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The Ultrasonographic Features of Female Reproductive System Extraosseous Ewing′s Sarcoma
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摘要:目的 研究女性生殖系统骨外尤文肉瘤的临床特点及超声图像特征，探讨超声对于女性生殖系统骨外尤文肉瘤的诊断价值。方法 回顾性分析2009年6月−2019年6月期间在我院经手术-病理证实的女性盆腔内尤文肉瘤的临床资料及超声表现，总结女性生殖系统骨外尤文肉瘤的临床特点及超声图像特征，提出超声对此类疾病的诊断思路。结果 10年间我院收治的经手术-病理确诊的女性生殖系统骨外尤文肉瘤患者共13例，患者年龄8月～40岁，无特异性临床症状，部分患者肿瘤标记物检测出现CA125升高。13例患者中共发现病灶19个，累及生殖系统病灶为16个，主要表现为回声不均匀的低回声实性占位或囊实性占位，体积较大，形态不规则，边界不清；少数患者可伴腹水；多数病灶血供为中量～丰富血流，以低阻血流为主，极少数病灶可能乏血供。所有病灶在术前均未能进行准确定性诊断。结论 女性生殖系统骨外尤文肉瘤术前定性诊断极困难，如遇边界欠清、形态欠规则的不均质低回声团块或宫体查见不均匀的回声减低团块时一定警惕骨外尤文肉瘤的可能，但最终的定性诊断仍需结合患者的病理检查结果。Abstract:Objective This study investigated the clinicopathological traits and ultrasound features of female reproductive system extraosseous Ewing's sarcoma (EES) and explored the diagnostic value of ultrasonography for this condition.Methods Cases of female pelvic EES diagnosed and treated at our hospital between June 2009 and June 2019 were included in this study. Pathology data and ultrasound manifestations were assessed retrospectively to summarize the clinical traits and ultrasound features of female reproductive system EES. Based on the results, recommendations for the ultrasonography-based diagnosis of this disease were proposed.Results During the 10-year study period, 13 female patients were diagnosed with EES in the pelvic cavity based on the results of postoperative pathology tests. The age of the patients ranged from 8 mouth to 40 years, and no patients demonstrated specific clinical symptoms. However, an examination of tumor biomarkers revealed that certain patients had elevated levels of CA125. In the 13 patients, 19 lesions were identified, including 16 that involved the reproductive system. The primary ultrasound manifestation was uneven, low-echo solidity or cystic solidity, exhibiting large size, irregular shape, and unclear boundary. A few patients had concurrent ascites. Although some lesions lacked blood supply, the blood supply of most lesions was medium to abundant, and the blood flow was mostly characterized by low resistant. Almost none of the lesions were definitively diagnosed preoperatively.Conclusions Preoperative definitive diagnosis of EES in the female reproductive system remains a great clinical challenge. Although certain clinical traits and ultrasound features are associated with this disease, and color Doppler ultrasonography might provide vital information indicating the presence of EES, the final diagnosis still depends on the pathological test results of the patients.
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Keywords:
- Genital system /
- Extraosseous Ewing's sarcoma /
- Clinics /
- Ultrasonography
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骨外尤文肉瘤（extraosseous Ewing's sarcoma，EES）是一种罕见的发生于软组织的由小圆形细胞组成的恶性肿瘤，来源于神经外胚层，故临床又称为外周原发性神经外胚层瘤（peripheral primary neuroectodermal tumors，PPNET）[1]。发生率低于所有肉瘤的1%[2]；可发生于全身各个部位，以四肢、脊柱旁软组织等为主要发生部位，而发生于女性生殖系统者十分罕见[3]，由于疾病本身缺乏特异性临床表现以及对该疾病的认识不足，女性生殖系统骨外尤文肉瘤术前诊断率极低。本研究回顾性分析2009年6月−2019年6月期间在我院经手术-病理证实的女性生殖系统骨外尤文肉瘤患者的临床资料及超声表现，总结女性生殖系统骨外尤文肉瘤的临床特点及超声图像特征，以期提高临床上对该疾病的认识和诊断水平。
1. 资料与方法
1.1 患者一般资料
收集来自2009年6月−2019年6月在四川大学华西第二医院妇科住院，术前超声检查提示盆腔内或外阴占位，经手术-病理确诊为骨外尤文肉瘤的13例患者所有临床资料。病理诊断标准[4]为：①肿瘤细胞由形态一致的小圆细胞组成，呈弥漫或分叶状排列，瘤细胞间可见纤维组织分隔；②肿瘤细胞核呈圆形，染色质均匀，可见核分裂像，过碘酸-雪夫（PAS）染色(+)；③光镜下大多数无菊形团坏死结构；④免疫组化: MIC基因产物CD99阳性表达率高，具有相对特异性。共19个病灶，排除3个肠道病灶，共计纳入16个生殖系统病灶。患者年龄8月～40岁，平均27.5岁。本研究已通过四川大学华西第二医院医学伦理委员会批准（医学科研2020伦审批第130号）。
1.2 仪器与方法
采用GE voluson730、GE E8、Philips iu22、Philips iu elite、Philips HD11、西门子Sequoia 512彩色多普勒超声诊断仪，经腹部探头频率为2.0～6.0 MHz，经阴道探头频率为5.0～9.0 MHz。
有性生活者采用经阴道及经腹部超声检查相结合，无性生活者采用经腹部超声检查。所有患者均进行二维及彩色多普勒超声检查及频谱多普勒检测。二维超声主要观察病灶形态、大小、内部回声强度及回声是否均匀、病灶后方有无回声增强效应，盆腹腔有无其他病灶、有无腹水；彩色多普勒超声主要观察病灶内有无血流、血流数目及形态，并按照Adler半定量方法进行血流分级[5]：0级，病灶内未见血流信号；Ⅰ级，少量血流，可见1～2处点状血流；Ⅱ级，中量血流，可见一条主要血管，其长度超过病灶的半径或见几条血管或3～4个点状血管；Ⅲ级，丰富血流，可见4条以上血管或血管相互连通，交织呈网状。
2. 结果
2.1 临床表现
13例EES患者多数是在体检时意外发现而就诊，最小8月龄，最大40岁，临床特点如下：①腹痛腹胀1例，阴道流血1例，月经紊乱1例，扪及包块者10例（盆腔包块4例、腹部3例、会阴包块1例、乳腺包块2例）；②超声发现生殖系统病灶16个，病灶位于卵巢9个（其中7例患者双侧卵巢受累），宫颈3个，宫体1个，会阴及阴道3个；③1例合并对侧卵巢良性肿物，1例合并子宫肌瘤；5例患者肿瘤标记物升高，CA125范围77.6~337.6 U/mL，伴CA199升高2例，分别为30.7 U/mL及40.86 U/mL。
2.2 术前超声表现
卵巢EES超声图像见图1，宫颈及阴道EES超声图像见图2，宫体EES超声图像见图3，外阴EES超声图像见图4。超声发现16个生殖系统病灶直径约3.9～24.1 cm，5例出现腹水（深度2.4～11.2 cm），频谱多普勒检测，血流阻力值（RI） 0.4～0.51，所有病灶的二维超声及彩色多普勒超声特征见表1。宫体、宫颈、会阴部病灶均为实性弱回声包块，回声不均匀，后方回声增强；卵巢病灶中有6例表现为囊实混合性包块，后方回声增强，回声不均匀，其中实性部分呈稍强回声，1例边界清楚；余3例呈实性弱回声包块，回声较均匀，后方回声增强不明显，2例边界清楚；所有病灶均可探及血流信号，Ⅰ级血流4例，Ⅱ级血流7例，Ⅲ级血流5例。
表 1 16个病灶超声特征Table 1. Ultrasonic features of the 16 lesionsUltrasonic manifestations Ovarian lesions Cervical lesions Uterine lesions Perineum and vagina lesions Morphology Cystic mixed mass 6 0 0 0 Solid 3 3 1 3 Boundary Clear 3 0 0 0 Blurred 6 3 1 3 Echo intensity Hyper echo 6 0 0 0 Poor echo 3 3 1 3 Echo uniformity Yes 3 0 0 0 No 6 3 1 3 Rear echo Enhancement 6 3 1 3 No apparent
enhancement3
0
0
0
Adler blood flow
classification0 0 0 0 0 Ⅰ 2 1 1 0 Ⅱ 3 2 0 2 Ⅲ 4 0 0 1 ![]()
图 1 卵巢病变的超声图像Figure 1. Sonographic images of the lesions in the ovaryA: Two-dimensional transabdominal ultrasound demonstrating a cystic mixed mass with clear boundary in the left Pelvic. Ascites were visible around the mass; B: Color Doppler flow image showed abundant blood flow of level Ⅲ in the lesion.![]()
图 2 宫颈及阴道病变的超声图像Figure 2. Sonographic images of the lesions in the cervix and vaginaA: Two-dimensional ultrasonic sagittal view of the uterus demonstrating that the cervix and upper vagina was obviously enlarged and presented a heterogeneous poorechoic mass with clear boundary; B: Color Doppler flow image showed rich blood flow of level Ⅱ in the lesion. UT: Uterus; M: Mass.![]()
图 3 子宫病变的超声图像Figure 3. Sonographic images of the lesions in the uterusA: Two-dimensional transvaginal ultrasound demonstrating a poor echo mass with unclear boundary in the uterus, small pieces of liquid dark area inside, echo enhancement behind the mass; B: Color Doppler flow image showed punctate blood flow of level Ⅰ in the lesion. UT: Uterus; M: Mass.![]()
图 4 会阴部病变的超声图像Figure 4. Sonographic images of the lesions in the perineumA: Two-dimensional superficial ultrasound a heterogeneous poor echo was found in the subcutaneous layer of the left labia majora, which had irregular shape, unclear boundary, scattered strong echoes inside; B: Color Doppler flow image showed abundant blood flow of level Ⅱ in the lesion.3. 讨论
原始神经外胚层肿瘤（primary neuroectodermal tumors，PNET）属于尤文肉瘤家族肿瘤，是一种罕见的具有多向分化潜能的高度恶性小圆细胞肿瘤，分为中枢和外周PNET，男女发病率为1.5∶1[1-2]，外周PNET又称为EES，女性生殖道发生的EES大多发生在青少年和绝经前，多在30岁以下，很少超过40岁[6-7]，本研究纳入病例最小年龄8月龄，最大40岁，30岁以下10例，30～40岁3例，与文献报道一致。
女性生殖系统骨外尤文肉瘤患者缺乏特异性临床症状，多数仅表现为腹胀、查体触及盆腔包块等，少数患者可出现发热、盗汗、体质量减轻，与其他盆腔肿瘤难以鉴别[8]。同时，由于此疾病发病罕见，临床及影像医生对疾病缺乏足够的认识，术前诊断率极低，有文献表明，国内大医院1996−2004年内对本病术前诊断明确率为0[4]，本研究中患者在术前也均未能进行准确的定性诊断，所有患者均依据手术-病理结果进行确诊。
本研究分析了患者的临床特点、超声表现并复习相关文献[1, 3-10]，总结出女性生殖系统骨外尤文肉瘤的特征，以期提高对该疾病的认识：①无特异性临床症状；肿瘤标记物CA125可出现升高，本组有5例患者CA125升高（70.1～337.6 U/mL）；②二维超声：附件区可见形态较规则、边界较清的实性占位及形态不规则、边界不清的囊实性占位，体积一般较大，回声多为较均匀的低回声或欠均匀的稍强回声，病灶后方可出现回声增强效应，实性团块内部可出现由变性坏死所形成的液化或钙化；宫颈部位为形态较规则、边界不清的实性团块，回声为不均匀低回声，病灶后方回声增强；阴道及会阴的病灶为形态较规则、边界不清的实性团块，回声多为不均匀低回声，病灶后方回声增强；宫体病变则表现为宫体肌层查见不均匀的回声减低团块，内可见片状液性暗区，未受累内膜形态及回声未见明显异常。出现以上超声征象，可能与骨外尤文肉瘤组织大体形态常呈多结节状或分叶状，瘤体由紧密成片或小叶状分布的小圆细胞组成，小叶间为宽窄不等的纤维结缔组织间隔，肿瘤内常见凝固性坏死有关；5例患者合并腹水；③CDFI检测：病灶内多可探及中量至丰富血流。本组16个病灶，其中12个为Ⅱ级及以上血流。血流频谱检测以低阻动脉血流为主，RI 0.4～0.51。
女性生殖系统骨外尤文肉瘤的超声诊断需要注意与其他生殖系统恶性肿瘤进行鉴别。卵巢尤文肉瘤与卵巢上皮性肿瘤、生殖细胞肿瘤、转移性肿瘤及附件囊性病变等鉴别，宫颈尤文肉瘤需与宫颈癌鉴别，而宫体尤文肉瘤则需与子宫肉瘤、子宫内膜癌等进行鉴别。超声检查能够判断病变的囊、实性，观察病变的形态、大小、包膜、内部细小结构回声改变、与周围组织的关系、内部血流分布及血流性质，有助于判断病变的性质，但确诊还需依靠病理诊断[3]。
综上所述，女性生殖系统骨外尤文肉瘤是一种发病率极低的女性生殖系统恶性肿瘤，缺乏特异性临床症状。超声检查具有一定的特点，可以提供有价值的诊断信息，如发现边界欠清、形态欠规则的不均质低回声肿块或宫体查见不均匀的回声减低团块时，需警惕骨外尤文肉瘤可能，可提示临床采取进一步的相关检查，以期提高该疾病的术前诊断水平。
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图 1 卵巢病变的超声图像
Figure 1. Sonographic images of the lesions in the ovary
A: Two-dimensional transabdominal ultrasound demonstrating a cystic mixed mass with clear boundary in the left Pelvic. Ascites were visible around the mass; B: Color Doppler flow image showed abundant blood flow of level Ⅲ in the lesion.
![]()
图 2 宫颈及阴道病变的超声图像
Figure 2. Sonographic images of the lesions in the cervix and vagina
A: Two-dimensional ultrasonic sagittal view of the uterus demonstrating that the cervix and upper vagina was obviously enlarged and presented a heterogeneous poorechoic mass with clear boundary; B: Color Doppler flow image showed rich blood flow of level Ⅱ in the lesion. UT: Uterus; M: Mass.
![]()
图 3 子宫病变的超声图像
Figure 3. Sonographic images of the lesions in the uterus
A: Two-dimensional transvaginal ultrasound demonstrating a poor echo mass with unclear boundary in the uterus, small pieces of liquid dark area inside, echo enhancement behind the mass; B: Color Doppler flow image showed punctate blood flow of level Ⅰ in the lesion. UT: Uterus; M: Mass.
![]()
图 4 会阴部病变的超声图像
Figure 4. Sonographic images of the lesions in the perineum
A: Two-dimensional superficial ultrasound a heterogeneous poor echo was found in the subcutaneous layer of the left labia majora, which had irregular shape, unclear boundary, scattered strong echoes inside; B: Color Doppler flow image showed abundant blood flow of level Ⅱ in the lesion.
表 1 16个病灶超声特征
Table 1 Ultrasonic features of the 16 lesions
Ultrasonic manifestations Ovarian lesions Cervical lesions Uterine lesions Perineum and vagina lesions Morphology Cystic mixed mass 6 0 0 0 Solid 3 3 1 3 Boundary Clear 3 0 0 0 Blurred 6 3 1 3 Echo intensity Hyper echo 6 0 0 0 Poor echo 3 3 1 3 Echo uniformity Yes 3 0 0 0 No 6 3 1 3 Rear echo Enhancement 6 3 1 3 No apparent
enhancement3
0
0
0
Adler blood flow
classification0 0 0 0 0 Ⅰ 2 1 1 0 Ⅱ 3 2 0 2 Ⅲ 4 0 0 1 
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1. 马雪影，叶玲，王晓慧. 妊娠合并骨外尤因肉瘤治疗后复发转移成功分娩一例. 国际妇产科学杂志. 2022(06): 687-690 . 
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