1.068-3.375, P=0.029), and pathological type ( RR=4.976, 95% CI 1.622-15.264, P=0.005) were independent factors affecting disease-free survival. The recurrence was an independent factor affecting the overall survival of patients ( RR=31.495, 95% CI 1.062-933.684, P=0.046). Conclusions:Retroperitoneal liposarcoma is a rare disease with high recurrence rate. The intraoperative blood loss, whether multiplicity and pathological type are independent factors affecting the disease-free survival, and recurrence is independent factors affecting the overall survival.

目的:探讨膀胱肉瘤样癌的临床表现、诊断、治疗及预后.方法:回顾性分析2010年6月-2018年10月我院收治的26例病理确诊为膀胱肉瘤样癌患者的临床资料,其中男18例,女8例.24例行手术治疗,其中7例行经尿道膀胱肿瘤电切术(transurethral resection for bladder tumor,TURBT),7例行膀胱部分切除术,10例行根治性膀胱切除术;余2例未行手术治疗.采用Kaplan-Meier法进行生存分析.结果:术后病理TNM分期:T1N0M0期7例,T2N0M0期7例,T3N0M0期8例,T3N2M0期2例,T4NxM0期1例,T4NxM1期1例.根据浸润深度分类:7例为非肌层浸润肿瘤,19例为肌层浸润肿瘤.24例获得随访,2例失访,随访时间2～80个月,中位随访时间为14个月,死亡病例19例,中位随访时间13个月.生存分析显示不同浸润深度及手术方式预后之间差异有统计学意义.结论:膀胱肉瘤样癌是一种罕见的高度恶性肿瘤,预后相对较差.非肌层浸润肿瘤行TURBT术可取得相对较好的预后.肿瘤分期是影响预后最重要的因素,因此早发现、早治疗是改善其预后的重要条件.

Objective To improve the understanding of primary mucoepidermoid carcinoma of lung.Methods Based on the clinical data of one patient with primary mucoepidermoid carcinoma,we collected the clinical data of 199 cases from 13 articles in China and reviewed the related domestic and foreign literatures.The etiology,clinical manifestations,radiological features,pathological characteristics,diagnosis,differential diagnosis,treatment and prognosis were analyzed and summarized.Results The etiology of primary mucoepidermoid carcinoma of lung was not clear,and the clinical symptoms of the disease included cough,expectoration,chest tightness and chest pain.However,these symptoms were unspecific.It was shown as atelectasis or pneumonia in chest X-ray.It was demonstrated as smooth and lobulated mass or polypoid mass with distal bronchial mucoid impaction and distal atelectasis.It was characterized by pathology as varying proportions of mucus-secreting,squamous (epidermoid) and intermediate cells,without keratinization.The primary choice of treatment for the disease was surgery,and the effects of radiotherapy and chemotherapy were not confirmed yet.The prognosis was better than the ordinary non-small cell lung cancer.Conclusions Primary mucoepidermoid carcinoma of lung is rare and easy to be misdiagnosed.It is necessary to further study the characteristics of the disease so as to improve curative effect and prognosis.