邵 宇,譚云波
(大理大學(xué)附屬醫(yī)院,云南大理 671000)
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脾竇岸細(xì)胞血管瘤1例并文獻(xiàn)復(fù)習(xí)
邵宇,譚云波*
(大理大學(xué)附屬醫(yī)院,云南大理671000)
[摘要]目的:探討脾竇岸細(xì)胞血管瘤的臨床病理特點(diǎn)、診斷與鑒別診斷要點(diǎn)。方法:對(duì)1例LCA進(jìn)行臨床資料、影像學(xué)特征、病理形態(tài)學(xué)及免疫組化分析并復(fù)習(xí)有關(guān)文獻(xiàn)。結(jié)果:腫瘤由竇樣腔隙構(gòu)成,內(nèi)皮細(xì)胞大多扁平,但腔壁上有少量乳頭狀結(jié)構(gòu)凸向管腔,血管間含多少不一的纖維組織。國(guó)外文獻(xiàn)報(bào)道的病例中LCA常合并惡性腫瘤。結(jié)論:LCA是罕見的來源于脾竇岸細(xì)胞的血管源性腫瘤,其診斷主要依靠病理組織學(xué)及免疫組化。LCA與惡性腫瘤相關(guān),應(yīng)密切觀察和長(zhǎng)期隨訪。
[關(guān)鍵詞]脾;竇岸細(xì)胞;血管瘤;免疫組化
[DOI]10. 3969 / j. issn. 2096-2266. 2016. 04. 014
脾竇岸細(xì)胞血管瘤(LCA)是一種少見的、特發(fā)于脾臟的血管源性腫瘤,由于腫瘤細(xì)胞的來源為脾竇內(nèi)皮細(xì)胞,即竇岸細(xì)胞,故命名為脾竇岸細(xì)胞血管瘤。由于脾竇岸細(xì)胞血管瘤發(fā)病率低,至今為止,國(guó)內(nèi)外僅有少量個(gè)案報(bào)道。本文結(jié)合2015年收治的1例與相關(guān)文獻(xiàn)報(bào)道對(duì)其臨床診治、影像學(xué)特征、病理特點(diǎn)進(jìn)行相關(guān)探討。
患者女,53歲,2015年7月6日因“發(fā)現(xiàn)雙側(cè)甲狀腺包塊1年余”入院,入院后完善相關(guān)檢查,頸部超聲提示:①右側(cè)甲狀腺腫大聲像。②雙側(cè)甲狀腺多發(fā)結(jié)節(jié)樣病灶,TI-RASD分類:4A類。腹部彩超提示:脾內(nèi)多發(fā)無(wú)回聲區(qū)聲像(性質(zhì)待查),后轉(zhuǎn)至大理大學(xué)附屬醫(yī)院外一科治療?;颊呒韧鶡o(wú)手術(shù)史、無(wú)肝炎、結(jié)核等傳染病史。查體:腹部平坦、腹壁柔軟、無(wú)壓痛、無(wú)反跳痛、無(wú)肌緊張。肝、脾、膽囊未觸及。移動(dòng)性濁音陰性,腸鳴音4次/min。血常規(guī)、血生化、凝血功能未見明顯異常。腹部CT平掃+增強(qiáng)提示:考慮多發(fā)脈管瘤。后于全麻下行腹腔鏡下脾切除術(shù),手術(shù)順利。脾臟大小10 cm×7 cm×3 cm,實(shí)性,質(zhì)軟,表面見散在分布的結(jié)節(jié)狀灰紅色腫物,直徑0.5~1.5 cm。切除脾臟送病理檢查。術(shù)后患者恢復(fù)良好,I/甲愈合,囑定期復(fù)診。
行腹部CT平掃+增強(qiáng),見脾實(shí)質(zhì)內(nèi)多發(fā)大小不等密度減低影像,增強(qiáng)性掃描動(dòng)脈期大多數(shù)病灶邊界清晰,邊緣有強(qiáng)化,靜脈期部分病灶有造影劑填充。見圖1。
圖1 CT平掃+增強(qiáng)
大體:大小10 cm×7 cm×3 cm,皮髓質(zhì)分界清楚,切面暗紅色,實(shí)性,質(zhì)軟,表面見散在分布的結(jié)節(jié)狀灰紅色腫物,直徑0.5~1.5 cm。鏡檢:腫瘤由竇樣腔隙構(gòu)成,內(nèi)皮細(xì)胞大多扁平,但腔壁上有少量乳頭狀結(jié)構(gòu)凸向管腔,血管間含多少不一的纖維組織。免疫組化:F8(+),CD31(+),CD34(-),CD68 (+),Lys(+)。病理診斷:LCA。見圖2。
圖2 術(shù)后病理
LCA是一種少見的特發(fā)于脾臟的腫瘤,病因不明。最近有學(xué)者認(rèn)為此腫瘤可能與免疫失調(diào)有關(guān)〔1〕,包括硫唑嘌呤、皮質(zhì)醇類激素及其他免疫抑制藥物的使用以及TNF-α的生物作用〔2〕等。TAN等〔3〕報(bào)道過1例腎移植術(shù)后接受免疫抑制治療13年發(fā)病的病例,他們認(rèn)為L(zhǎng)CA的發(fā)生機(jī)制與Castleman病十分類似。LCA發(fā)病年齡較廣,中位年齡50歲,但也有年僅1歲患者的病例報(bào)道,男女比例為3:5〔4-5〕。LCA的癥狀沒有明顯特異性,可表現(xiàn)為腹痛、脾大、脾功能亢進(jìn)、血小板減少、不明原因的發(fā)熱等,也有患者無(wú)明顯癥狀,由體檢等偶然情況發(fā)現(xiàn)。在影像學(xué)上LCA多表現(xiàn)為脾臟多發(fā)結(jié)節(jié),偶有散發(fā),結(jié)節(jié)大小0.2~6 cm,多為圓形或橢圓形,CT平掃期結(jié)節(jié)多為均勻或不均勻略低密度,也有少量呈現(xiàn)等密度,動(dòng)脈期呈輕度強(qiáng)化,病灶與正常強(qiáng)化的脾組織對(duì)比更清,靜脈期病灶逐漸均勻強(qiáng)化,至平衡期病灶與正常脾臟相仿、或略高于脾臟。LCA在癥狀、體征及影像學(xué)表現(xiàn)上沒并有特異性,最終確診需手術(shù)切除脾臟后行病理及免疫組化檢查。
LCA是一種良性腫瘤,但在已報(bào)道的的LCA病例中有1/3伴發(fā)內(nèi)臟器官的惡性腫瘤,包括結(jié)直腸、腎、肝、肺和胰腺癌、惡性淋巴瘤、骨髓增生異常綜合征、再生障礙性貧血等。經(jīng)查閱,已報(bào)道的文獻(xiàn)中僅有1 例LCA與甲狀腺腫瘤伴發(fā)的病例〔6〕。此文獻(xiàn)中患者甲狀腺乳頭狀腺癌術(shù)后1年發(fā)現(xiàn)頸部淋巴結(jié)、縱膈及脾臟轉(zhuǎn)移,腔鏡下切除脾臟行病理檢查提示LCA,這是第一次發(fā)現(xiàn)LCA與甲狀腺癌有關(guān)的報(bào)道。本文報(bào)道的患者頸部超聲提示:雙側(cè)頸部未探及異常淋巴結(jié)回聲,雙側(cè)甲狀腺多發(fā)結(jié)節(jié)樣病灶,TI-RASD分類:4A類。有甲狀腺癌可能,且周圍淋巴結(jié)未轉(zhuǎn)移,但因患者未行穿刺或甲狀腺手術(shù)及病理檢查,故無(wú)法確診。若為甲狀腺癌,則有遠(yuǎn)處轉(zhuǎn)移導(dǎo)致LCA可能;若甲狀腺結(jié)節(jié)為良性腫瘤,則目前尚無(wú)與LCA有確切聯(lián)系的相關(guān)報(bào)道,有待行進(jìn)一步的研究。
目前LCA的治療主要是手術(shù)切除,脾切除術(shù)后患者均可痊愈,且目前尚無(wú)復(fù)發(fā)及轉(zhuǎn)移的報(bào)道,但因部分病例合并惡性腫瘤,術(shù)后仍應(yīng)長(zhǎng)期隨訪。腹腔鏡下脾切除術(shù)相對(duì)傳統(tǒng)脾切除術(shù),具有創(chuàng)傷小、恢復(fù)快、住院時(shí)間短、術(shù)后腹腔黏連少、術(shù)口相對(duì)美觀等優(yōu)點(diǎn),應(yīng)作為L(zhǎng)CA的首選治療方案。
[參考文獻(xiàn)]
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(責(zé)任編輯董杰)
Littoral Cell Angioma of the Spleen: A Case Report and Literature Review
Shao Yu,Tan Yunbo*
(Affiliated Hospital of Dali University,Dali,Yunnan 671000,China)
〔Abstract〕Objective: To investigate the clinicopathologic features and the main points of diagnosis of littoral cell angioma(LCA)of spleen. Methods: The clinical features,radiologic findings and histopathologic changes were analyzed in a case of littoral cell angioma of spleen. Immunohistochemical study was carried out and relevant literature were reviewed. Results: Histologically,the lesions consisted of anastomosing ascular channels with papillary and few had cavernous appearance. And the vessels containing much of different fibrous tissue. LCA is often studied combining with visceral cancer in foreign literature. Conclusion: LCA is a rare angiogenic tumors from the littoral cell of spleen,whose diagnosis is on the basis of microscopic and immunohistochemical features. There is correlation with LCA and malignancy,and it should be closely observed with long-term follow-up.
〔Key words〕spleen;littoral cell;hemangioma;immunohistochemistry
[中圖分類號(hào)]R733.2
[文獻(xiàn)標(biāo)志碼]B
[文章編號(hào)]2096-2266(2016)04-0050-02
[收稿日期]2015-09-02[修回日期]2016-01-04
[作者簡(jiǎn)介]邵宇,醫(yī)師,主要從事普外科研究.
*通信作者:譚云波,教授.