北京大学学报(医学版) ›› 2018, Vol. 50 ›› Issue (6): 1098-1101. doi: 10.19723/j.issn.1671-167X.2018.06.028

• 疑难/罕见病例分析 • 上一篇    下一篇

盆腹腔软组织侵袭性血管黏液瘤临床病理分析

刘蕾(),王丽华,任玉波,饶晓松,杨邵敏   

  1. 北京大学国际医院病理科, 北京 102206
  • 收稿日期:2017-07-04 出版日期:2018-12-18 发布日期:2018-12-18
  • 通讯作者: 刘蕾 E-mail:leids_l@hotmail.com

Clinicopathological analysis of aggressive angiomyxoma of soft tissue in abdomino-pelvic cavity

Lei LIU(),Li-hua WANG,Yu-bo REN,Xiao-song RAO,Shao-min YANG   

  1. Department of Pathology, Peking University International Hospital, Beijing 102206, China
  • Received:2017-07-04 Online:2018-12-18 Published:2018-12-18
  • Contact: Lei LIU E-mail:leids_l@hotmail.com

摘要:

侵袭性血管黏液瘤是一种少见的间叶性肿瘤,为探讨其临床病理学特征、诊治及预后,本研究收集北京大学国际医院4例盆腹腔软组织侵袭性血管黏液瘤的临床影像资料,分析其病理学形态、免疫表型及治疗随访情况,并回顾相关文献。本组4例均为女性,年龄27~49岁,平均年龄33岁,无诱因腹胀或无明显不适经体检发现,3例原发、1例复发。3例CT及磁共振扫描可见分层/漩涡征。肿瘤主体均位于盆腔,1例同时累及腹腔。肿瘤最大径5~22 cm,切面灰白、灰黄色,实性,质软,灶性水肿或囊性改变。显微镜下瘤细胞短梭形,异型不明显,间质富疏松水肿黏液或胶原,有丰富的薄壁及厚壁血管,部分血管壁玻璃样变性。肿瘤可浸润周围脂肪、神经组织。免疫组织化学染色提示4例肿瘤均雌激素受体、孕激素受体、结蛋白、平滑肌肌动蛋白弥漫阳性,CD34仅血管表达,S-100蛋白、CD117、Dog1均阴性。手术完整切除肿瘤,术后随访有1例二次复发。侵袭性血管黏液瘤确诊以病理形态学为基础,辅以免疫组织化学进一步诊断及鉴别诊断;手术切除后可多次复发。

关键词: 黏液瘤, 盆腔肿瘤, 病理学, 临床, 免疫组织化学

Abstract:

Aggressive angiomyxoma is a rare mesenchymal tumor. To discuss the clinicopathological characteristics, treatment and prognosis of aggressive angiomyxoma, four cases of aggressive angiomyxoma of soft tissue in abdominopelvic cavity were collected from January 2015 to August 2017 in Peking University International Hospital. The clinical data, imaging examination, histopathological features, immunophenotype, therapy and prognosis were analysed. The related literatures were reviewed. All of the patients were adult females, age range from 27 to 49 years and mean 33 years. The clinical complaint was abdominal distention with no definite predisposing factor, or occasional physical-exam finding with no obvious discomfort. Three cases were primary and one case was recurrent. Typical layered or swirled structural sign was presented by CT and MRI scanning of three cases. All tumors located in the pelvic cavity, and attached to the uterus, vagina, rectum, bladder or ureter. One case was involved in the abdominal cavity simultaneously,adhesive to the spine, inferior vena cava and spleen. The gross appearance of tumors was from 5 to 22 cm in maximum diameter. The sectioned surfaces were soft, solid, white or yellow-gray, focally accompanied by edema, mucoid degeneration or cystic change. Microscopic observation showed that tumor cells were short spindle shaped and little atypical, the stroma was loose like ede-matous mucus or collagen, and the vessels were rich in thin and thick-wall. Partially the vessel wall expressed hyaline degeneration. Also tumors might infiltrate surrounding tissue, such as fat or nerve. The immunohistochemistry results of all cases were estrogen receptor and progesterone receptor diffusely mo-derate positive, Desmin and smooth muscle actin mostly positive, whereas CD34 expressed only in vessel and S-100 protein, CD117 and Dog1 all negative. All the tumors were complete surgical excision. During follow-up, one case recurred the second time. Our conclusions are the diagnosis of aggressive angiomy-xoma is based on pathological morphology supplemented by immunohistochemistry, and the tumor may relapse after surgical resection.

Key words: Myxoma, Pelvic neoplasms, Pathology, clinical, Immunohistochemistry

中图分类号: 

  • R739.9

图1

肿瘤切面水肿、黏液样变性"

图2

肿瘤细胞短梭形、稀疏,间质富黏液样基质,血管壁玻璃样变性(HE ×40)"

图3

肿瘤富含薄壁和厚壁血管,网织纤维染色(A, 银染法 ×40)和免疫组织化学染色CD34阳性(B,EnVision ×40)"

图4

肿瘤细胞免疫组织化学染色(EnVision ×100):A,ER弥漫核阳性;B,PR弥漫核阳性;C,SMA胞浆阳性"

[1] Fletcher CDM, Bridge JA, Hogendoorn PCW , et al. World Health Organization classification of tumours of soft tissue and bone [M]. Lyon: IARC Press, 2013.
[2] Zhang JP, Zhu CF . Clinical experiences on aggressive angio-myxoma in China (report of 93 cases)[J]. Int J Gynecol Cancer, 2010,20(2):303-307.
doi: 10.1111/IGC.0b013e3181cc339c pmid: 20134274
[3] Ahmed MA, Uehelie MA, Rage AM , et al. Aggressive angio-myxoma of the penis: the first case report in a 9-month-old infant[J]. Urology, 2017,104(6):187-190.
doi: 10.1016/j.urology.2016.12.045
[4] Surabhi VR, Garg N, Frumovitz M , et al. Aggressive angio-myxomas: a comprehensive imaging review with clinical and histopathologic correlation[J]. Am J Roentgenol, 2014,202(6):1171-1178.
doi: 10.2214/AJR.13.11668 pmid: 24848813
[5] Petscavage-Thomas JM, Walker EA, Logie CI , et al. Soft-tissue myxomatous lesions: review of salient imaging features with pathologic comparison[J]. Radiographics, 2014,34(4):964-980.
doi: 10.1148/rg.344130110 pmid: 25019435
[6] Sun Y, Zhu L, Chang X , et al. Clinicopathological features and treatment analysis of rare aggressive angiomyxoma of the female pelvis and perineum: a retrospective study[J]. Pathol Oncol Res, 2017,23(1):131-137.
doi: 10.1007/s12253-016-0109-y pmid: 27571990
[7] Chen H, Zhao H, Xie Y , et al. Clinicopathological features and differential diagnosis of aggressive angiomyxoma of the female pelvis: 5 case reports and literature review[J]. Medicine (Baltimore), 2017,96(20):e6820.
doi: 10.1097/MD.0000000000006820
[8] 魏红权, 刘珺, 冯晶晶 . 侵袭性血管黏液瘤临床病理观察[J]. 中华医学杂志, 2012,92(22):1553-1555.
doi: 10.3760/cma.j.issn.0376-2491.2012.22.013
[9] Alameda F, Munne A, Baro T , et al. Vulvar angiomyxoma, aggressive angiomyxoma, and angiomyofibroblastoma: an immunohistochemical and ultrastructural study[J]. Ultrastruct Pathol, 2006,30(3):193-205.
doi: 10.1080/01913120500520911 pmid: 16825121
[10] Rawlinson NJ, West WW, Nelson M , et al. Aggressive angio-myxoma with t(12;21) and HMGA2 rearrangement: report of a case and review of the literature[J]. Cancer Genet Cytogenet, 2008,181(2):119-124.
doi: 10.1016/j.cancergencyto.2007.11.008 pmid: 18295664
[11] Jiang XX, Qian LH, Xu HK , et al. Mutation of an X chromosome in aggressive angiomyxoma: Report of a case and review of the literature[J]. Gynecol Oncol Case Rep, 2011,2(2):39-41.
doi: 10.1016/j.gynor.2011.12.001 pmid: 3860615
[12] Bai HM, Yang JX, Huang HF , et al. Individualized managing strategies of aggressive angiomyxoma of female genital tract and pelvis[J]. Eur J Surg Oncol, 2013,39(10):1101-1108.
doi: 10.1016/j.ejso.2013.06.013 pmid: 23899874
[13] Han-Geurts IJ, van Geel AN, van Doorn L , et al. Aggressive angiomyxoma: multimodality treatments can avoid mutilating surgery[J]. Eur J Surg Oncol, 2006,32(10):1217-1221.
doi: 10.1016/j.ejso.2006.06.008 pmid: 16870390
[14] Orfanelli T, Kim CS, Vitez SF , et al. A case report of aggressive angiomyxoma in pregnancy: do hormones play a role[J]. Case Rep Obstet Gynecol, 2016(3):1-4.
doi: 10.1155/2016/6810368
[15] 宫丽华, 孙晓淇, 孟淑琴 , 等. 骨内侵袭性血管黏液瘤的诊断及与其他黏液性骨肿瘤的鉴别[J]. 临床与实验病理学杂志, 2012,28(10):1143-1145.
doi: 10.3969/j.issn.1001-7399.2012.10.019
[16] Schwartz PE, Hui P , McCarthy S. Hormonal therapy for aggressive angiomyxoma: a case report and proposed management algorithm[J]. J Low Genit Tract Dis, 2014,18(2):55-61.
doi: 10.1097/LGT.0b013e3182a22019
[17] Wang Z, Liu Y, Yang L , et al. Maxillary aggressive angiomyxoma showing ineffective to radiotherapy: a rare case report and review of literature[J]. Int J Clin Exp Pathol, 2015,8(1):1063-1067.
pmid: 25755820
[18] Coppola S, Desai A, Tzanis D , et al. Conservative en bloc surgery for aggressive angiomyxoma achieves good local control: analysis of 14 patients from a single institution[J]. Int J Gynecol Cancer, 2013,23(3):540-545.
doi: 10.1097/IGC.0b013e3182843063
[19] Blandamura S, Cruz J, Faure Vergara L , et al. Aggressive angiomyxoma: a second case of metastasis with patient’s death[J]. Hum Pathol, 2003,34(10):1072-1074.
doi: 10.1053/S0046-8177(03)00419-2 pmid: 14608546
[20] Geng J, Cao B, Wang L . Aggressive angiomyxoma: an unusual presentation[J]. Korean J Radiol, 2012,13(1):90-93.
doi: 10.3348/kjr.2012.13.1.90 pmid: 22247641
[1] 薛江,张建运,时瑞瑞,谢晓艳,白嘉英,李铁军. 105例口腔颅颌面部纤维性结构不良的临床病理分析[J]. 北京大学学报(医学版), 2022, 54(1): 54-61.
[2] 魏慧,罗增,次旦央宗,白玛央金. 高原地区不同海拔高度腹型过敏性紫癜患者临床特征分析[J]. 北京大学学报(医学版), 2021, 53(6): 1072-1077.
[3] 宋志博,耿研,邓雪蓉,张晓慧,张卓莉. 肌肉骨骼超声在指导银屑病关节炎临床分型中的价值[J]. 北京大学学报(医学版), 2021, 53(6): 1061-1066.
[4] 刘梅歌,方朴,王严,丛璐,范洋溢,袁远,徐燕,张俊,洪道俊. 远端型遗传性运动神经病8例的临床、病理及遗传学特点[J]. 北京大学学报(医学版), 2021, 53(5): 957-963.
[5] 蒋艳芳,王健,王永健,刘佳,裴殷,刘晓鹏,敖英芳,马勇. 前交叉韧带翻修重建术后中长期临床疗效及影响因素[J]. 北京大学学报(医学版), 2021, 53(5): 857-863.
[6] 于妍斐,何世明,吴宇财,熊盛炜,沈棋,李妍妍,杨风,何群,李学松. 延胡索酸水合酶缺陷型肾细胞癌的临床病理特征及预后[J]. 北京大学学报(医学版), 2021, 53(4): 640-646.
[7] 邱敏,费月阳,邓绍晖,刘承,卢剑,何为,陆敏,田晓军,张树栋,马潞林. 后肾腺瘤的诊治经验及文献回顾[J]. 北京大学学报(医学版), 2021, 53(2): 417-419.
[8] 夏芳芳,鲁芙爱,吕慧敏,杨国安,刘媛. 系统性红斑狼疮伴间质性肺炎的临床特点及相关因素分析[J]. 北京大学学报(医学版), 2021, 53(2): 266-272.
[9] 高阳旭,孙青,李辉,谢瑶,姚红新,赵卫红. 儿童肝母细胞瘤多学科协作模式下的治疗效果及临床成本[J]. 北京大学学报(医学版), 2021, 53(1): 200-203.
[10] 池彦廷,张延平,张秋露,刘翠苓,李斌斌. 唾液腺干燥综合征继发黏膜相关淋巴组织淋巴瘤的临床病理分析[J]. 北京大学学报(医学版), 2021, 53(1): 40-45.
[11] 甘雨舟,李玉慧,张丽华,马琳,何文雯,金月波,安媛,栗占国,叶华. 临床无肌病性皮肌炎与皮肌炎临床及免疫学特征比较[J]. 北京大学学报(医学版), 2020, 52(6): 1001-1008.
[12] 魏士雄,黎苏佳,刘毅. 幼年特发性关节炎成人后的临床特点及生物制剂治疗[J]. 北京大学学报(医学版), 2020, 52(6): 1014-1022.
[13] 程功,张霞,杨菲,程嘉渝,刘燕鹰. 以发热、关节炎、皮肤色素沉着为主要表现的血管免疫母细胞性T细胞淋巴瘤1例[J]. 北京大学学报(医学版), 2020, 52(6): 1150-1152.
[14] 刘毅,刘志坚,沈棋,吴静云,范宇,李德润,虞巍,何志嵩. 14例恶性潜能未定的前列腺间质肿瘤病例分析[J]. 北京大学学报(医学版), 2020, 52(4): 621-624.
[15] 郝一昌,颜野,张帆,邱敏,周朗,刘可,卢剑,肖春雷,黄毅,刘承,马潞林. 穿刺活检单针阳性的前列腺癌手术策略选择及经验总结[J]. 北京大学学报(医学版), 2020, 52(4): 625-631.
Viewed
Full text


Abstract

Cited

  Shared   
  Discussed   
[1] 田增民, 陈涛, Nanbert ZHONG, 李志超, 尹丰, 刘爽. 神经干细胞移植治疗遗传性小脑萎缩的临床研究(英文稿)[J]. 北京大学学报(医学版), 2009, 41(4): 456 -458 .
[2] 郭岩, 谢铮. 用一代人时间弥合差距——健康社会决定因素理论及其国际经验[J]. 北京大学学报(医学版), 2009, 41(2): 125 -128 .
[3] 成刚, 钱振华, 胡军. 艾滋病项目自愿咨询检测的技术效率分析[J]. 北京大学学报(医学版), 2009, 41(2): 135 -140 .
[4] 卢恬, 朱晓辉, 柳世庆, 郑杰, 邱晓彦. 白细胞介素2促进宫颈癌细胞系HeLaS3免疫球蛋白G的表达[J]. 北京大学学报(医学版), 2009, 41(2): 158 -161 .
[5] 袁惠燕, 张苑, 范田园. 离子交换型栓塞微球及其载平阳霉素的制备与性质研究[J]. 北京大学学报(医学版), 2009, 41(2): 217 -220 .
[6] 徐莉, 孟焕新, 张立, 陈智滨, 冯向辉, 释栋. 侵袭性牙周炎患者血清中抗牙龈卟啉单胞菌的IgG抗体水平的研究[J]. 北京大学学报(医学版), 2009, 41(1): 52 -55 .
[7] 董稳, 刘瑞昌, 刘克英, 关明, 杨旭东. 氯诺昔康和舒芬太尼用于颌面外科术后自控静脉镇痛的比较[J]. 北京大学学报(医学版), 2009, 41(1): 109 -111 .
[8] 祁琨, 邓芙蓉, 郭新彪. 纳米二氧化钛颗粒对人肺成纤维细胞缝隙连接通讯的影响[J]. 北京大学学报(医学版), 2009, 41(3): 297 -301 .
[9] Jian-wei GU, Emily YOUNG, Zhi-jun PAN, Kevan B. TUCKER, Megan SHPARAGO, Min HUANG, Amelia Purser BAILEY. SD大鼠长期高盐饮食可导致其高血压并改变肾细胞因子基因表达谱[J]. 北京大学学报(医学版), 2009, 41(5): 505 -515 .
[10] 李宏亮*, 安卫红*, 赵扬玉, 朱曦. 妊娠合并高脂血症性胰腺炎行血液净化治疗1例[J]. 北京大学学报(医学版), 2009, 41(5): 599 -601 .