馬豫茜+李國華+樊淑梅


[摘要] 目的 分析消化道顆粒細胞瘤(GCT)患者的臨床特征,對治療效果進行分析。方法 回顧分析 2012年 12月~2016年10月在我院住院的5 例消化道 GCT患者的臨床資料,分析其臨床特征,治療效果。結果5 例消化道 GCT 患者中,食管 GCT4 例(其中1例合并早期食管癌),盲腸 GCT 1例,所有患者均表現為非特異性消化道癥狀或無消化道癥狀,均行術前內鏡及超聲內鏡檢查診斷為消化道黏膜下隆起性病變,分別疑診脂肪瘤、 類癌及異位胰腺。1例術前考慮消化道 GCT,5例患者術前均未活檢,4例行內鏡下黏膜剝離術(ESD),1例行單環套扎+圈套器切除,術后病理及免疫組化分析顯示腫瘤細胞均表達 S-100,確診為消化道 GCT,未見明顯惡性特征,術后隨訪3~48個月,均無復發及轉移。結論 消化道GCT 可無特異性消化道癥狀、發病率低,內鏡下亦無特異性表現,因此明確診斷仍依賴于病理結果,對于<2cm 的黏膜下 GCT,內鏡下治療效果好,但需長期隨訪,排除惡變可能。
[關鍵詞] 消化道;顆粒細胞瘤;特點
[中圖分類號] R735 [文獻標識碼] A [文章編號] 2095-0616(2017)06-154-04
[Abstract] Objective To explore the clinical characteristicas of granular cell tuomr, and to analyze the curative effect. Methods Data of 5 cases of GCT patients cured in our hospital from December 2012 to October 2016 were retrospectively analyzed. The clinical characteristics and therapeutic effect were analyzed. Results Among the 5 cases of GCT patients, there were GCT4 cases of esophagus(1 cases with early esophageal cancer) and GCT of cecum in 1 cases. All patients presented with nonspecific gastrointestinal symptoms or no gastrointestinal symptoms. All cases were diagnosed by endoscopic and endoscopic ultrasonography, and they respectively were lipoma, carcinoid and ectopic pancreas. 1 cases of gastrointestinal tract GCT were considered before operation, and all of the 5 patients had no biopsy before operation, and endoscopic mucosal resection(ESD) was performed in all of the 4 patients, and the other cases were treated with single loop ligation and snare excision(n=1). Postoperative pathological and immunohistochemical analysis showed that the tumor cells all expressed S-100, which was diagnosed as digestive tract GCT, and there was no obvious malignant feature. No recurrence and metastasis were observed after 3-48 months of follow-up. Conclusion There are no specific gastrointestinal symptoms and low incidence of GCT in the digestive tract. Therefore, the definite diagnosis depends on pathological results. For less than 2cm of submucosal GCT, endoscopic treatment effect is good, but need long-term follow-up, exclude the possibility of malignancy.
[Key words] Digestive tract; Granular cell tumor; Characteristic
顆粒細胞瘤(granular cell tumor,GCT)是一種來源于外周神經,具分化的有雪旺細胞(Schwanns cell)分化特點的罕見軟組織腫瘤,免疫組化顯示顆粒細胞瘤細胞主要表達S-100蛋白,1926年由Abrikossof首次報道[1],臨床發生率低,任何年齡均可發病,但以30~60歲多見[2],可發生于身體任何部位,一般好發于口腔、頭頸部、軀干和四肢及外陰的皮膚或皮下軟組織,而消化道僅占8%,其中食管占2%[3-4],下段>中段>上段,發生于胃、小腸和膽道的少見,發生于盲腸者及食管GCT合并早期食管癌者更加罕見,目前該病多為個案報道。我們對5例消化道GCTs的臨床、內鏡特點及內鏡下治療效果進行研究、總結,分析其特點及治療效果,現報道如下。
1 資料與方法
1.1 一般資料
2012年12月~2016年10月在佛山市順德區第一人民醫院住院的5例消化道GCTs患者,男3例,女2例,年齡31~64歲,平均49.5歲。
1.2 方法
回顧性分析5例消化道GCTs患者的臨床資料:一般資料、臨床表現、內鏡表現、超聲內鏡表現、病理及免疫組化結果、治療方法及預后,分析臨床特點及治療效果。
2 結果
2.1 5例消化道GCT患者臨床表現
消化系統顆粒細胞瘤患者臨床癥狀以腹部不適、吞咽不暢、胸骨后不適、燒心,見表1,圖1。
2.2 5例消化道GCT患者超聲內鏡表現
5例位于黏膜下層,呈低回聲改變,均未累及肌層,界限清楚,見表2,圖1~2。
2.3 5例消化道GCTs治療方法
4例行ESD治療,1例行單環黏膜套扎+圈套器切除術,1例出血術中穿孔,予以鈦夾夾閉傷口,2例創面少量滲血,予以電凝止血后無活動性出血,5例均完整切除,送病理學檢查,術后恢復良好,見表3,圖3。
2.4 病理及免疫組化結果
術后病理HE(圖4)腫瘤細胞排列緊密,呈巢狀或條索狀,細胞大小一致,胞質豐富有大量嗜酸性顆粒細胞,核小、圓而居中。免疫組化(圖5)腫瘤細胞S-100均呈彌漫性強陽性。
2.5 術后隨訪
5例患者其中4例隨訪1~2年,復查胃鏡及腸鏡未見腫瘤復發。1例顆粒細胞瘤并淺表食管癌患者(內鏡治療)術后3個月隨訪1次,復查食管黏膜未見復發。
3 討論
消化道顆粒細胞瘤可發生在食管、胃、小腸、大腸、肛管、甚至膽管、胰腺等部位[5-10]。其中食管發生比例最高,盲腸相對較少,食管顆粒細胞瘤合并覆蓋黏膜鱗狀細胞癌的,罕見報道。本組5例消化道GCT,1例發生于盲腸,1例食管顆粒細胞瘤合并被覆上皮早期鱗癌。
消化道GCT無特異性癥狀,多表現為腹部不適、胸骨后不適,吞咽不暢等消化道癥狀,根據腫瘤大小及部位的不同而癥狀各異。部分患者為體檢時意外發現,本研究5例患者的臨床表現列表可以看出,GCT可無任何癥狀或僅有非特異性的消化道癥狀。除此之外,消化道GCT可與其他疾病并存,甚至多部位腫瘤。有臨床發現,食管GCT與食管鱗狀細胞癌并存[11],這與我們研究發現的其中1例病例符合。因此,我們在內鏡檢查過程中,不但要觀察隆起型黏膜下病變,其覆蓋表面上皮或臨近組織也應仔細觀察,避免多發腫瘤的漏診。
消化道顆粒細胞瘤的內鏡表現具有一定特征性[12]。主要為:(1)白光鏡下表現為表面光滑的淡黃色或灰白色隆起,邊界清晰;(2)超聲內鏡表現為來源與黏膜下層或黏膜肌層為主的低回聲病變,病變邊界清晰,內部回聲均勻,隨著診斷技術的發展,超聲內鏡對GCT的診斷率有所提高[13],但單純內鏡檢查仍無法與間質瘤、類癌及脂肪瘤等鑒別。臨床診斷最終依靠病理及免疫組化[14],診斷標準:(1)瘤細胞胞漿豐富,嗜酸性顆粒狀,排列成索狀或巢狀;(2)免疫組化S-100表達陽性。
消化道GCTs以良性居多,且以黏膜下層為主,適合內鏡下EMR、ESD黏膜套扎切除等治療。我組研究5例病例采用ESD及單環套扎+切除術治療GCTs均未出現出血、穿孔、腹膜炎或縱膈感染等并發癥,患者恢復良好,且經復查無復發或轉移等并發癥,因此,內鏡治療不失為臨床值得應用及推廣的微創治療方法,但GCT仍有惡變可能,通過淋巴及血道轉移至肝、肺、骨甚至胰腺,導致死亡[15],因此應強調術后復查的重要性。
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(收稿日期:2017-02-04)