原發(fā)性胰腺小細(xì)胞癌的薈萃分析

彭春艷 呂瑛 姚仁玲 徐肇敏 鄒曉平

·論著·

原發(fā)性胰腺小細(xì)胞癌的薈萃分析

彭春艷 呂瑛 姚仁玲 徐肇敏 鄒曉平

目的探討原發(fā)性胰腺小細(xì)胞癌的臨床病理特點(diǎn)、治療及預(yù)后情況。方法聯(lián)機(jī)檢索中國(guó)期刊全文數(shù)據(jù)庫、維普中文科技期刊數(shù)據(jù)庫、Medline/Pubmed及OVID全文數(shù)據(jù)庫，檢索截止至2012年4月，并結(jié)合南京鼓樓醫(yī)院診治的1例患者，進(jìn)行系統(tǒng)評(píng)價(jià)。結(jié)果納入28篇文獻(xiàn)46例診斷明確的病例，加上鼓樓醫(yī)院1例，共47例胰腺小細(xì)胞癌患者。該病以男性多見，中位年齡62歲，臨床表現(xiàn)以腹痛、黃疸及消瘦為主，較少產(chǎn)生副腫瘤綜合征；多見血清神經(jīng)元特異性烯醇化酶升高；CT檢查示病灶多呈不均勻強(qiáng)化，確診依靠病理檢查；63.8%(30/47)的患者確診時(shí)已發(fā)生轉(zhuǎn)移，患者總的中位生存期為28周。目前無統(tǒng)一治療模式，推薦以化療為主的系統(tǒng)治療。結(jié)論胰腺小細(xì)胞癌是一種發(fā)生率低、惡性程度高、侵襲性強(qiáng)、預(yù)后差的神經(jīng)內(nèi)分泌腫瘤。

神經(jīng)內(nèi)分泌瘤； 癌，小細(xì)胞； 胰腺； Meta分析

小細(xì)胞癌是一種惡性程度高、侵襲性強(qiáng)及預(yù)后極差的神經(jīng)內(nèi)分泌腫瘤，多見于肺臟，約占所有支氣管起源腫瘤的20%～25%[1]，肺外部位少見，僅占所有小細(xì)胞癌的2.5%～4%[2]，可發(fā)生于膀胱、前列腺、子宮、食管、膽囊、胰腺及結(jié)腸等部位。原發(fā)性胰腺小細(xì)胞癌最早報(bào)道于1973年[3]，其占所有胰腺惡性腫瘤的1%[4]。目前研究多以個(gè)案報(bào)道為主，故對(duì)其臨床表現(xiàn)、生物學(xué)特征及治療方案的系統(tǒng)了解非常有限。此外，臨床實(shí)踐顯示此類患者早期確診困難，就診時(shí)多屬中晚期，預(yù)后差。因此，如何正確認(rèn)識(shí)和處理好該病，對(duì)于改善患者預(yù)后有重要意義。本研究回顧性分析國(guó)內(nèi)外文獻(xiàn)，并結(jié)合我院診治的1例原發(fā)性胰腺小細(xì)胞癌資料，以期提高對(duì)該病的認(rèn)識(shí)。

材料與方法

一、文獻(xiàn)檢索及入選、排除標(biāo)準(zhǔn)

聯(lián)機(jī)檢索中英文數(shù)據(jù)庫，包括Medline/Pubmed(1960.1-2012.4)及OVID(1978.1-2012.4)全文數(shù)據(jù)庫、中國(guó)期刊全文數(shù)據(jù)庫(Chinese Journal Full-text Database, 1979.1-2012.4)及維普中文科技期刊數(shù)據(jù)庫(VIP Database for Chinese Technical Periodicals, 1989.1-2012.4)，手工檢索所獲文獻(xiàn)的參考文獻(xiàn)。關(guān)鍵詞：neuroendocrine carcinoma pancreas，small cell cancer pancreas，胰腺小細(xì)胞癌，胰腺神經(jīng)內(nèi)分泌癌。

入選標(biāo)準(zhǔn)：(1)通過活檢穿刺或手術(shù)病理確診的病例；(2)患者資料至少包括年齡、性別、臨床表現(xiàn)、實(shí)驗(yàn)室檢查、影像學(xué)資料、病理表現(xiàn)、免疫組化、治療方法及預(yù)后情況等項(xiàng)目中的5項(xiàng)；(3)重復(fù)報(bào)道病例僅納入最近發(fā)表的一篇文章。排除標(biāo)準(zhǔn)：(1)臨床資料不全，僅達(dá)上述納入標(biāo)準(zhǔn)5項(xiàng)以下者；(2)重復(fù)病例；(3)除外其他部位轉(zhuǎn)移的繼發(fā)性胰腺小細(xì)胞癌。

二、統(tǒng)計(jì)學(xué)分析

結(jié) 果

一、納入文獻(xiàn)

共檢索出1057篇文獻(xiàn)，經(jīng)仔細(xì)閱讀標(biāo)題和摘要，初步排除不符合研究目的的文獻(xiàn)1020篇，獲得可能符合納入標(biāo)準(zhǔn)的英文文獻(xiàn)27篇，中文文獻(xiàn)10篇。仔細(xì)閱讀全文，9篇文獻(xiàn)因未能提供患者詳細(xì)信息而被排除。最終納入22篇英文文獻(xiàn)[3-24]和6篇中文文獻(xiàn)[25-30]。

二、一般資料及臨床特征

28篇文獻(xiàn)共報(bào)道46例胰腺小細(xì)胞癌患者，加上我院1例，共47例。其中男性26例，女性21例，男女比為1.2∶1，發(fā)病年齡23～75歲，中位年齡62歲。最常見臨床表現(xiàn)為腹痛(15/47,31.9%)、黃疸(9/47,19.1%)、體重下降(8/47,17.0%)。個(gè)例表現(xiàn)為副腫瘤綜合征，例如異位促腎上腺皮質(zhì)激素增多癥[3]和高鈣血癥[14]。

胰腺小細(xì)胞癌可發(fā)生于胰腺任何部位，本組胰頭部26例(55.3%)，胰尾部10例(21.3%)，胰體部6例(12.8%)，全胰彌漫性病變2例(4.1%)，胰頭體部1例(2.1%)，頭尾部1例(2.1%)，體尾部1例(2.1%)；腫瘤長(zhǎng)徑2～15 cm，平均(5.5±2.7)cm；單發(fā)45例(95.7%)，多發(fā)2例。確診時(shí)30例(63.8%)發(fā)生轉(zhuǎn)移，常見轉(zhuǎn)移部位為胰周淋巴結(jié)(18/47，38.3%)、肝臟(11/47，23.4%)，少見的轉(zhuǎn)移部位有腎上腺、骨髓、脾臟、鄰近血管及皮膚等。

三、腫瘤標(biāo)記物及激素分泌

18例檢測(cè)血清CEA和CA19-9，各有2例輕度升高[16-17,19-20]。10例檢測(cè)血清神經(jīng)元特異性烯醇化酶(neuron-specific enolase，NSE)，5例顯著升高[11,16-17, 20]。5例測(cè)定血胰島素、促胃液素及胰高血糖素水平，結(jié)果均在正常范圍[9, 11, 17-18,20]。3例行血乳酸脫氫酶檢查，結(jié)果均顯著升高[8-9,11]。1例血促腎上腺皮質(zhì)激素升高[3]，1例高鈣血癥[14]，1例血胃泌素釋放肽前體升高，1例(術(shù)后檢查)血泌乳素升高[28]，1例檢測(cè)血腸血管活性多肽及尿5-羥吲哚乙酸，結(jié)果正常[17]。

四、影像學(xué)特征

共35例有詳細(xì)的影像學(xué)特征記錄。20例患者行上腹部CT平掃+增強(qiáng)[7, 9-14, 18-21, 24,27-29]，其中18例平掃時(shí)顯示為低密度軟組織占位，增強(qiáng)掃描示腫塊邊界清晰，11例[7, 11, 13, 18, 27-29]內(nèi)部呈不均勻強(qiáng)化，7例呈低密度灶[9-10, 12, 16-17, 21, 24]；2例胰腺?gòu)浡暂p度強(qiáng)化[10,19]。8例行腹部B超，其中6例呈低回聲占位[7, 16-18, 27-28]，2例胰腺?gòu)浡栽龃?，回聲輕度增強(qiáng)[10,19]。5例行內(nèi)鏡超聲(EUS)，顯示腫塊輪廓清晰，內(nèi)部呈低回聲，邊緣呈高回聲[13,18]。4例行上腹部磁共振掃描，僅2例詳細(xì)描述特征，病灶在T1加權(quán)呈低信號(hào)，T2加權(quán)呈高信號(hào)。2例行選擇性血管造影，提示病灶血管豐富或腫瘤濃染[17-18]。2例行ERCP，顯示肝內(nèi)外膽管及膽總管擴(kuò)張[12,17]。2例行正電子發(fā)射斷層顯像-計(jì)算機(jī)斷層掃描(PET)檢查，顯示高代謝病灶[13,24]。1例采用生長(zhǎng)抑素受體閃爍成像檢測(cè)轉(zhuǎn)移灶[20]。

五、組織病理學(xué)及免疫組化特征

26例通過手術(shù)、9例通過B超及CT或EUS引導(dǎo)下穿刺活檢[4, 9, 19，13, 24]、1例通過腹腔鏡下胰體和淋巴結(jié)活檢、2例通過ERCP活檢[11-12]、9例通過尸檢[3-4, 10, 14]獲得組織標(biāo)本。光鏡下見癌細(xì)胞體積較小，胞質(zhì)稀少或呈裸核狀，核呈圓形或短梭形，深染，核仁不明顯，細(xì)胞界限不清，彌漫排列成片或呈巢團(tuán)狀，并可見大片壞死，部分可見菊形團(tuán)樣結(jié)構(gòu)。44例為單一小細(xì)胞型(圖1a)，3例為小細(xì)胞癌-腺癌混合型[8,15]。

18例有詳細(xì)的免疫組化結(jié)果[13,15-22,24,28-29]，突觸融素(synaptophysin, Syn)陽性率100%(8/8，圖1b)，嗜鉻蛋白A (chromogranin A, CgA)陽性率90%(9/10)，NSE陽性率77.8%(7/9)，角蛋白陽性率100%(5/5)，CA19-9陽性率10% (1/10)，CEA陽性率20% (2/10)，CD20陰性(0/10)。

圖1原發(fā)性胰腺小細(xì)胞癌的病理學(xué)改變(a HE×100)及Syn蛋白表達(dá)(b 免疫組化×100)

六、治療方式及預(yù)后

單純手術(shù)治療6例，單純化療9例，手術(shù)聯(lián)合化療16例，手術(shù)聯(lián)合放療1例，放療聯(lián)合化療3例，手術(shù)聯(lián)合放化療3例，對(duì)癥處理9例。常用化療方案為鉑類+吉西他濱，其他如5-FU、表阿霉素、干擾素及生長(zhǎng)抑素等也有應(yīng)用。

43例患者記錄了詳細(xì)的生存時(shí)間。截止文獻(xiàn)報(bào)道之日，有5例患者存活。患者生存2～692周，中位生存期28周(圖2a)，局限期和進(jìn)展期患者的中位生存期分別為60周和16周(圖2b)，兩組差異有統(tǒng)計(jì)學(xué)意義(χ2=5.90，P=0.015)。單純手術(shù)組的中位生存期為24周，單純化療組為200周，手術(shù)聯(lián)合化療組為24周，手術(shù)聯(lián)合放療組為224周，化療聯(lián)合放療組為16周，手術(shù)聯(lián)合放化療的1例生存期為692周，對(duì)癥處理組為4周。

討 論

原發(fā)性胰腺小細(xì)胞癌又稱低分化內(nèi)分泌癌、高度惡性神經(jīng)內(nèi)分泌癌，是胰腺神經(jīng)內(nèi)分泌癌的一種。目前對(duì)其組織來源及發(fā)生機(jī)制尚未明了。傳統(tǒng)認(rèn)為胰腺神經(jīng)內(nèi)分泌癌起源于胰島細(xì)胞，而目前學(xué)者則傾向于多能干細(xì)胞學(xué)說，即多極分化能力的胰腺導(dǎo)管細(xì)胞在致癌因素作用下既可轉(zhuǎn)變?yōu)樾〖?xì)胞癌,也可轉(zhuǎn)變?yōu)橄侔31]。本研究中3例混合型癌的免疫組化顯示角蛋白高表達(dá)，似乎支持多能干細(xì)胞分化學(xué)說。近期有學(xué)者已建立胰腺小細(xì)胞癌的穩(wěn)定細(xì)胞株[32]，這將有助于對(duì)其分子生物學(xué)和遺傳學(xué)特性的深入研究。

圖2原發(fā)性胰腺小細(xì)胞癌患者總的生存曲線(a)及不同分期患者的生存曲線(b)

原發(fā)性胰腺小細(xì)胞癌好發(fā)于中老年男性，病灶多為單發(fā)，可發(fā)生于胰腺各個(gè)部位，以胰頭部多見。臨床常表現(xiàn)為腹痛、黃疸及體重下降，而反映神經(jīng)內(nèi)分泌功能的臨床特征較為少見，且多為無功能性，故早期診斷極為困難。CEA、CA19-9等胰腺腫瘤標(biāo)記物多為正常，而反映神經(jīng)內(nèi)分泌功能的標(biāo)記物例如NSE表達(dá)率較高，可作為療效觀察的指標(biāo)之一[11, 17]。因此，對(duì)臨床上CA19-9正常的胰腺占位需考慮該癥可能，聯(lián)合血清NSE檢查可協(xié)助診斷。

CT是最常用的診斷原發(fā)性胰腺小細(xì)胞癌的手段之一。CT征象包括：(1)平掃時(shí)腫瘤密度比較均勻，亦可發(fā)生囊變壞死；(2)腫瘤強(qiáng)化程度高于胰腺導(dǎo)管腺癌；(3)發(fā)生肝轉(zhuǎn)移的概率相對(duì)較高,且亦具有富血供特點(diǎn)。但CT征象無法與其他富血供腫瘤例如胰島細(xì)胞瘤、類癌、腺泡細(xì)胞癌等鑒別。EUS的優(yōu)勢(shì)在于增加胰腺小病灶檢出率及進(jìn)行病灶細(xì)針穿刺獲取組織有助于定性診斷。近年來，生長(zhǎng)抑素受體閃爍成像是根據(jù)內(nèi)分泌腫瘤的生物學(xué)特性開發(fā)的成像技術(shù)，可顯示其他常規(guī)影像學(xué)檢查無法檢測(cè)的小病灶和轉(zhuǎn)移灶[33]，可作為胰腺小細(xì)胞癌分期及預(yù)后評(píng)估的首選方法[20]。其他一些檢查手段例如選擇性血管造影、PET及MRI也可用于胰腺小細(xì)胞癌的定位診斷。

原發(fā)性胰腺小細(xì)胞癌確診主要依靠病理檢查。神經(jīng)內(nèi)分泌腫瘤標(biāo)記物Syn、CgA及NSE陽性率較高，而CA19-9、CEA以及CD20等多為陰性。值得注意的是，原發(fā)性胰腺小細(xì)胞癌發(fā)生率低，故確診該病首先須排除其他部位的小細(xì)胞癌的轉(zhuǎn)移灶。

原發(fā)性胰腺小細(xì)胞癌局限期患者的中位生存期顯著長(zhǎng)于進(jìn)展期患者，不同治療方案組患者中位生存期也有差別。本研究納入的均屬個(gè)案報(bào)道，存在選擇性偏倚，故不能得出最佳的治療模式。有學(xué)者認(rèn)為肺外小細(xì)胞癌的組織學(xué)表現(xiàn)、基因改變和高度侵襲性的生物學(xué)特點(diǎn)與小細(xì)胞肺癌相似，故強(qiáng)調(diào)了化療在胰腺小細(xì)胞癌治療中的地位[34]。目前常用的化療方案以鉑類和健擇為主。部分學(xué)者認(rèn)為手術(shù)不僅可切除病灶，而且可延長(zhǎng)患者的無瘤生存時(shí)間[35]。部分學(xué)者則認(rèn)為因局限期患者存在微小轉(zhuǎn)移，手術(shù)可導(dǎo)致癌細(xì)胞的擴(kuò)散，加速腫瘤進(jìn)展，從而強(qiáng)調(diào)以化療為基礎(chǔ)的全身系統(tǒng)治療[36]。本組采用手術(shù)聯(lián)合放化療的1例生存時(shí)間長(zhǎng)達(dá)10余年。因此，三者聯(lián)合的綜合治療不失為一種可選擇的療法。其他如采用生長(zhǎng)抑素受體介導(dǎo)的靶向治療也有一定療效。

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Systematicreviewof47casesofprimarysmallcellcarcinomaofthepancreas

PENGChun-yan,LüYing,YAORen-ling,XUZhao-min,ZOUXiao-ping.

DepartmentofGastroenterology,DrumTowerHospital,MedicalSchool,NanjingUniversity,Nanjing210008,China

Correspondingauthor:ZOUXiao-ping,Email:zouxiaoping795@hotmail.com

ObjectiveTo investigate the clinicopathologic features, therapy, and prognosis of primary small cell carcinoma of the pancreas.MethodsDatabases including Chinese Journal Full-text Database, VIP Database for Chinese Technical Periodicals, Medline/Pubmed, and OVID were searched electronically up to April 2012. A systematic review was performed together with one case in our hospital.ResultsTwenty-eight articles fulfilling the criteria consisting of 46 patients with pathologically confirmed diagnosis of primary small cell carcinoma of the pancreas were studied, together with 1 patient in our Drum Tower Hospital, finally 47 patients were included. The results of this systematic review showed: (1)Primary small cell carcinoma of the pancreas was more common in men with a median age of 62. The most common clinical presentations were abdominal pain, jaundice and weight loss. Para-neoplastic syndrome was rarely observed. (2)Most cases were found to have abnormally elevated serum levels of neuron-specific enolase. CT displayed heterogeneous, and marked enhancing masses in most cases. The conclusive diagnosis depended on histological confirmation. (3)63.8% of the cases were found to be associated with metastasis at the time of diagnosis. The overall median survival time was 28 weeks. (4) There was no consensus on the treatment of primary small cell carcinoma of the pancreas. Chemotherapy was currently considered as the treatment of choice among the systematic management for these patients.ConclusionsPrimary small cell carcinoma of the pancreas was a rare and aggressive neuroendocrine tumor with a poor prognosis.

Neuroendorine tumors; Carcinoma, small cell； Pancreas； Meta-analysis

10.3760/cma.j.issn.1674-1935.2012.04.003

210008 南京，南京大學(xué)醫(yī)學(xué)院附屬鼓樓醫(yī)院消化科

鄒曉平，Email:zouxiaoping795@hotmail.com

2012-04-27)

(本文編輯：呂芳萍)