任省身 周聰強(qiáng) 張旭△
1中國人民解放軍總醫(yī)院泌尿外科 100853 北京
綜 述
腹腔副神經(jīng)節(jié)瘤的診治現(xiàn)狀
任省身1周聰強(qiáng)1張旭1△
1中國人民解放軍總醫(yī)院泌尿外科 100853 北京
△審校者
副神經(jīng)節(jié)瘤是一種臨床上罕見的神經(jīng)內(nèi)分泌腫瘤,病因尚未明確,最常出現(xiàn)于腹部和盆腔,多數(shù)為良性病變,約33%的病灶呈惡性?,F(xiàn)針對(duì)發(fā)生于腹腔的副神經(jīng)節(jié)瘤,系統(tǒng)復(fù)習(xí)近些年國內(nèi)外相關(guān)文獻(xiàn)和最新研究,對(duì)當(dāng)前其不同的診斷和治療方法進(jìn)行綜述。
腹腔;副神經(jīng)節(jié)瘤;診斷;治療
副神經(jīng)節(jié)瘤(paragangliomas,PGL)是一種罕見的神經(jīng)內(nèi)分泌腫瘤,關(guān)于PGL的確切定義尚未有定論,目前相對(duì)統(tǒng)一的觀點(diǎn)是將嗜鉻細(xì)胞瘤特指為腎上腺嗜鉻細(xì)胞瘤(pheochromocytoma,PHEO),而將傳統(tǒng)觀念中腎上腺外或異位嗜鉻細(xì)胞瘤統(tǒng)稱為PGL[1]。PGL既可以來源于腹腔、盆腔等部位的交感神經(jīng)[2],也可以源于頭頸部的副交感神經(jīng)[3],本文就腹腔PGL的診斷及最新治療進(jìn)展進(jìn)行論述。
PGL在人群中的發(fā)病率為0.01‰~0.03‰,男女發(fā)病率無顯著差異,可發(fā)生于任何年齡,多在35~50歲[4]。關(guān)于PGL的病因目前尚不明確,有研究認(rèn)為其與遺傳相關(guān),是一種多基因遺傳病[5,6],攜帶下述基因型的人群更易罹患該?。憾喟l(fā)性內(nèi)分泌腺瘤 2A型(MEN-2A)和2B型(MEN-2B)、Von Hippel-Lindau綜合征(VHL)、神經(jīng)纖維瘤病1型(NF-1)等。另有研究發(fā)現(xiàn)線粒體琥珀酸脫氫酶B亞基基因(SDHx,包括SDHA、SDHB、SDHC、SDHD)的突變與PGL的發(fā)生有著密切聯(lián)系[7],因此,許多學(xué)者建議PGL患者及其一級(jí)親屬均應(yīng)進(jìn)行相關(guān)的基因檢測,對(duì)疾病的診斷和治療大有裨益。
95%以上的PGL發(fā)生于腹部和盆腔,以腹主動(dòng)脈旁和腎門周圍居多,來源于頭頸及胸腔縱膈等部位的PGL不足5%[8],前者多具有兒茶酚胺功能活性,而后者罕見兒茶酚胺分泌。PGL多數(shù)為良性病變,惡性者約占33%,兒童患者中有26%~35%為惡性,在某些攜帶特殊變異基因(如SDHB)的患者中惡性率更高[9]。
功能性PGL患者的癥狀和體征主要是由于嗜鉻細(xì)胞分泌的兒茶酚胺(包括腎上腺素、去甲腎上腺素和多巴胺)進(jìn)入外周循環(huán)引起,典型癥狀包括陣發(fā)性高血壓、心動(dòng)過速、發(fā)汗、頭痛、焦慮和驚恐發(fā)作[10]。高血壓為PGL患者最常出現(xiàn)的癥狀,發(fā)生率超過80%,在陣發(fā)性或頑固性高血壓的前提下出現(xiàn)不明原因的體位性低血壓,往往會(huì)給臨床醫(yī)師提供診斷線索[11]。除此之外,少數(shù)患者可能出現(xiàn)嚴(yán)重的腦功能紊亂[12]、擴(kuò)張性心肌病[13]、心律失常[13]、視力下降[14]等癥狀。非功能性PGL患者常常由于緩慢生長的腫塊壓迫周圍組織器官才出現(xiàn)癥狀[15],如壓迫輸尿管導(dǎo)致腎積水、壓迫小腸導(dǎo)致腸梗阻,抑或單純出現(xiàn)腹部腫塊。另外,有10%~20%的家族性PGL患者以家族性遺傳病為主要臨床表現(xiàn),如MEN-2A、MEN-2B、VHL等[16]。
鑒于PGL有著多變的臨床和影像學(xué)表現(xiàn),因此準(zhǔn)確地診斷腹腔PGL往往有一定的難度,需要有經(jīng)驗(yàn)的醫(yī)師結(jié)合病史、實(shí)驗(yàn)室檢查、影像學(xué)資料,甚至基因檢測對(duì)患者進(jìn)行綜合分析做出判斷。
2.1 臨床診斷
有下述可疑病史者應(yīng)考慮PGL的可能[15,17,18]:①高血壓伴有頭痛、心悸、多汗者(PGL經(jīng)典“三聯(lián)征”);②頑固性或不穩(wěn)定性高血壓患者;③無法解釋的陣發(fā)性高血壓患者;④特發(fā)性擴(kuò)張性心肌病患者;⑤家族有多發(fā)性內(nèi)分泌腫瘤遺傳背景者;⑥手術(shù)、麻醉或侵入性檢查過程中出現(xiàn)血壓劇烈波動(dòng)者;⑦偶發(fā)的腎上腺或腹部腫塊者;⑧對(duì)麻醉藥、抗抑郁藥、胰高血糖素等藥物出現(xiàn)與藥效相反的患者。
2.2 生化診斷
大多數(shù)功能性PGL患者會(huì)分泌大量的兒茶酚胺,因此,診斷PGL很大程度上依賴患者體內(nèi)產(chǎn)生的兒茶酚胺水平,對(duì)可疑病例均應(yīng)進(jìn)行兒茶酚胺及其代謝產(chǎn)物的測定。24 h尿兒茶酚胺或尿香草基扁桃酸(VMA,一種兒茶酚胺代謝產(chǎn)物)含量測定是目前為止應(yīng)用最多的檢查手段[19],但是這兩種檢查均會(huì)造成較高的假陰性率,分別為14%[14]和41%[20]。為了避免假陰性結(jié)果帶來的漏診,有些醫(yī)療單位還會(huì)對(duì)高危人群的血漿游離甲氧基腎上腺素類物質(zhì)(metanephrines,MNs)和24 h尿分餾MNs進(jìn)行檢測[21]。MNs是兒茶酚胺在體內(nèi)的代謝中間產(chǎn)物,包括甲氧基腎上腺素(MN)和甲氧基去甲腎上腺素(NMN),二者以持續(xù)“漏出”的方式釋放入血[21]。與傳統(tǒng)方法相比,檢測MNs會(huì)獲得更高的特異性,分別為82%~96%和96%~98%[22]。除此之外,測定血漿兒茶酚胺和24 h尿總MNs含量亦可作為PGL的診斷措施。值得一提的是,對(duì)于非功能性PGL患者,上述檢查幾乎不出現(xiàn)特征性異常。
2.3 影像診斷
對(duì)于功能性與非功能性腹腔PGL患者均適用,常用措施包括超聲檢查、計(jì)算機(jī)體層成像(CT)和磁共振成像(MRI),部分有條件的醫(yī)療機(jī)構(gòu)還可進(jìn)行間碘芐胍(MIBG)顯像、奧曲肽顯像、PET顯像等手段[23]。
由于腹腔PGL組織來源廣泛,超聲檢查往往很難獲得特異性的聲像圖,對(duì)于腹部隱痛患者或偶發(fā)腹部包塊者,超聲可以作為一種輔助性診斷措施。另外,超聲引導(dǎo)下的穿刺活檢也會(huì)為診斷提供幫助。CT檢查是PGL的首選影像學(xué)診斷方法,可發(fā)現(xiàn)腎上腺外1.0 cm以上的結(jié)節(jié),平掃顯示瘤體呈等密度或低密度灶,伴鈣化者密度增高,增強(qiáng)掃描發(fā)現(xiàn)實(shí)性腫塊常表現(xiàn)出明顯均質(zhì)強(qiáng)化,囊性腫塊強(qiáng)化不明顯,體積較大的病灶多伴隨出血和壞死[24~26]。MRI與CT相比,二者具有相似的靈敏度(90%~100%)和特異性(70%~80%)[27],可用于不適合接受大劑量放射者、對(duì)造影劑過敏者或CT掃描陰性者[23]。
123I-MIBG對(duì)PGL的解剖及功能定位有著良好的特異性(95%~100%),131I-MIBG與之相比特異性稍差,二者對(duì)檢測多灶性或轉(zhuǎn)移性腫瘤以及患者的術(shù)后隨訪可發(fā)揮不容忽視的作用[28,29]。奧曲肽作為生長抑素類似物,經(jīng)131I或111In標(biāo)記后可與生長抑素受體2、5型(SSTR2、SSTR5)穩(wěn)固結(jié)合,從而用于神經(jīng)內(nèi)分泌型腫瘤的診斷[30]。由于MIBG檢查方法已十分成熟,加上111In-DTPA-奧曲肽顯像與之相比敏感性也略低,因此限制了其在臨床上的廣泛應(yīng)用[31]。
2.4 基因診斷
PGL是一種具有遺傳傾向的疾病,因此,推薦對(duì)PGL家族史的患者及家庭成員進(jìn)行相關(guān)的基因檢測。Neumann等[32]通過對(duì)334例胸腔、腹腔PGL患者進(jìn)行基因檢測,發(fā)現(xiàn)SDHD突變基因攜帶者更易出現(xiàn)多灶性腫瘤,而具有SDHB突變基因的PGL患者則更易進(jìn)展為惡性腫瘤,相似的結(jié)論在Gimenez-Roqueplo等[33]、Persu等[7]的研究成果中也得到了印證。除了SDHB、SDHD外,RET和VHL也是推薦納入基因檢測的指標(biāo)。為了主動(dòng)監(jiān)測PGL患者的復(fù)發(fā)情況、其他器官受累情況與其家屬潛在的發(fā)病情況,國內(nèi)外許多學(xué)者建議對(duì)所有PGL患者均行常規(guī)基因檢測。
開放手術(shù)和腹腔鏡下腫物切除是目前治療腹腔PGL的最常用的手術(shù)方法,近些年,隨著微創(chuàng)外科的進(jìn)步,機(jī)器人輔助腹腔鏡腫瘤切除也越來越多地用于此類患者的治療[34]。鑒于PGL患者多變的臨床表現(xiàn),尤其是心血管癥狀,完善的術(shù)前準(zhǔn)備是手術(shù)能否成功的關(guān)鍵。
3.1 術(shù)前準(zhǔn)備
術(shù)前高血壓控制情況不理想可能使腹腔PGL患者在全身麻醉或腫瘤摘除過程中出現(xiàn)高血壓危象,嚴(yán)重者危及生命[35]。因此,術(shù)前合理降壓顯得尤為重要。最常用的降壓藥為α-腎上腺素受體阻滯劑,術(shù)前7~21 d開始使用,從小劑量開始,逐漸增加劑量,當(dāng)患者出現(xiàn)心動(dòng)過速或直立性低血壓時(shí)提示劑量恰當(dāng),可以開始給予β-受體阻滯劑[4,36]。酚芐明是最常用的α-受體阻滯劑,同時(shí),Weingarten等[37]證實(shí)選擇性α1-受體阻滯劑亦可用于PGL患者的術(shù)前降壓。在單用α-受體阻滯劑降壓效果不明顯的情況下,鈣離子通道阻滯劑(calcium channel blocker,CCB)可以作為一種輔助降壓措施。PGL手術(shù)死亡率曾高達(dá)30%~45%,但近年研究表明,經(jīng)充分術(shù)前準(zhǔn)備后,PGL患者的手術(shù)死亡率已下降到0~2.9%[38]。
3.2 手術(shù)治療
腹腔鏡下腫瘤切除是治療PHEO的標(biāo)準(zhǔn)術(shù)式,與PHEO相比,腹腔PGL患者潛在的惡性程度更高,且更易在腹主動(dòng)脈旁、腸系膜下動(dòng)脈周圍及膀胱等部位出現(xiàn)多發(fā)性結(jié)節(jié),因此開放手術(shù)可以為術(shù)者提供更好的視野和操作空間[39]。對(duì)于腫瘤病灶較少的患者,有經(jīng)驗(yàn)的醫(yī)師可以在充分的術(shù)前評(píng)估和準(zhǔn)備后選擇腹腔鏡或機(jī)器人輔助腹腔鏡的手術(shù)方式。惡性PGL通常會(huì)和周圍脈管之間形成緊密的纖維聯(lián)結(jié),增大了手術(shù)切除的難度[40],在術(shù)前或術(shù)中若發(fā)現(xiàn)有區(qū)域淋巴結(jié)受累,應(yīng)一并切除受累的淋巴結(jié)[41]。對(duì)于無法施行手術(shù)的患者,可以施以131I-MIBG為代表的放射性核素治療[42]或CVD方案(環(huán)磷酰胺、長春新堿/阿霉素、氮烯唑胺)為代表的化療[43]。由于PGL病例的稀缺性,尚未檢索到該類患者接受化療的高質(zhì)量隨機(jī)對(duì)照研究和系統(tǒng)隨訪資料[39]。
3.3 其他治療
除了上述的療法外,還有針對(duì)不同的患者進(jìn)行的個(gè)體化治療,比如對(duì)攜帶SDHB變異基因的患者施以舒尼替尼(sunitinib)和帕唑帕尼(pazopanib)為代表的分子靶向治療[44,45],對(duì)PGL骨轉(zhuǎn)移患者施以二膦酸鹽(bisphosphonates)和狄諾塞麥(denosumab)為代表的抗骨吸收治療[46]和以減輕患者疼痛為目的的放療[47]。
3.4 術(shù)后隨訪
盡管手術(shù)切除良性PHEO/PGL病灶效果顯著,但是有研究指出術(shù)后仍有大約50%的患者持續(xù)出現(xiàn)高血壓[41]。Amar等[48]通過對(duì)192例PHEO/PGL患者調(diào)查發(fā)現(xiàn),PGL患者術(shù)后腫瘤復(fù)發(fā)率為PHEO患者的11.2倍,且復(fù)發(fā)情況與家族性遺傳史緊密相關(guān)。因此,對(duì)有家族性病史者應(yīng)于術(shù)后終生隨訪,隨訪內(nèi)容包括臨床癥狀、生化檢查和影像學(xué)檢查等。
綜上所述,腹腔PGL作為一種罕見的家族性遺傳病,近年來得到了越來越多的關(guān)注,其診斷和治療方法也在不斷地進(jìn)步。對(duì)PGL患者的診斷既要重視其臨床表現(xiàn),更要結(jié)合實(shí)驗(yàn)室和影像學(xué)檢查進(jìn)行綜合判斷,必要時(shí)應(yīng)行基因檢測。手術(shù)是治療腹腔PGL的首選措施,根據(jù)患者情況及術(shù)者經(jīng)驗(yàn)可酌情選擇開放手術(shù)或腹腔鏡手術(shù)。對(duì)于失去手術(shù)機(jī)會(huì)的患者,可以給予放療、化療、分子靶向治療等姑息療法,由于缺乏大樣本隨機(jī)對(duì)照研究,因此很難決定上述哪項(xiàng)療法應(yīng)作為一線治療措施。隨著精準(zhǔn)醫(yī)學(xué)的興起,針對(duì)不同患者的個(gè)體化治療越來越受到重視。對(duì)于高復(fù)發(fā)和惡變風(fēng)險(xiǎn)的患者,術(shù)后應(yīng)積極進(jìn)行長期隨訪。
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Current status of diagnosis and treatment for abdominal paragangliomas
RenXingshen1ZhouCongqiang1ZhangXu1
(1Department of Urology,Chinese PLA General Hospital,Beijing 100853,China)
Zhang Xu,xzhang@foxmail.com
Paragangliomas(PGL) is a rare neuroendocrine tumor in clinic,and the etiology of PGL is not clear at present.PGL is most likely to occur in abdomen and pelvis,and the majority of tumors are benign with the malignant incidence of 33% approximately.In this study,we systematically reviewed relative and updated literatures about abdominal PGL in recent years,and discussed the diagnosis and treatment methods comprehensively.
abdominal; paragangliomas; diagnosis; treatment
張旭,xzhang@foxmail.com
2017-05-10
R737
A
10.19558/j.cnki.10-1020/r.2017.03.014