中华外科杂志
中華外科雜誌
중화외과잡지
CHINESE JOURNAL OF SURGERY
2009年
22期
1685-1688
,共4页
汤坚强%杨尹默%熊焰%万远廉%朱学骏
湯堅彊%楊尹默%熊燄%萬遠廉%硃學駿
탕견강%양윤묵%웅염%만원렴%주학준
巨淋巴结增生%腹膜后肿瘤%副肿瘤性天疱疮%外科手术
巨淋巴結增生%腹膜後腫瘤%副腫瘤性天皰瘡%外科手術
거림파결증생%복막후종류%부종류성천포창%외과수술
Giant lymph node hyperplasia%Retroperitoneal neoplasms%Pemphigus,paraneoplastic%Surgical procedures,operative
目的 探讨腹膜后局限性Castleman病的临床特征、诊治策略及影响预后的因素,提高伴副肿瘤性天疱疮(PNP)的Castleman病的诊治水平.方法 回顾性分析1993年1月至2009年5月在北京大学第一医院普通外科接受手术治疗的20例腹膜后Castleman病患者的临床资料,并将13例腹膜后Castleman病伴PNP患者的临床资料与同期7例单纯腹膜后Castleman病患者进行比较,分析两者在发病特点、部位、实验室检查、手术策略、病理特征及外科治疗效果的不同.结果 (1)本组腹膜后Castleman病多见于中青年(中位年龄36岁),发病部位多位于肾旁(14/20,70%)及髂窝(4/20,20%),左侧腹膜后多见,病理分型以透明血管型为主,腹膜后Castleman病合并PNP组的性别、年龄、肿瘤发病部位、大小及病理分型与单纯腹膜后Castleman病组无明显区别(P>0.05);(2)本组腹膜后Castleman病合并PNP患者较易合并闭塞性细支气管炎(8/13),有血清肿瘤标志物癌胚抗原或CA242升高现象(3/8);(3)本组腹膜后Castleman病常有包膜,与邻近脏器边界清晰,手术较为容易,但合并PNP的腹膜后Castleman病的生物学行为有恶性倾向及伴发子灶特征,有侵及邻近血管及术后局部复发可能;(4)Kaplan-Meier及Log-Rank生存分析显示,腹膜后Castleman病合并PNP患者5年生存率为42.8%,平均生存时间30个月,明显低于单纯腹膜后Castleman病组(P<0.05),是否合并闭塞性细支气管炎以及肿瘤能否根治切除是影响腹膜后Castleman病患者预后的重要因素.结论 腹膜后Castleman病伴PNP具有独特的临床特征,早期诊断和切除肿瘤、及时终止致病抗体的产生,是成功治愈的关键.
目的 探討腹膜後跼限性Castleman病的臨床特徵、診治策略及影響預後的因素,提高伴副腫瘤性天皰瘡(PNP)的Castleman病的診治水平.方法 迴顧性分析1993年1月至2009年5月在北京大學第一醫院普通外科接受手術治療的20例腹膜後Castleman病患者的臨床資料,併將13例腹膜後Castleman病伴PNP患者的臨床資料與同期7例單純腹膜後Castleman病患者進行比較,分析兩者在髮病特點、部位、實驗室檢查、手術策略、病理特徵及外科治療效果的不同.結果 (1)本組腹膜後Castleman病多見于中青年(中位年齡36歲),髮病部位多位于腎徬(14/20,70%)及髂窩(4/20,20%),左側腹膜後多見,病理分型以透明血管型為主,腹膜後Castleman病閤併PNP組的性彆、年齡、腫瘤髮病部位、大小及病理分型與單純腹膜後Castleman病組無明顯區彆(P>0.05);(2)本組腹膜後Castleman病閤併PNP患者較易閤併閉塞性細支氣管炎(8/13),有血清腫瘤標誌物癌胚抗原或CA242升高現象(3/8);(3)本組腹膜後Castleman病常有包膜,與鄰近髒器邊界清晰,手術較為容易,但閤併PNP的腹膜後Castleman病的生物學行為有噁性傾嚮及伴髮子竈特徵,有侵及鄰近血管及術後跼部複髮可能;(4)Kaplan-Meier及Log-Rank生存分析顯示,腹膜後Castleman病閤併PNP患者5年生存率為42.8%,平均生存時間30箇月,明顯低于單純腹膜後Castleman病組(P<0.05),是否閤併閉塞性細支氣管炎以及腫瘤能否根治切除是影響腹膜後Castleman病患者預後的重要因素.結論 腹膜後Castleman病伴PNP具有獨特的臨床特徵,早期診斷和切除腫瘤、及時終止緻病抗體的產生,是成功治愈的關鍵.
목적 탐토복막후국한성Castleman병적림상특정、진치책략급영향예후적인소,제고반부종류성천포창(PNP)적Castleman병적진치수평.방법 회고성분석1993년1월지2009년5월재북경대학제일의원보통외과접수수술치료적20례복막후Castleman병환자적림상자료,병장13례복막후Castleman병반PNP환자적림상자료여동기7례단순복막후Castleman병환자진행비교,분석량자재발병특점、부위、실험실검사、수술책략、병리특정급외과치료효과적불동.결과 (1)본조복막후Castleman병다견우중청년(중위년령36세),발병부위다위우신방(14/20,70%)급가와(4/20,20%),좌측복막후다견,병리분형이투명혈관형위주,복막후Castleman병합병PNP조적성별、년령、종류발병부위、대소급병리분형여단순복막후Castleman병조무명현구별(P>0.05);(2)본조복막후Castleman병합병PNP환자교역합병폐새성세지기관염(8/13),유혈청종류표지물암배항원혹CA242승고현상(3/8);(3)본조복막후Castleman병상유포막,여린근장기변계청석,수술교위용역,단합병PNP적복막후Castleman병적생물학행위유악성경향급반발자조특정,유침급린근혈관급술후국부복발가능;(4)Kaplan-Meier급Log-Rank생존분석현시,복막후Castleman병합병PNP환자5년생존솔위42.8%,평균생존시간30개월,명현저우단순복막후Castleman병조(P<0.05),시부합병폐새성세지기관염이급종류능부근치절제시영향복막후Castleman병환자예후적중요인소.결론 복막후Castleman병반PNP구유독특적림상특정,조기진단화절제종류、급시종지치병항체적산생,시성공치유적관건.
Objective To investigate the clinical characteristics,surgical treatment and prognosis analysis of localized retroperitoneal Casfleman disease (CD),and to improve the level of diagnosis and treatment of retroperitoneal Castleman disease with paraneoplastic pemphigus(PNP).Methods The clinical data of retroperitoneal CD with PNP from January 1993 to May 2009 were compared with CD without PNP retrospectively,including clinical features,tumor site,lab examination,surgical treatment,pathologic subtype and results of surgery.Results (1) Retroperitoneal Castleman disease more likely originated in para-kidney and iliac fossa with middle age of 36 years old,especially in left retroperitoneum.Of the 20 cases,18 tumors (90%) were hyaline vascular variants and 2 were mixed variants.There was no statistical difference in gender,age,tumor site and pathological subtype between two groups.(2) Retroperitoneal CD with PNP more likely complicated with bronchiolitis obliterans (BO) and high level of serum CEA/CA242.(3)Retroperitoneal Castleman tumors had clear margin,intact envelop and were easily resectable,however the biological behavior of CD with PNP might tend malignant changing,invade adjacent blood vessel or seed locally,and eventually relapse after operation.(4)The 5-year survival rate of retroperitoneal CD with PNP was 42.8%,significantly lower than those without PNP.The average survival time was 30 months.Bronchiolitis obliterans and radical resection were the key effect in prognosis of retroperitoneal CD.Conclusions Retroperitoneal CD with PNP has distinctive clinical features.Early diagnosis,prompt removal of tumor and termination secretion of causative antibody are critical to the management of this disease.
