原發(fā)性腹膜后軟組織肉瘤和脂肪肉瘤臨床病理特點(diǎn)及預(yù)后分析
發(fā)布時(shí)間：2018-09-19 20:52
【摘要】：背景原發(fā)性腹膜后軟組織肉瘤(Primary retroperitoneal soft tissue sarcoma,PRSTS)是較多見的腹膜后惡性腫瘤,來源復(fù)雜。原發(fā)性腹膜后脂肪肉瘤(Primary retroperitoneal liposarcoma,PRPLS)是最常見的腹膜后軟組織肉瘤,約占其40%[1]。PRPLS常起源于腎周脂肪組織。由于起病隱匿,缺乏典型的臨床癥狀,且由于腹膜后可擴(kuò)展的間隙大,瘤體常生長(zhǎng)巨大,與鄰近臟器關(guān)系復(fù)雜,故早期診斷較為困難。手術(shù)切除是最有效的治療手段[2],但術(shù)后復(fù)發(fā)率極高,且輔助性放化療對(duì)其不敏感,預(yù)后較差。國(guó)內(nèi)外對(duì)PRSTS和PRPLS報(bào)道較少,研究不夠系統(tǒng)和全面,故其診治也成為臨床工作中急需解決的困難。研究目的回顧性分析PRSTS和PRPLS的臨床病理特點(diǎn),探討并總結(jié)影響PRPLS預(yù)后的相關(guān)因素。研究方法病例收集自2005年1月至2015年3月于我院術(shù)后病理診斷為PRSTS 298例,PRPLS67例。采用門診復(fù)查、電話及書信方式進(jìn)行隨訪。隨訪時(shí)間截止2015年10月。詳細(xì)統(tǒng)計(jì)PRSTS患者的性別、年齡、主要臨床癥狀、腫瘤生長(zhǎng)部位、治療情況和病理類型。對(duì)PRPLS患者的腫瘤標(biāo)本行HE染色,采用S-P法行免疫組化,分析Ki-67、Vimentin和S-100的免疫組化表達(dá)結(jié)果,并分析Ki-67陽(yáng)性表達(dá)指數(shù)與患者預(yù)后的關(guān)系。詳細(xì)統(tǒng)計(jì)PRPLS患者初發(fā)腫瘤的病理亞型、危險(xiǎn)度分級(jí)、手術(shù)方式、腫瘤大小、年齡性別、術(shù)后第一次復(fù)發(fā)時(shí)間和術(shù)后隨訪時(shí)間,對(duì)比分析影響其預(yù)后、復(fù)發(fā)的因素及臨床療效。所有數(shù)據(jù)分析均采用SPSS 18.0軟件進(jìn)行。采用Kaplan-Meier法繪制生存曲線,單因素生存分析采用Log-rank檢驗(yàn),多因素分析采用COX回歸模型,相關(guān)性分析采用χ2檢驗(yàn)和非參數(shù)檢驗(yàn)。P0.05為差異有統(tǒng)計(jì)學(xué)意義。結(jié)果截止到2015年3月,我院術(shù)后病理確診PRSTS共298例,男141例,女157例,年齡15-78歲,中位年齡52歲�；颊吲R床癥狀主要表現(xiàn)為腹部包塊伴腹圍增大、腹脹腹痛、消瘦納差、腰部痛或腎區(qū)占位等。腫瘤位于左上腹64例,右上腹71例,下腹部106例,盆腔57例。161例腹腔或盆腔臟器受侵犯,51例血管受侵犯。174例行初發(fā)腫瘤完整切除術(shù),124例行姑息性手術(shù)。術(shù)后病理確診脂肪肉瘤67例,平滑肌肉瘤47例,惡性纖維組織細(xì)胞瘤50例,纖維肉瘤25例,橫紋肌肉瘤13例,剩余96例為其他罕見病理類型。共有65例PRPLS患者被隨訪。男35例,女30例,年齡16-77歲,平均生存時(shí)間38.1個(gè)月,總生存率為58.5%。51例行原發(fā)腫瘤完整切除術(shù),男性27例,女性24例,平均生存時(shí)間43.3個(gè)月,總生存率64.7%;其中25例(49%)為高分化型,8例(15.7%)為黏液型/圓形細(xì)胞型,8例(15.7%)為多形性/混合型,10例(19.6%)為去分化型;33例(64.7%)為低危險(xiǎn)度,18例(35.3%)為高危險(xiǎn)度;32例行聯(lián)合臟器切除,其中15例術(shù)后病理提示鏡下切緣陽(yáng)性;22例行腫瘤完整切除術(shù)后出現(xiàn)局部復(fù)發(fā),平均復(fù)發(fā)時(shí)間為29.2個(gè)月。14例行腫瘤姑息性切除或活檢術(shù),其中位生存時(shí)間為19.4個(gè)月,總生存率為35.7%。免疫組化結(jié)果顯示:65例中,Ki-67表達(dá)陽(yáng)性64例,Vimentin表達(dá)陽(yáng)性62例,S-100表達(dá)陽(yáng)性23例。Ki-67和Vimentin在PRPLS中陽(yáng)性表達(dá)較S-100敏感性更高。Ki-67主要在高危險(xiǎn)度PRPLS中,即去分化型、多形性和混合型中表達(dá),而S-100主要在高分化PRPLS中表達(dá)。此外,PRPLS患者預(yù)后與Ki-67的表達(dá)呈負(fù)相關(guān)。生存分析結(jié)果顯示:影響PRPLS患者預(yù)后的因素分別為初發(fā)腫瘤的病理亞型(χ2=19.467,P0.01)、病理危險(xiǎn)度(χ2=19.053,P0.01)、腫瘤直徑(χ2=6.826,P0.05)、腫瘤完整切除術(shù)(χ2=15.471,P0.01)、聯(lián)合臟器切除術(shù)(χ2=7.130,P0.01)。影響患者術(shù)后局部復(fù)發(fā)的因素分別為病理亞型(χ2=14.995,P0.01)、病理危險(xiǎn)度(χ2=14.810,P0.01)。肉眼切緣陽(yáng)性顯著影響預(yù)后(χ2=15.471,P0.01),但鏡下切緣陽(yáng)性不影響預(yù)后(p0.05)。多因素分析顯示:病理亞型、危險(xiǎn)度及聯(lián)合臟器切除術(shù)是影響預(yù)后的獨(dú)立因素;危險(xiǎn)度是影響復(fù)發(fā)的獨(dú)立因素�；颊吣挲g、性別不影響預(yù)后與復(fù)發(fā)。相關(guān)性結(jié)果顯示:術(shù)后局部復(fù)發(fā)與預(yù)后顯著相關(guān)(P0.01)。病理危險(xiǎn)度與腫瘤直徑無關(guān)(P0.05),而與鄰近臟器的侵犯有關(guān)(P0.01)。腫瘤直徑與鄰近臟器侵犯(P0.05)、局部復(fù)發(fā)(P0.05)均無明顯相關(guān)性。結(jié)論1.PRSTS好發(fā)于40-60歲,性別無差異;腫瘤主要位于下腹部,易侵犯鄰近臟器和血管。初發(fā)腫瘤完整切除率不高。脂肪肉瘤、惡性纖維組織細(xì)胞瘤和平滑肌肉瘤為最常見病理類型。2.PRPLS是最常見的PRSTS病理類型;手術(shù)切除目前是PRPLS最有效的治療手段3.診斷PRPLS時(shí),Ki-67和Vimentin較S-100靈敏度高;Ki-67表達(dá)指數(shù)與PRPLS患者預(yù)后負(fù)相關(guān)。4.影響PRPLS患者預(yù)后的獨(dú)立危險(xiǎn)因素分別為初發(fā)腫瘤的病理亞型、病理危險(xiǎn)度、聯(lián)合臟器切除。5.腫瘤危險(xiǎn)度是影響PRPLS患者復(fù)發(fā)的獨(dú)立因素。6.鏡下切緣陽(yáng)性不影響預(yù)后;術(shù)中肉眼切緣陽(yáng)性顯著影響預(yù)后。7.PRPLS患者術(shù)后最常見、最主要的死因是術(shù)后腫瘤復(fù)發(fā)。
[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.
【學(xué)位授予單位】：第三軍醫(yī)大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2017
【分類號(hào)】：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.
【學(xué)位授予單位】：第三軍醫(yī)大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2017
【分類號(hào)】：R735.4
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