精索旁侵袭性血管黏液瘤的临床特征(附2例报告及复习文献)

目的探讨精索旁侵袭性血管黏液瘤的临床特征。方法报告2例精索旁侵袭性血管黏液瘤患者的临床资料,结合文献复习进行讨论。结果2例患者的精索旁侵袭性血管黏液瘤均手术完整切除,经随访6~8个月,无肿瘤复发。病理诊断为精索旁侵袭性血管黏液瘤。结论侵袭性血管黏液瘤临床罕见,在男性好发于精索旁,确诊依赖病理学检查,治疗需手术完整切除肿瘤,术后应长期严密随访。     

误诊为尿路上皮癌的膀胱平滑肌肉瘤1例并文献分析

目的探讨膀胱平滑肌肉瘤的临床表现、病理学特征、治疗及预后,提高对该病的认知和诊疗水平。方法回顾分析1例膀胱平滑肌肉瘤患者的临床资料及诊疗过程并复习相关国内外文献。结果患者经术前各项检查明确手术指征,充分完善术前准备后行腹腔镜下根治性膀胱全切+乙状结肠代膀胱术。术后病理结果示:(膀胱)间叶性恶性肿瘤,倾向平滑肌肉瘤,免疫组化SMA(+)、Vimentin(+)、Galdesmon(-)、Bcl2(-)、Ki67(约15%)、Desmin(-)、CD34(血管+)、P53(灶+)、EMA(-)、S-100(-)、CDK4(-)、CK(-)。术后辅以AI方案化疗:异磷酰胺(130 mg/m^2)、多柔比星(20 mg/m^2)。随访10个月未见肿瘤转移及复发,治疗效果明显。结论膀胱平滑肌肉瘤系一种极其少见的侵袭性间质瘤,临床和影像学表现均不典型,临床上很容易误诊,其治疗方法可考虑以手术联合化疗为主。     

膀胱平滑肌肉瘤6例诊疗分析及文献复习

目的:结合临床资料和文献复习,探讨膀胱平滑肌肉瘤的诊断和治疗方法。方法:自1996～2006年收治膀胱平滑肌肉瘤6例,均表现为肉眼血尿,膀胱镜检查提示肿瘤瘤体光滑,呈菜花或乳头状,位于膀胱侧壁、顶壁或后壁。行根治性全膀胱切除术1例,肿物及部分膀胱切除术5例。结果:病理检查证实均为膀胱平滑肌肉瘤,免疫组织化学检查SMA强阳性6例,MSA阳性6例,Des( )2例,S-100(-)4例,S-100( /-)2例,CD34(-)6例。结论:膀胱平滑肌肉瘤是一种少见的泌尿生殖系恶性肿瘤,治疗以手术为主,除非肿块很小,一般不考虑采用经尿道电切术。该病的发生可能与环磷酰胺化疗和放射治疗等因素相关。     

左侧闭孔神经鞘瘤1例

患者女, 43岁, 因"体检时发现盆腔肿物6 d"入院。全腹部CT检查:左侧髂血管区软组织密度, 右侧附件区囊状低密度影。盆腔增强MRI检查示:左侧髂血管区见椭圆形囊实性异常信号, 大小约4.7 cm×3.9 cm, 考虑神经源性肿瘤(图1)。行腹膜后肿物切除术。术中见肿物位于左侧输尿管腹下神经筋膜外侧, 上缘进入闭孔, 下缘位于左侧髂静脉分叉处, 肿物呈囊实性, 质韧, 大小约5 cm×4 cm×4 cm, 表面包膜完整, 肿物表面及两端均可见神经纤维, 充分游离肿物, 完整切除(图2)。术后病理检查示:(腹膜后)神经鞘瘤, 伴骨化(图3A)。免疫组织化学检查示:Vimentin(+), S-100(+), SOX10(+)(图3B), SMA(-), Desmin(-), CD17(-), CD34(-), Ki67增殖指数<1%。     

空肠原发性侵袭性纤维瘤病1例

患者女, 57岁, 因"体检发现腹腔肿物2个月"入院。腹部CT检查提示:中上腹偏右侧见团片状异常密度影, 呈囊实性改变, 小肠间质瘤可能性大(图1)。术中探查见肿瘤来源于空肠起始部, 瘤体大小13 cm×11 cm×6.5 cm, 大部分外生性生长, 瘤体导致肠腔轻度狭窄并压迫周围肠管, 对应肠系膜内多发肿大淋巴结。行肿瘤完整切除。术后随访6个月, 未见肿瘤复发。术后病理检查示:肿瘤主体位于肌层内, 呈浸润性生长, 破坏肠壁平滑肌, 侵达黏膜下层及浆膜下层(图2A), 由纤维母细胞及肌纤维母细胞组成, 呈平行束状、交织状排列, 部分呈波浪状排列, 间质疏松, 可见水肿及黏液样变区, 瘤细胞核染色质稀疏或空泡状, 可见小核仁, 胞质嗜双色, 两端细长(图2B)。黏液样区瘤细胞呈星芒状, 未见明确核分裂象, 肿瘤内含有小至中等大血管, 血管周围伴水肿。肠周淋巴结未见转移(0/4)。免疫组化染色结果示:Vimentin(+)(图3A)、β-Catenin(核+)(图3B)、Calponin(部分阳性)(图3C)、CD117(-)、Dog-1(-)、CD34(血管+)、SMA(-)、Desmi...     

膀胱平滑肌肉瘤3例报告并文献复习

目的 探讨膀胱平滑肌肉瘤的临床病理和诊治方法.方法 患者3例,肉眼血尿,膀胱镜检见肿瘤分别发生于膀胱左侧壁、左前壁和右后壁,直径2.0～3.5 cm,以菜花样和乳头样为主,基底较宽,瘤体表面光滑.结果 3例均行全膀胱切除术,病理表现为上皮下间质富于黏液,黏液中可见散在或束状分布的细长梭形瘤细胞,其胞质淡染,核呈长梭形,有轻度异型,核分裂象易见.免疫组化SMA(+)3例、MSA(+)3例、Des(+)2例、S-100(-)2例、CD34(-)3例,均诊断为膀胱低级别平滑肌肉瘤.随访6个月～8年,1例术后2.5年死亡,2例存活.结论 膀胱平滑肌肉瘤临床罕见,确诊依赖病理及免疫组化检查,应与平滑肌瘤、横纹肌肉瘤、癌肉瘤、炎性肌纤维母细胞瘤等鉴别,治疗以手术为主,需根据肿瘤分级、分期决定手术方案及术后是否需行辅助治疗.     

腹腔良性转移性平滑肌瘤1例

患者女, 34岁, 因"体检发现腹腔肿物"入院。5年前于当地妇幼保健院行"子宫肌瘤切除术", 病理检查为平滑肌瘤。增强CT检查提示左下腹实性占位(图1)。行腹腔镜探查术, 可见降结肠中段大小约8 cm×6 cm×4 cm肿物, 子宫左后方侧腹膜可见大小约3.5 cm×2 cm×2 cm肿物, 术后病理检查示:梭形细胞肿瘤, 细胞形态温和, 胞质红染, 富于扩张的血管, 倾向平滑肌瘤(图2)。免疫组化检查示:Dog-1(-)、CD117(-)、CD34(-)、Actin(+)、Desmin(+)、SMA(+)、Ki-67阳性率<1%, 雌激素受体(+)、孕激素受体(+)(图3, 4), 结果支持平滑肌肿瘤。结合临床病史, 诊断为良性转移性平滑肌瘤(benign metastasizing leiomyoma, BML), 术后随访3年患者病情稳定。     

精索侵袭性血管黏液瘤1例报告并文献复习

目的:分析1例精索侵袭性血管黏液瘤的临床表现、影像学和病理学特点以探讨其诊疗方式及预后。方法:回顾性分析我院2014年1月收治的1例精索侵袭性血管黏液瘤患者的临床资料,患者术前无特异性临床表现,术前诊断为阴囊肿物,遂行阴囊肿物切除术。结果:术后病理检查诊断为侵袭性血管黏液瘤,随访24个月肿瘤无复发和转移。结论:精索侵袭性血管黏液瘤较为罕见仅能依靠病理检查确诊;临床上容易与其他阴囊肿瘤混淆,手术切除是目前最为有效的治疗方式.因为该肿瘤的高复发性,需密切随访。     

附睾旁复发性侵袭性血管黏液瘤一例报告

患者,男,41岁。因发现右侧阴囊内无痛性肿物2年于2004年9月6日入院。6年前因右阴囊肿物在当地医院行肿物切除术,切除肿物2.0cm×1.0cm×1.0cm。病理诊断:侵袭性血管黏液瘤。体检:阴囊无红肿,右附睾上极可触及1.5cm×1.5cm×1.0cm和1.0cm×0.5cm×0.5cm肿物2枚,质韧,表面光滑,与阴囊无粘连,无触痛,透光实验(-),对侧睾丸无异常。腹部B超未见异常。临床诊断:右附睾旁侵袭性血管黏液瘤。持续硬膜外麻醉下行肿瘤切除术,肿物境界清,包膜完整。病理报告:送检组织部分有被膜,表面光滑,切面灰红色,质地较软,部分胶冻状,内含黏液样物质。镜检:瘤细胞…     

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侵袭性血管黏液瘤是一种罕见的软组织肿瘤,新疆维吾尔自治区中医医院于2011年3月收治1例,行手术治疗。报告如下。1病历简介病人女性,75岁。1999年因右大腿前侧肿块在外院行肿物切除术,病理诊断为神经纤维腺瘤。术后7年原位复发,外院再次手术切除,由于医院条件有限未做病理检     

Aggressive angiomyxoma of the pelvis and perineum: a case report and review of the literature

Aggressive angiomyxoma is a rare mesenchymal tumour arising from soft tissue of the pelvis and perineum. Other features of this tumour are non-specific clinical signs, local invasion, a high local recurrence rate, and recurrence long after initial excision. We provide a case report with a review of the literature. A 57-year-old woman with a large bulky mass in the perineum was admitted to our institution. She had previously undergone iterative surgery for excision of pelvic and perineal masses. The preoperative diagnostic procedure is described as well as the therapeutic surgical approach (a double simultaneous laparotomic and perineal approach was opted for). The surgical procedure was difficult, time-consuming and dangerous owing to an extensive area of hard retroperitoneal sclerosis involving the low urinary viscera, the vaginal stump and the mesorectum. Radical excision of the mass favoured the re-establishment of the normal pelvic anatomy, resolution of the preoperative symptoms and complete recovery of working capability. Surgical excision is the gold standard in the treatment of aggressive angiomyxoma. This benign neoplasm may sometimes present a malignant course owing to involvement of pelvic viscera. It is also associated with a high late recurrence rate due to local aggressiveness, and longterm follow-up is therefore necessary.     

Aggressive angiomyxoma of the pelvis surgical management in a case with delayed diagnosis

Introduction and importanceAggressive angiomyxoma is characterized as a non-capsulated soft mass with the ability to progress to surrounding tissues but without metastasis to distant tissues. Slowing tumor extension leading delayed tumor diagnosis, expression of different types of hormonal receptors, therapeutic ineffectiveness of noninvasive treatment approaches and misdiagnosis have remained as the major challenges for managing this tumor.Case presentationHerein, we described a case of aggressive angiomyxoma located in the posterior of the uterus and vagina that as successfully managed surgically to remove tumor mass followed by gonadotropin-releasing hormone (GnRH) agonist to prevent tumor recurrence.Clinical discussionSurgical resection is the treatment of choice in aggressive angiomyxoma with complete success rate, however despite such successfulness, about two-thirds of patients experienced postoperative recurrence rate that could be prevented by hormone-based therapy especially GnRH agonist.ConclusionAggressive angiomyxoma is a rare tumor with locally invasive behavior. As misdiagnosis is common imaging like MRI with DWI should be considered. The best treatment is surgical resection by experienced surgeons in tertiary referral hospitals. Even with complete resection, the recurrence rate is high. So adjuvant medical treatment seems to be necessary.     

Aggressive angiomyxoma of the scrotum

Aggressive angiomyxoma is a rare and nonmetastasizing soft tissue tumor of the pelvis and perineum and occurs almost exclusively in adult females. It infiltrates locally and has a high risk of local recurrence. Recommended treatment of the symptomatic patient is wide excision with tumor-free margins and close postoperative monitoring. Herein, a case of aggressive angiomyxoma in an adult male is described, which arose in the scrotum over 12 months. The tumor showed an intermediate signal on T1-weighted MRI images. Contrast-enhanced T1-weighted images showed good enhancement. Wide excision of the tumor was performed. The surgical specimen measured 7 x 5 x 5 cm in size and weighed 80 g. The tumor's surface was smooth and had a gelatinous cut surface. Grossly, it was encapsulated with a pleura-like membrane and had a finger-like projection. Microscopically, sections showed many walled vessels of various sizes, collagen fibrils, a loose myxoid background. and spindle stroma cells. MRI and CT showed the angiomatous and myxomatous nature of the tumor wall. To the authors' knowledge, this is the first report to describe MRI findings in scrotal angiomyxoma.     

成人肾恶性横纹肌样瘤15例临床病理及免疫表型分析

目的：探讨成人肾恶性横纹肌样瘤（MRTK）的病理组织学,分子免疫表型的特点及临床意义.方法：对我院1983～2009年收治15例肾脏恶性横纹肌样瘤的石蜡组织切片运用SP法进行免疫组化染色,检测Vi-mentin、Myoglobin、Desmin、α-SMA、EMA、NSE、S-100的表达,并结合临床病理特征进行分析.结果：组织学上瘤细胞胞界清楚.胞浆丰富具有嗜酸性颗粒、常见大的嗜酸性包涵体,呈圆形或椭圆形,将细胞核挤向一侧.核膜明显而核仁突出,核分裂多见.经SP染色后,Vimentin均警阳性或强阳性表达,EMA、NSE和S-100均有不同程度的阳性表达,α-SMA有2例呈阳性表达,而Myoglobin、Desmin均呈阴性表达.结论：成人MRTK是一种表达多种免疫抗原,独立的可多向分化的肾恶性肿瘤,Vimentin和Myoglobin、Desmin、α-SMA有助于MRTK的诊断和鉴别诊断.     

Angiomyofibroblastoma-like tumors (cellular angiofibroma)

Angiomyofibroblastoma-like tumor (cellular angiofibroma) is a rare, circumscribed, slow-growing mesenchymal tumor that occurs predominantly in the vulva, perineum, and pelvis of women. We report two cases of this tumor in men arising as paratesticular masses of the scrotum, summarize the history of this tumor, and discuss why efforts should be made to differentiate it from aggressive angiomyxoma. Recommended treatment is complete surgical excision with long-term follow up exams, as local recurrence may occur many years after resection of the lesion.     

Deep (aggressive) angiomyxoma of the vagina misdiagnosed as Gartner cyst: A case report

Introduction and importanceAggressive angiomyxoma is a rare soft tissue tumor. Aggressive angiomyxoma is a slow-growing vulvovaginal mesenchymal neoplasm with a marked tendency for local recurrence, but with a low tendency to metastasize. As it has a predilection for the pelvic and perineal regions, Aggressive angiomyxoma is often misdiagnosed.This case report documented rare case of misdiagnosed Aggressive Angiomyxoma as Gartner duct cyst.Presentation of caseThis article report a case of 31 year old women who complained mass came out from vagina without any urinary symptom and trauma. Physical examination and ultrasound finding suggested that the mass was Gartner Duct cyst. Management in this case was excision of the vaginal cyst. Histopathology examination revealed Deep (aggressive) angiomyxoma.DiscussionThe rarity of Deep (Aggressive) Angiomyxoma makes the preoperative diagnosis fairly difficult. Aggressive angiomyxoma is often misdiagnosed as it may have similar clinical presentation to common lesions such as Bartholin cyst or prolapse vaginal wall, Gartner cyst or levator hernia. Aggressive Angiomyxoma should be considered as differential diagnosis in patient with vaginal cyst.ConclusionAggressive Angiomyxoma is rare condition. Preoperative diagnosis and management are challenging. Long term follow op and evaluation should be done due to high rate of recurrence.     

Periurethral aggressive angiomyxoma: a case report

Aggressive angiomyxoma is a rare soft tissue tumor of the pelvis. Notorious for its locally infiltrative behavior, this tumor is identified by pathological appearance. Grossly gelatinous, spindle cells widely separated by collagen fibrils with vascular components can be microscopically visualized. Wide local excision is the treatment of choice. A 19-year-old woman presented with a periurethral mass that extended beyond the hymen with Valsalva. With imaging, the differential was narrowed to a soft tissue mass. Surgical excision was performed and histopathological findings were consistent with the diagnosis of aggressive angiomyxoma. Twenty-four months later the patient remains recurrence free. The typical findings of aggressive angiomyxoma are highlighted as well as the novel presentation as a periurethral mass. Histological and radiological findings are reviewed as well as current treatment options.     

Local recurrent aggressive angiomyxoma presenting as a para urethral tumor 64 months after the first resection: a case report

We report a case of local recurrent aggressive angiomyxoma presenting as a para-urethral tumor. A 28-year-old woman visited our hospital with a complaint of a painless vulval mass. Magnetic resonance imaging (MRI) of the pelvis showed the para-urethral tumor to be 2.5 x 3.0 cm. The tumor was resected, and diagnosed histopathologically as aggressive angiomyxoma. The patient showed a painless vulval mass again at 64 months after the first resection. MRI of the pelvis showed the paraurethral tumor to be 2.5 x 3.0 cm. The tumor was resected, and diagnosed histopathologically as aggressive angiomyxoma. The patient showed no recurrence at 4 months after the second resection.     

Medical management of recurrent aggressive angiomyxoma with gonadotropin-releasing hormone agonist

Patients with aggressive angiomyxoma may experience local recurrences. We report a case of recurrent aggressive angiomyxoma medically treated successfully with a gonadotropin-releasing hormone agonist. A 34-year-old woman with a huge perineal tumor underwent an extensive resection of the abdominoperineal tumor combined with total pelvic exenteration. Histology showed aggressive angiomyxoma and the tumor cells were immunoreactive for estrogen and progesterone receptors. Although the patient had experienced no local recurrence for 12 months under adjuvant therapy with a gonadotropin-releasing hormone agonist, a recurrence occurred 3 months after the completion of adjuvant therapy. The patient underwent medical treatment with a gonadotropin-releasing hormone agonist and had a complete resolution of the recurrent tumor again. Hormonal treatment with a gonadotropin-releasing hormone agonist can be applied for small primary aggressive angiomyxomas in addition to adjuvant therapy for residual tumors.