原发性腹膜后软组织肉瘤和脂肪肉瘤临床病理特点及预后分析
发布时间：2018-09-19 20:52
【摘要】：背景原发性腹膜后软组织肉瘤(Primary retroperitoneal soft tissue sarcoma,PRSTS)是较多见的腹膜后恶性肿瘤,来源复杂。原发性腹膜后脂肪肉瘤(Primary retroperitoneal liposarcoma,PRPLS)是最常见的腹膜后软组织肉瘤,约占其40%[1]。PRPLS常起源于肾周脂肪组织。由于起病隐匿,缺乏典型的临床症状,且由于腹膜后可扩展的间隙大,瘤体常生长巨大,与邻近脏器关系复杂,故早期诊断较为困难。手术切除是最有效的治疗手段[2],但术后复发率极高,且辅助性放化疗对其不敏感,预后较差。国内外对PRSTS和PRPLS报道较少,研究不够系统和全面,故其诊治也成为临床工作中急需解决的困难。研究目的回顾性分析PRSTS和PRPLS的临床病理特点,探讨并总结影响PRPLS预后的相关因素。研究方法病例收集自2005年1月至2015年3月于我院术后病理诊断为PRSTS 298例,PRPLS67例。采用门诊复查、电话及书信方式进行随访。随访时间截止2015年10月。详细统计PRSTS患者的性别、年龄、主要临床症状、肿瘤生长部位、治疗情况和病理类型。对PRPLS患者的肿瘤标本行HE染色,采用S-P法行免疫组化,分析Ki-67、Vimentin和S-100的免疫组化表达结果,并分析Ki-67阳性表达指数与患者预后的关系。详细统计PRPLS患者初发肿瘤的病理亚型、危险度分级、手术方式、肿瘤大小、年龄性别、术后第一次复发时间和术后随访时间,对比分析影响其预后、复发的因素及临床疗效。所有数据分析均采用SPSS 18.0软件进行。采用Kaplan-Meier法绘制生存曲线,单因素生存分析采用Log-rank检验,多因素分析采用COX回归模型,相关性分析采用χ2检验和非参数检验。P0.05为差异有统计学意义。结果截止到2015年3月,我院术后病理确诊PRSTS共298例,男141例,女157例,年龄15-78岁,中位年龄52岁。患者临床症状主要表现为腹部包块伴腹围增大、腹胀腹痛、消瘦纳差、腰部痛或肾区占位等。肿瘤位于左上腹64例,右上腹71例,下腹部106例,盆腔57例。161例腹腔或盆腔脏器受侵犯,51例血管受侵犯。174例行初发肿瘤完整切除术,124例行姑息性手术。术后病理确诊脂肪肉瘤67例,平滑肌肉瘤47例,恶性纤维组织细胞瘤50例,纤维肉瘤25例,横纹肌肉瘤13例,剩余96例为其他罕见病理类型。共有65例PRPLS患者被随访。男35例,女30例,年龄16-77岁,平均生存时间38.1个月,总生存率为58.5%。51例行原发肿瘤完整切除术,男性27例,女性24例,平均生存时间43.3个月,总生存率64.7%;其中25例(49%)为高分化型,8例(15.7%)为黏液型/圆形细胞型,8例(15.7%)为多形性/混合型,10例(19.6%)为去分化型;33例(64.7%)为低危险度,18例(35.3%)为高危险度;32例行联合脏器切除,其中15例术后病理提示镜下切缘阳性;22例行肿瘤完整切除术后出现局部复发,平均复发时间为29.2个月。14例行肿瘤姑息性切除或活检术,其中位生存时间为19.4个月,总生存率为35.7%。免疫组化结果显示:65例中,Ki-67表达阳性64例,Vimentin表达阳性62例,S-100表达阳性23例。Ki-67和Vimentin在PRPLS中阳性表达较S-100敏感性更高。Ki-67主要在高危险度PRPLS中,即去分化型、多形性和混合型中表达,而S-100主要在高分化PRPLS中表达。此外,PRPLS患者预后与Ki-67的表达呈负相关。生存分析结果显示:影响PRPLS患者预后的因素分别为初发肿瘤的病理亚型(χ2=19.467,P0.01)、病理危险度(χ2=19.053,P0.01)、肿瘤直径(χ2=6.826,P0.05)、肿瘤完整切除术(χ2=15.471,P0.01)、联合脏器切除术(χ2=7.130,P0.01)。影响患者术后局部复发的因素分别为病理亚型(χ2=14.995,P0.01)、病理危险度(χ2=14.810,P0.01)。肉眼切缘阳性显著影响预后(χ2=15.471,P0.01),但镜下切缘阳性不影响预后(p0.05)。多因素分析显示:病理亚型、危险度及联合脏器切除术是影响预后的独立因素;危险度是影响复发的独立因素。患者年龄、性别不影响预后与复发。相关性结果显示:术后局部复发与预后显著相关(P0.01)。病理危险度与肿瘤直径无关(P0.05),而与邻近脏器的侵犯有关(P0.01)。肿瘤直径与邻近脏器侵犯(P0.05)、局部复发(P0.05)均无明显相关性。结论1.PRSTS好发于40-60岁,性别无差异;肿瘤主要位于下腹部,易侵犯邻近脏器和血管。初发肿瘤完整切除率不高。脂肪肉瘤、恶性纤维组织细胞瘤和平滑肌肉瘤为最常见病理类型。2.PRPLS是最常见的PRSTS病理类型;手术切除目前是PRPLS最有效的治疗手段3.诊断PRPLS时,Ki-67和Vimentin较S-100灵敏度高;Ki-67表达指数与PRPLS患者预后负相关。4.影响PRPLS患者预后的独立危险因素分别为初发肿瘤的病理亚型、病理危险度、联合脏器切除。5.肿瘤危险度是影响PRPLS患者复发的独立因素。6.镜下切缘阳性不影响预后;术中肉眼切缘阳性显著影响预后。7.PRPLS患者术后最常见、最主要的死因是术后肿瘤复发。
[Abstract]:Background Primary retroperitoneal soft tissue sarcoma (PRSTS) is a common retroperitoneal malignant tumor with complex origin. Primary retroperitoneal liposarcoma (PRPLS) is the most common retroperitoneal soft tissue sarcoma, accounting for 40% [1]. Tissue. Because of the concealed onset, lack of typical clinical symptoms, and because of the large retroperitoneal space, tumor growth is often huge, and the complex relationship with adjacent organs, early diagnosis is difficult. Surgical resection is the most effective treatment, but the recurrence rate is very high, and adjuvant radiotherapy and chemotherapy is insensitive to it, poor prognosis at home and abroad. Objective To retrospectively analyze the clinicopathological characteristics of PRSTS and PRPLLS, and to explore and summarize the related factors affecting the prognosis of PRPLS. 298 PRSTS cases were diagnosed and 67 PRPLS cases were followed up by outpatient follow-up, telephone and letter. The follow-up period was up to October 2015. The sex, age, main clinical symptoms, tumor growth site, treatment and pathological types of PRSTS patients were analyzed in detail. The tumor specimens of PRPLS patients were stained with HE, and immunohistochemistry was performed with S-P method. Immunohistochemical expression of i-67, Vimentin and S-100, and the relationship between the positive expression index of Ki-67 and prognosis were analyzed. The pathological subtypes, risk classification, surgical methods, tumor size, age and sex, the first recurrence time and the follow-up time after surgery were analyzed in detail. All data were analyzed by SPSS 18.0 software. Survival curve was drawn by Kaplan-Meier method. Log-rank test was used for univariate survival analysis. COX regression model was used for multivariate analysis. _2 test and nonparametric test were used for correlation analysis. Postoperative pathological diagnosis of PRSTS was made in 298 patients, 141 males and 157 females, aged 15-78 years with a median age of 52. The main clinical symptoms were abdominal mass with abdominal circumference enlargement, abdominal distention, abdominal pain, poor appetite for emaciation, low back pain or kidney space occupancy. Total of 174 patients underwent complete resection of primary tumor and 124 palliative surgery. 67 patients with liposarcoma, 47 patients with leiomyosarcoma, 50 patients with malignant fibrous histiocytoma, 25 patients with fibrosarcoma, 13 patients with rhabdomyosarcoma and 96 patients with PRPLS were followed up. There were 35 males and 30 females, aged 16-77 years, with an average survival time of 38.1 months and a total survival rate of 58.5%. 51 patients underwent complete resection of primary tumors, 27 males and 24 females, with an average survival time of 43.3 months and a total survival rate of 64.7%. Among them, 25 (49%) were highly differentiated, 8 (15.7%) were mucinous/round cell type, 8 (15.7%) were polymorphic/mixed type, and 10.7% were polymorphic/mixed type. Thirty-three cases (64.7%) were low risk, 18 cases (35.3%) were high risk, 32 cases underwent combined organ resection, of which 15 cases had positive surgical margin, 22 cases had local recurrence after complete tumor resection, with an average recurrence time of 29.2 months. Ki-67 was expressed in 64 of 65 cases, Vimentin in 62, S-100 in 23. Ki-67 and Vimentin were more sensitive in PRPLS than S-100. Ki-67 was mainly expressed in high risk PRPLS, i.e. dedifferentiated PRPLS, polymorphic PRPLS and mixed PRPLS. The prognosis of PRPLS patients was negatively correlated with the expression of Ki-67. Survival analysis showed that the prognostic factors of PRPLS patients were pathological subtype of primary tumor (2 = 19.467, P 0.01), pathological risk (2 = 19.053, P 0.01), tumor diameter (2 = 6.826, P 0.05), complete resection of tumor (2 = 15.471, P 0.01). The factors affecting local recurrence were pathological subtype (_2 = 14.995, P 0.01), pathological risk (_2 = 14.810, P 0.01). Positive margin of naked eye significantly affected prognosis (2 = 15.471, P 0.01), but positive margin of incision did not affect prognosis (P 0.05). Age and sex did not affect prognosis and recurrence. Correlation analysis showed that local recurrence was significantly associated with prognosis (P 0.01). Pathological risk was not associated with tumor diameter (P 0.05), but with invasion of adjacent organs (P 0.01). There was no significant correlation between tumor diameter and adjacent organ invasion (P 0.05), and local recurrence (P 0.05). Conclusion 1. PRSTS was predominant in 40-60 years old, with no gender difference. The tumors were mainly located in the lower abdomen and easily invaded adjacent organs and blood vessels. Type 2. PRPLS is the most common pathological type of PRSTS. Surgical resection is currently the most effective treatment for PRPLS. 3. Ki-67 and Vimentin are more sensitive than S-100 in the diagnosis of PRPLS. Ki-67 expression index is negatively correlated with the prognosis of PRPLS patients. 4. The independent risk factors affecting the prognosis of PRPLS patients are pathological subtypes of primary tumor, pathological risk, and association. 5. Tumor risk is an independent factor affecting the recurrence of PRPLS patients. 6. Positive margin under microscope does not affect the prognosis; Positive margin during surgery significantly affects the prognosis. 7. Postoperative PRPLS patients are the most common, the main cause of death is postoperative tumor recurrence.
【学位授予单位】：第三军医大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R735.4
[Abstract]:Background Primary retroperitoneal soft tissue sarcoma (PRSTS) is a common retroperitoneal malignant tumor with complex origin. Primary retroperitoneal liposarcoma (PRPLS) is the most common retroperitoneal soft tissue sarcoma, accounting for 40% [1]. Tissue. Because of the concealed onset, lack of typical clinical symptoms, and because of the large retroperitoneal space, tumor growth is often huge, and the complex relationship with adjacent organs, early diagnosis is difficult. Surgical resection is the most effective treatment, but the recurrence rate is very high, and adjuvant radiotherapy and chemotherapy is insensitive to it, poor prognosis at home and abroad. Objective To retrospectively analyze the clinicopathological characteristics of PRSTS and PRPLLS, and to explore and summarize the related factors affecting the prognosis of PRPLS. 298 PRSTS cases were diagnosed and 67 PRPLS cases were followed up by outpatient follow-up, telephone and letter. The follow-up period was up to October 2015. The sex, age, main clinical symptoms, tumor growth site, treatment and pathological types of PRSTS patients were analyzed in detail. The tumor specimens of PRPLS patients were stained with HE, and immunohistochemistry was performed with S-P method. Immunohistochemical expression of i-67, Vimentin and S-100, and the relationship between the positive expression index of Ki-67 and prognosis were analyzed. The pathological subtypes, risk classification, surgical methods, tumor size, age and sex, the first recurrence time and the follow-up time after surgery were analyzed in detail. All data were analyzed by SPSS 18.0 software. Survival curve was drawn by Kaplan-Meier method. Log-rank test was used for univariate survival analysis. COX regression model was used for multivariate analysis. _2 test and nonparametric test were used for correlation analysis. Postoperative pathological diagnosis of PRSTS was made in 298 patients, 141 males and 157 females, aged 15-78 years with a median age of 52. The main clinical symptoms were abdominal mass with abdominal circumference enlargement, abdominal distention, abdominal pain, poor appetite for emaciation, low back pain or kidney space occupancy. Total of 174 patients underwent complete resection of primary tumor and 124 palliative surgery. 67 patients with liposarcoma, 47 patients with leiomyosarcoma, 50 patients with malignant fibrous histiocytoma, 25 patients with fibrosarcoma, 13 patients with rhabdomyosarcoma and 96 patients with PRPLS were followed up. There were 35 males and 30 females, aged 16-77 years, with an average survival time of 38.1 months and a total survival rate of 58.5%. 51 patients underwent complete resection of primary tumors, 27 males and 24 females, with an average survival time of 43.3 months and a total survival rate of 64.7%. Among them, 25 (49%) were highly differentiated, 8 (15.7%) were mucinous/round cell type, 8 (15.7%) were polymorphic/mixed type, and 10.7% were polymorphic/mixed type. Thirty-three cases (64.7%) were low risk, 18 cases (35.3%) were high risk, 32 cases underwent combined organ resection, of which 15 cases had positive surgical margin, 22 cases had local recurrence after complete tumor resection, with an average recurrence time of 29.2 months. Ki-67 was expressed in 64 of 65 cases, Vimentin in 62, S-100 in 23. Ki-67 and Vimentin were more sensitive in PRPLS than S-100. Ki-67 was mainly expressed in high risk PRPLS, i.e. dedifferentiated PRPLS, polymorphic PRPLS and mixed PRPLS. The prognosis of PRPLS patients was negatively correlated with the expression of Ki-67. Survival analysis showed that the prognostic factors of PRPLS patients were pathological subtype of primary tumor (2 = 19.467, P 0.01), pathological risk (2 = 19.053, P 0.01), tumor diameter (2 = 6.826, P 0.05), complete resection of tumor (2 = 15.471, P 0.01). The factors affecting local recurrence were pathological subtype (_2 = 14.995, P 0.01), pathological risk (_2 = 14.810, P 0.01). Positive margin of naked eye significantly affected prognosis (2 = 15.471, P 0.01), but positive margin of incision did not affect prognosis (P 0.05). Age and sex did not affect prognosis and recurrence. Correlation analysis showed that local recurrence was significantly associated with prognosis (P 0.01). Pathological risk was not associated with tumor diameter (P 0.05), but with invasion of adjacent organs (P 0.01). There was no significant correlation between tumor diameter and adjacent organ invasion (P 0.05), and local recurrence (P 0.05). Conclusion 1. PRSTS was predominant in 40-60 years old, with no gender difference. The tumors were mainly located in the lower abdomen and easily invaded adjacent organs and blood vessels. Type 2. PRPLS is the most common pathological type of PRSTS. Surgical resection is currently the most effective treatment for PRPLS. 3. Ki-67 and Vimentin are more sensitive than S-100 in the diagnosis of PRPLS. Ki-67 expression index is negatively correlated with the prognosis of PRPLS patients. 4. The independent risk factors affecting the prognosis of PRPLS patients are pathological subtypes of primary tumor, pathological risk, and association. 5. Tumor risk is an independent factor affecting the recurrence of PRPLS patients. 6. Positive margin under microscope does not affect the prognosis; Positive margin during surgery significantly affects the prognosis. 7. Postoperative PRPLS patients are the most common, the main cause of death is postoperative tumor recurrence.
【学位授予单位】：第三军医大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R735.4
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