angiofibroma

血管纤维瘤
  • 文章类型: English Abstract
    Objective:To summarize the procedures and efficacy of surgical treatment for Andrew stage Ⅰ-Ⅲ juvenile nasopharyngeal angiofibroma(JNA). Methods:A total of 12 patients with JNA who underwent surgery from 2016 to 2021 were enrolled, including 1 case in stage Ⅰ, 3 cases in stage Ⅱ, and 8 cases in stage Ⅲ. JNA was resected by transnasal endoscopic approach alone, or combined with transoral approach or Caldwell-Luc approach was performed. Results:Eleven cases underwent complete resection without recurrence and 1 case had residual tumor. There were no serious complications. The median intraoperative blood loss was 200 mL, and 1 patient received blood transfusion. The median operative time was 110 minutes. Conclusion:JNA in Andrew stage Ⅰ-Ⅲ can be quickly and completely resected by standardized surgical procedures using endoscopy and coblation technology.
    目的:总结AndrewⅠ~Ⅲ期鼻咽纤维血管瘤的手术方法和疗效。 方法:收集2016年1月-2021年12月手术的鼻咽纤维血管瘤患者12例,Ⅰ期1例,Ⅱ期3例,Ⅲ期8例。采用单独经鼻内镜,或联合经口或柯陆氏入路鼻咽纤维血管瘤切除术。 结果:手术未出现严重并发症。术中中位数出血量为200 mL,1例输血。中位数手术时间为110 min。中位数随访时间3年。11例完整切除,术后无复发,1例残留,行二次手术,未再复发残留。 结论:通过建立标准化的手术步骤,借助内镜和低温等离子技术,AndrewⅠ~Ⅲ期的鼻咽纤维血管瘤基本可达到快速、安全、完整切除。.
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    BiZact装置,设计用于扁桃体切除术的双极电刀,最大限度地减少热组织损伤,并密封直径<3毫米的血管,同时分割软组织。这项研究描述了作者使用BiZact进行鼻窦肿瘤手术的经验,并讨论了其临床实用性和优势。作者分析了2021年1月至2023年5月的BiZact辅助内镜下鼻窦肿瘤手术病例。收集了关于患者人口统计学的数据,组织病理学,肿瘤受累的程度,手术记录,和术后医疗记录.使用完整肿瘤切除的成功率评估临床效用,手术期间估计的失血量,装置相关并发症,和操作时间。还对外科医生的BiZact经验进行了调查。本研究中20例患者的诊断包括鳞状细胞癌(n=2),恶性黑色素瘤(n=1),肉瘤(n=1),自然杀伤细胞淋巴瘤(n=1),内翻性乳头状瘤(n=12),血管纤维瘤(n=2),和神经鞘瘤(n=1)。这项初步研究表明手术时间缩短,中位数为0.8小时,术中失血<100mL。此外,未观察到BiZact相关并发症。BiZact装置允许有效的鼻窦手术,因为它具有一步密封和切割的独特优势。BiZact辅助内窥镜鼻窦肿瘤手术是一种有益且安全的手术,可减少手术过程中的失血,缩短手术时间,减少术后并发症。
    The BiZact device, a bipolar electrosurgical scissor designed for tonsillectomy, minimizes thermal tissue damage and seals blood vessels <3 mm in diameter while dividing the soft tissue. This study describes the authors\' experience with sinonasal tumor surgery using a BiZact and discusses its clinical utility and advantages. The authors analyzed BiZact-assisted endoscopic sinonasal tumor surgery cases between January 2021 and May 2023. Data were collected on patients\' demographics, histopathology, extent of tumor involvement, surgical records, and postoperative medical records. Clinical utility was assessed using the success rate of complete tumor excision, estimated blood loss during surgery, device-related complications, and operation time. A survey of the surgeons\' BiZact experience was also conducted. The diagnoses of the 20 patients in this study included squamous cell carcinoma (n = 2), malignant melanoma (n = 1), sarcoma (n = 1), natural killer cell lymphoma (n = 1), inverted papilloma (n = 12), angiofibroma (n = 2), and schwannoma (n = 1). This pilot study demonstrated a shortened operative time, with a median of 0.8 hours and <100 mL of intraoperative blood loss. In addition, no BiZact-related complications were observed. The BiZact device allows efficient sinonasal surgery because it has the unique advantage of one-step sealing and cutting. BiZact-assisted endoscopic sinonasal tumor surgery is a beneficial and safe procedure that reduces blood loss during surgery, shortens the operative time, and minimizes postoperative complications.
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  • 文章类型: Case Reports
    背景:直肠皮瘘很常见。感染起源于肛门腺内,随后扩展到相邻区域,最终导致瘘管的发展。细胞性血管纤维瘤(CAF),也被称为血管肌纤维母细胞瘤样肿瘤,是一种罕见的良性软组织肿瘤,主要在阴囊中观察到,会阴,男性腹股沟区和女性外阴区。我们描述了第一个记录的病例CAF,该病例在直肠皮肤瘘中发展并表现为会阴肿块。
    方法:在门诊,一名52岁的男性患者,有2年的会阴肿块病史,伴有阵痛和轻微的阴囊擦伤。体检显示,定义明确,左臀部向会阴延伸的非触痛肿块,没有可见的开口。最初的评估确定了一个软组织肿瘤,实验室数据在正常范围内。腹部和盆腔计算机断层扫描(CT)显示脓肿腔肿胀,与直肠皮肤瘘有关,左侧会阴区域有一条6cm×0.7cm的径迹样病变,并附着在左侧直肠上。直肠镜检查未发现明显的内孔。左臀侧切口显示厚壁肿块,随着延伸的管道被切除,进行了刮宫。组织病理学检查证实CAF诊断。患者在随访评估期间达到了总分辨率,不需要额外住院。
    结论:CT成像支持会阴病变的诊断和治疗。会阴血管纤维瘤,即使有皮肤瘘,可以经会阴切除。
    BACKGROUND: Rectocutaneous fistulae are common. The infection originates within the anal glands and subsequently extends into adjacent regions, ultimately resulting in fistula development. Cellular angiofibroma (CAF), also known as an angiomyofibroblastoma-like tumor, is a rare benign soft tissue neoplasm predominantly observed in the scrotum, perineum, and inguinal area in males and in the vulva in females. We describe the first documented case CAF that developed within a rectocutaneous fistula and manifested as a perineal mass.
    METHODS: In the outpatient setting, a 52-year-old male patient presented with a 2-year history of a growing perineal mass, accompanied by throbbing pain and minor scrotal abrasion. Physical examination revealed a soft, well-defined, non-tender mass at the left buttock that extended towards the perineum, without a visible opening. The initial assessment identified a soft tissue tumor, and the laboratory data were within normal ranges. Abdominal and pelvic computed tomography (CT) revealed swelling of the abscess cavity that was linked to a rectal cutaneous fistula, with a track-like lesion measuring 6 cm × 0.7 cm in the left perineal region and attached to the left rectum. Rectoscope examination found no significant inner orifices. A left medial gluteal incision revealed a thick-walled mass, which was excised along with the extending tract, and curettage was performed. Histopathological examination confirmed CAF diagnosis. The patient achieved total resolution during follow-up assessments and did not require additional hospitalization.
    CONCLUSIONS: CT imaging supports perineal lesion diagnosis and management. Perineal angiofibromas, even with a cutaneous fistula, can be excised transperineally.
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  • 文章类型: Case Reports
    髓外浆细胞瘤(EMP)是一种罕见的肿瘤,其特征是浆细胞的单克隆生长,而没有多发性骨髓瘤的特征。EMP占所有浆细胞肿瘤的3%。一名89岁的男性高血压患者因单侧鼻出血不受控制而入院。经过一年半的反复发作,患者的出血变得更加频繁,不能再通过鼻腔填塞来控制。怀疑血管纤维瘤是最初的鉴别诊断,并进行血管纤维瘤栓塞术。患者出现吞咽困难和言语不清,并被诊断为缺血性中风。然而,抗血小板药物和氨甲环酸药物因出血风险而被禁用.患者接受了功能性内窥镜鼻窦手术,出乎意料的是,组织学结果显示浆细胞瘤。手术后,患者保持稳定,出院接受进一步治疗.
    Extramedullary plasmacytoma (EMP) is an uncommon tumor marked by the monoclonal growth of plasma cells without the characteristics of multiple myeloma. EMP represents 3% of all plasma cell tumors. An 89-year-old male patient with hypertension was admitted to our tertiary care hospital with uncontrolled unilateral epistaxis. After a year and a half of recurring epistaxis, the patient\'s bleeding became more frequent and could no longer be controlled with nasal packing. Angiofibroma was suspected as the initial differential diagnosis, and angiofibroma embolization was performed. The patient experienced difficulty swallowing and slurred speech and was diagnosed with an ischemic stroke. However, antiplatelet and tranexamic acid medications were contraindicated due to bleeding risks. The patient underwent functional endoscopic sinus surgery, and unexpectedly, histology results revealed a plasmacytoma. After surgery, the patient remained stable and was discharged for further management.
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  • 文章类型: Journal Article
    面部血管纤维瘤指数(IFA),一种新的血管纤维瘤评分系统,已在结节性硬化症(TSC)患者中得到验证。该分析的目的是使用来自TSC患者的局部西罗莫司的临床试验的数据进一步验证IFA。这是对来自在日本进行的III期试验(NCT02635789)的照片的分析。患者(n=62)以1:1随机分配接受西罗莫司或安慰剂凝胶12周。使用主要复合终点独立评估血管纤维瘤的变化,面部血管纤维瘤严重程度指数(FASI),和IFA。使用接受者操作特征(ROC)分析评估IFA评分的临床意义变化的阈值。IFA分数具有良好到优异的评估者间可靠性,非常高的评估员内部可靠性,并可用于评估基线时疾病严重程度的分布。在IFA评分的基线分类变化与主要复合终点之间观察到高度相关(Kendall的一致性系数,W=0.8655,p<0.0001),以及IFA和FASI分数从基线的变化(肯德尔的协调系数,W=0.745,p<0.0001)。通过ROC分析,我们确定了最佳IFA临界点1.667,以将血管纤维瘤显著改善或改善的患者与由主要复合终点确定的血管纤维瘤轻微改善或未改变的患者(曲线下面积0.937)区分开.IFA评分由于其高有效性和可靠性而可能在临床上有用。评分从基线下降≥1.667可能被认为是有临床意义的。
    The Index for Facial Angiofibromas (IFA), a novel scoring system for angiofibromas, has been validated in patients with tuberous sclerosis complex (TSC). The objective of this analysis was to further validate the IFA using data from a clinical trial of topical sirolimus in patients with TSC. This was an analysis of photographs from a Phase III trial conducted in Japan (NCT02635789). Patients (n = 62) were randomized 1:1 to receive sirolimus or placebo gel for 12 weeks. Changes in angiofibromas were independently assessed using the primary composite endpoint, the Facial Angiofibroma Severity Index (FASI), and the IFA. Thresholds for a clinically meaningful change in IFA score were evaluated using receiver operating characteristic (ROC) analysis. The IFA scores had good-to-excellent inter-assessor reliability, very high intra-assessor reliability, and could be used to evaluate the distribution of disease severity at baseline. High correlations were observed between the categorized change from baseline in IFA scores and the primary composite endpoint (Kendall\'s coefficient of concordance, W = 0.8655, p < 0.0001), and between the change from baseline in IFA and FASI scores (Kendall\'s coefficient of concordance, W = 0.745, p < 0.0001). By ROC analysis, an optimal IFA cut-off point of 1.667 was determined to distinguish patients with markedly improved or improved angiofibromas from those with slightly improved or unchanged angiofibromas (area under the curve 0.937) as determined by the primary composite endpoint. The IFA score is potentially clinically useful because of its high validity and reliability. A decrease in score from baseline of ≥1.667 may be considered clinically meaningful.
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  • 文章类型: Case Reports
    背景:睾丸旁细胞血管纤维瘤是一种罕见的良性间叶性肿瘤。由于术前难以准确诊断,因此最佳的治疗方法是手术切除。
    方法:一名51岁的日本男性到我院就诊,抱怨无症状的左阴囊肿胀。体格检查显示无弹性的睾丸旁肿块(直径5.5厘米)。虽然睾丸生殖细胞肿瘤在临床上被排除,肿瘤的恶性可能性仍然存在。既然病人同意完全切除,进行了经腹股沟根治性睾丸切除术.病理诊断为细胞性血管纤维瘤。患者康复,无围手术期并发症,术后5年无明显复发。
    结论:病理结果与细胞性血管纤维瘤相符。肿瘤被成功切除,术后5年无明显复发。
    BACKGROUND: Paratesticular cellular angiofibroma is a rare benign mesenchymal tumor. The optimal management is surgical resection due to the difficulty of preoperative accurate diagnosis.
    METHODS: A 51-year-old Japanese male visited our hospital complaining of asymptomatic left scrotal swelling. Physical examination revealed a nontender elastic paratesticular mass (5.5 cm in diameter). Although testicular germ cell tumor was ruled out clinically, the possibility of malignant potential remained for the tumor. Since the patient consented to complete resection, a transinguinal radical orchiectomy was performed. The pathological diagnosis revealed cellular angiofibroma. The patient recovered without perioperative complications, and no apparent recurrence was observed at 5 years after surgery.
    CONCLUSIONS: The pathological findings were compatible for cellular angiofibroma. The tumor was successfully resected, and no apparent recurrence was observed at 5 years after surgery.
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  • 文章类型: Journal Article
    通过CD34免疫染色评估青少年鼻咽血管纤维瘤(JNA)的微血管密度(MVD),并评估其与临床人口统计学特征的关系。
    这项前瞻性研究包括接受内镜切除的JNA患者。使用CD-34抗体对组织病理学样本进行染色以计算MVD。MVD与临床人口学特征相关。
    该研究包括12名患者,中位年龄为15.5岁。平均MVD为39个血管/高功率场(范围5至151个血管)。MVD仅与肿瘤体积显着相关(r=0.65,p=0.02)。复发发生在一名MVD为107的患者中。中位随访时间为38个月。
    MVD与JNA肿瘤体积显著相关,这意味着血管生成在肿瘤病理中的强大作用。此外,较高的MVD可能是复发的危险因素.
    UNASSIGNED: To assess the microvascular density (MVD) in juvenile nasopharyngeal angiofibroma (JNA) with CD34 immunostaining and evaluate its relationship with clinico-demographic features.
    UNASSIGNED: This prospective study included patients with JNA undergoing endoscopic excision. The histopathological specimen was stained using CD-34 antibodies to calculate MVD. MVD and clinico-demographic features were correlated.
    UNASSIGNED: The study included 12 patients with a median age of 15.5 years. The mean MVD was 39 vessels/high power field (range 5 to 151 vessels). MVD was significantly associated only with the volume of tumour (r = 0.65, p = 0.02). The recurrence occurred in one patient with an MVD of 107. The median follow-up was 38 months.
    UNASSIGNED: MVD is significantly associated with tumour volume in JNA, which implies a robust role of angiogenesis in the pathology of the tumour. Also, higher MVD may be a risk factor for recurrence.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    我们报告了第一例由颈内动脉(ICA)的多个动脉喂养的青少年鼻血管纤维瘤(JNA),用两个气球暂时阻塞ICA,无并发症。患有JNA的早期青少年在术前栓塞了由颈外动脉(ECA)引起的供血动脉(匹兹堡大学医学中心分类IV)。尝试一次内镜切除,但由于大量出血(7000mL)而中断。17个月后,JNA已经生长到充满两个鼻腔。对ECA的进料器进行了重复的术前栓塞,然后手术结合内窥镜和外切口。术中,两个气球被插入右ICA,在饲养血管的近端和远端膨胀,以切断流向肿瘤的血流。肿瘤几乎完全切除,失血6270mL,术后无神经功能恶化。
    We report the first case of a juvenile nasal angiofibroma (JNA) fed by multiple arteries from the internal carotid artery (ICA), removed without complications by temporarily blocking the ICA with two balloons. An early adolescent with JNA underwent preoperative embolisation of feeding arteries arising from the external carotid artery (ECA) (University of Pittsburgh Medical Centre classification IV). Endoscopic resection was attempted once but discontinued due to massive bleeding (7000 mL). 17 months later, the JNA had grown to fill both nasal cavities. Repeated preoperative embolisation of the feeders from the ECA was performed, followed by surgery combined with endoscopic and external incision. Intraoperatively, two balloons were inserted into the right ICA, which were inflated at the proximal and distal sites of the feeder vessels to cut-off blood flow to the tumour. The tumour was almost completely resected with 6270 mL of blood loss and no postoperative neurological deterioration.
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