幕下小腦實質間變性室管膜瘤1例

呂青青，張 勇，程敬亮，汪衛建

(鄭州大學第一附屬醫院核磁共振科，河南 鄭州 450052)

幕下小腦實質間變性室管膜瘤1例

呂青青，張 勇，程敬亮，汪衛建

(鄭州大學第一附屬醫院核磁共振科，河南 鄭州 450052)

圖1 幕下小腦實質間變性室管膜瘤MR圖像 A、B.軸位T1WI及T2WI示右側小腦半球團塊狀長T1混雜長T2信號影； C.增強掃描示病灶明顯強化 圖2 病理圖 瘤細胞呈“菊形”團樣，瘤細胞異形明顯，核分裂活躍，圍繞血管排列(HE，×100)

患者女，41歲，因“頭痛、頭昏20天，雙下肢無法站立2天”就診。神經系統檢查及實驗室檢查無異常，初步診斷為“精神障礙”。MR檢查：右側小腦半球可見團塊狀長T1混雜長T2信號(圖1A、1B)，周邊見片狀長T1長T2水腫信號，FLAIR呈高信號，DWI見高信號，小腦中腳及4腦室明顯受壓變形；增強后右側小腦半球病變呈團塊狀明顯強化，邊界清晰，約4.0 cm×3.9 cm×4.3cm(圖1C)。MRI診斷：室管膜瘤。行后正中右拐入路腫瘤切除術，術中見腫瘤位于右側小腦及枕骨大孔，呈灰紅色，質軟，血供豐富，邊界不清。術后大體病理示腫瘤呈灰白灰紅碎組織，鏡下示瘤細胞呈“菊形”團樣，瘤細胞異形明顯，核分裂活躍，圍繞血管排列(圖2)。免疫組化：GFAP(局部+)，S-100(+)，Oligo-2(散在+)，EMA(-/+)，NSE(-)，Syn(-)，CD99(-/+)，FLI-1(+/-)，CD56(+)，CK(-)，CK-L(-)，Ki-67(60%+)，LCA(-)。病理診斷：右側小腦半球間變性室管膜瘤(WHO Ⅲ級)。

討論 腦實質室管膜瘤是發生于腦室系統外，起源于胚胎殘余的室管膜靜止細胞的腫瘤。好發于兒童及青少年，少數可見于50歲左右的中老年人。幕下腦實質間變性室管膜瘤多發生于小腦半球，多為實性，瘤內出血少見；MRI多呈等或稍長T1信號，等或稍長T2信號，腫瘤周圍可見水腫；增強掃描腫瘤實質成分呈明顯強化，中心壞死區無強化；常與腦室關系密切，易壓迫腦室造成梗阻性腦積水。本例患者腫瘤發病年齡和部位均不典型，MRI表現與上述表現基本相符。幕下腦實質間變性室管膜瘤需與髓母細胞瘤、腦膜瘤等相鑒別：髓母細胞瘤為兒童后顱窩常見惡性腫瘤，囊變壞死少見，增強掃描后強化較均勻，常通過腦脊液播散轉移；腦膜瘤形態較規整，邊緣常清晰，信號較均勻，基底部常與鄰近腦膜相接觸，增強掃描可見腦膜尾征。幕下腦實質內的室管膜瘤影像表現雖有一些特征，但因發病率低，發病部位少見，誤診率高；若MRI表現為幕下腦實質內實性腫塊，呈長T1長T2信號，增強掃描呈明顯強化，應考慮幕下腦實質內室管膜瘤的可能。

[Key words] Salivary gland scintigraphy; Quantitative analysis; Sj?gren's syndrome

DOI：10.13929/j.1003-3289.201609058

Subtentorial； Anaplastic ependymoma； Magnetic resonance imaging [關鍵詞] 幕下；間變性室管膜瘤；磁共振成像

Subtentorial intraparenchymal anaplastic ependymoma in cerebellum: Case report

Salivary gland scintigraphy in diagnosis of Sj?gren's syndrome

ZOUHuifeng1，SHENYang1，YOUJiaxi2，YANGYi1*

(1.DepartmentofNuclearMedicine,SuzhouScience&TechnologyTownHospital,Suzhou215153,China; 2.DepartmentofNuclearMedicine,theSecondHospitalAffiliatedtoSoochowUniversity,Suzhou215004,China)

Objective To assess the value of salivary gland scintigraphy in diagnosis of Sj?gren's syndrome (SS). Methods A total of 44 patients with clinically suspicious SS were included. The data of salivary gland scintigraphy were retrospectively analyzed and the time-radioactivity curve (TAC) was obtained by outlining ROI in bilateral parotid glands and submaxillary glands. Uptake index (UI) and excretion fraction (EF) were defined. Both UI and EF were compared with the visual assessment and final diagnosis respectively. Results UI and EF of bilateral parotid glands and submaxillary glands in SS patients were significantly lower than those in non-SS patients (allP<0.05). The impaired salivary gland function was classified as 0—3 grades by visual assessment. The UI of bilateral parotid glands and submaxillary glands were negatively correlated with the qualitative classification. While there were no significant correlations between EF and qualitative classification (allP>0.05), except for that of right submaxillary gland (r=-0.312,P=0.039). The comprehensive diagnostic efficacy of UI on SS patients was higher than those of visual assessment, but their area under curves of ROC were not significantly different (allP>0.05). Conclusion UI and EF can effectively evaluate salivary gland function and serve as objective tools to distinguish patients with SS.

呂青青(1993—)，女，河南商丘人，在讀碩士。

E-mail： 478071645@qq.com

2016-09-21

2016-11-24

10.13929/j.1003-3289.201609097

R739.41; R445.2

B

1003-3289(2017)03-0398-01