Cellular angiofibroma arising from the rectocutaneous fistula in an adult: A case report

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 angiomy ofibroblastoma-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.CASE SUMMARY 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.CONCLUSION CT imaging supports perineal lesion diagnosis and management.Perineal angiofibromas,even with a cutaneous fistula,can be excised transperineally.

Three-Dimensional Conformal and Intensity Modulated Dynamic Radiotherapy in Juvenile Nasopharyngeal Angiofibroma

Objective: Juvenile Nasopharyngeal Angiofibroma (JNA) is a benign neoplasm with a high vascularity component, greater craniofacial involvement in adolescent patients, and aggressive local behaviour. In unresectable patients, radiotherapy is a therapeutic option for local control. Our aim in this study was to analyze the clinical benefit and local control provided by two modalities of radiotherapy: the Three-Dimensional Conformal (3DC) technique and volumetric modulated arc therapy (VMAT), applied to pediatric patients with JNA considered unresectable and non-recurrent. Methods: In retrospective study, the information was recorded from pediatric patients with a diagnosis of non-recurrent and unresectable JNA treated with radiotherapy at the Oncology Hospital of the National Medical Center SXXI of Mexico City, from March 2010 to March 2021. Radiotherapy management and its association with clinical outcomes of tumour control, and symptoms were assessed. In addition, an evaluation of acute and chronic toxicity was performed. Results: It was found that the median age was 14 years. 9 patients (37.5%) underwent 3DC and 15 (62.5%) VMAT. In terms of local control, and progression-free survival, we did not find significant difference between radiotherapy modalities (p ≤ 0.57). Acute toxicity for both modalities presented statistical differences for radio epithelitis (p = 0.03). Only Grade I and II radiation-induced acute toxicity was observed. Regarding chronic toxicity, statistical significance was observed for craniofacial hypoplasia, in relation to its absence in the VMAT group (p = 0.001). Conclusion: The VMAT presents improvements in dosimetry parameters that improve patient toxicity. In both techniques adequate tumour control was observed, however, the rarity of the disease is a limitation to establish the most appropriate therapeutic technique.

Application of Superselective Artery Embolization in Nasopharyngeal Angiofibroma before Operation

Objective： To evaluate the clinical application of superselective transcatheter arterial embolization （TAE） in nasopharyngeal angiofibroma before operation. Methods： Superselective angiography was done via femoral. TAE with polyvinyl alcohol particles and/or gelfoam （GF） was performed in 12 patients with nasopharyngeal angiofibroma. All patients underwent surgical removal of devascularized tumors in 3 to 7 days after TAE. Results： During digital subtraction angiography, tumor staining was seen in 12 patients. Embolization of maxillary artery was performed in 12 cases and additional embolization of ascending pharyngeal artery in 8 cases. Conclusion： Superselective angiographic diagnosis, embolization and appropriate embolic particle size are important for successful treatment of nasopharyngeal angiofibroma. TAE is safe and effective in decreasing haemorrhage during surgical operation for nasopharyngeal angiofibroma.

右肩部软组织血管纤维瘤一例

软组织血管纤维瘤是较为罕见的软组织来源的肿瘤,女性相对好发,临床表现及影像学表现无明显特异性,我们报道此病例,旨在提高对该病例的认识。

Combined Treatment with Electrocauterization,Carbon Dioxide Laser,and Microneedle Fractional Radiofrequency for Facial Angiofibromas in Tuberous Sclerosis Complex:A Case Report and Literature Review

Tuberous sclerosis complex is a type of genetic multisystem disease that causes hamartomas in various organs.Facial angiofibromas commonly occur in 80%of patients and are prominently distributed over the cheek,chin,and nasolabial folds with severe disfigurement and emotional distress.Recently,photoelectric devices have been identified for the treatment of angiofibromas with great efficacy and fewer side effects.We report a case of a 42-year-old man with facial angiofibromas,who was treated with a combination of high-frequency electrocauterization,Ultrapulse CO_(2) laser,and microneedle fractional radiofrequency with 7 sessions and a 6-month follow-up.The patient showed great improvement in relation to the elevated lesions and nodules.A low recurrence rate was observed.This is the first study to investigate the efficacy of high-frequency electrocauterization and microneedle fractional radiofrequency in angiofibromas.It may provide an optimal approach for clinicians wherein a combined treatment of various lasers and electric devices is effective for complicated,protuberant,and firm angiofibromas of specific patients.

Extranasopharyngeal angiofibroma in children:A case report

BACKGROUND Sporadic cases of extranasopharyngeal angiofibroma in children,especially preschool children,have been reported in the literature.CASE SUMMARY We present a case of extranasopharyngeal angiofibroma in a 4-year-old boy.The presenting symptoms,imaging findings,treatment,histological appearance,and follow-up data are described in detail.For this patient,we performed embolization on two occasions,and then,resected the tumor completely.During the treatment,the patient developed a soft-palate perforation due to aseptic necrosis.However,the healing ability was good,and the perforation healed spontaneously.We additionally reviewed all pediatric cases of extranasopharyngeal angiofibroma published up to 30 June 2020 in the PubMed,Baidu Scholar,Scopus,and Web of Science databases.We identified 45 pediatric patients[average(10.98±4.86),boys 39(86.7%)].The highest proportion of cases occurred in adolescence[22(48.9%)].The top three sites of occurrence of extranasopharyngeal angiofibroma in children were the maxillary sinus,nasal septum,and inferior turbinate.CONCLUSION Extranasopharyngeal angiofibromas can occur throughout childhood,and predominantly present with nasal obstruction and spontaneous rhinorrhagia.

Vaginal Cellular Angiofibroma with Heavy Bleeding: A Case Report

Cellular angiofibroma is a rare mesenchymal tumor that most commonly presents as an asymptomatic well-circumscribed mass in the vulva. Very few cases have been found in the vagina and even fewer presented with vaginal bleeding. A 54-year-old female presented to the emergency department with five months of vaginal bleeding and symptomatic anemia requiring blood transfusion. Upon physical examination, she was found to have a firm 4 cm posterior vaginal mass, distinctly distal to the cervix. The mass was resected and diagnosed as a vaginal cellular angiofibroma on pathologic evaluation. The patient did well post-operatively with recommended follow-up every six months. The common differential diagnosis for a bleeding vaginal mass includes leiomyoma, angiofibroblastoma, aggressive angiomyxoma, squamous cell carcinoma, and adenocarcinoma. In this case, cellular angiofibroma presented as a vaginal mass with heavy bleeding leading to severe anemia. Early involvement of gynecologic oncology can help to optimize patients’ evaluation and management.

青少年鼻咽血管纤维瘤中HSP90表达及临床意义

目的探讨青少年鼻咽血管纤维瘤(JNA)中热休克蛋白90(HSP90)的表达及其与肿瘤复发的关联。方法回顾性分析了2018年—2022年在我院接受手术治疗的组织学诊断为JNA的男性患者60例,使用包含60例JNA患者和10例对照受试者的组织微阵列进行免疫组织化学以评估HSP90表达,通过Pearson卡方、Spearman以及单变量和多变量Cox回归分析HSP90表达与临床病理特征和肿瘤复发的关系。结果与正常中鼻甲样本相比,免疫组织化学显示JNA中HSP90高表达。HSP90高表达与微血管密度(MVD)(R=0.379,P=0.001)、雌激素受体α(ER-α)(R=0.396,P=0.001)、血管内皮生长因子(VEGF)(R=0.612,P<0.001)和JNA复发(P=0.011)呈正相关,是复发时间的独立预后因素(HR=3.251,95%CI:1.187-8.634,P=0.012)。结论HSP90可能是接受手术治疗的JNA患者的独立预后标志物。

多次复发的下颌骨软组织血管纤维瘤1例

1临床资料患者,女,66岁,2014-04-09曾因“下颌骨肿胀不适”于外院就诊,CT示“右下颌骨囊肿”,遂在聊城市人民医院行“右下颌骨囊肿刮治术+病灶牙拔除术”。术后病理诊断:囊肿伴间质肉芽组织增生,黏液变性明显。2019-04-12、2019-12-04患者再次先后因“右下颌后牙区肿痛”就诊,CT均提示右侧下颌支膨胀骨破坏,内见类软组织密度影,骨皮质不完整,病变范围约2.4cm×1.4cm。

富于细胞性血管纤维瘤27例的临床病理分析

目的探讨富于细胞性血管纤维瘤的临床特点、病理、免疫组织化学染色情况。方法收集2000年1月至2022年6月中国知网和万方数据库报道的国内26例及清华大学第一附属医院(以下简称“我院”)2022年2月18日收治的1例富于细胞性血管纤维瘤患者临床资料,分析其临床特点、病理学及免疫组化特征。结果我院患者年龄44岁,因“发现外阴肿物3个月,增大伴触痛2周”就诊。肿物位于右侧小阴唇内侧,大小为9 cm,病理镜下表现为短梭形细胞,分布均一,可见较多核分裂象,细胞异型性较大。间质可见中小血管,部分血管壁较厚,伴玻璃样变性。免疫组织化学染色可见CD34(血管+)、Desimn(血管+)、Vimentin(+)、SATA6(-)、S-100(-)、Ki-67指数(30%)、SMA(血管+)、ER(+)、PR(+)。文献检索的26例患者年龄为16~78岁,其中女18例,男8例;无痛21例,轻度疼痛5例;生长部位大阴唇13例,阴囊4例,中指2例,腹股沟2例,阴阜1例,外阴1例,精索1例,小阴唇1例,宫颈1例;病程为1周~10年。病理特征是由形态一致的短梭形细胞和丰富的血管组成,免疫组织化学染色可见瘤细胞Vimentin呈弥漫性阳性,CD34、ER、PR、SMA呈阳性或部分阳性,Desimin、HMB45、S-100大部分呈阴性。文献中26例患者均接受手术治疗,有2例疑似复发,我院1例患者接受手术后未见复发。结论富于细胞性血管纤维瘤是一种罕见的间叶性肿瘤,多发于两性生殖区,其镜下以密集的短梭形细胞和丰富血管为特征,治疗以手术切除为主,患者预后良好。

鼻咽纤维血管瘤术前上颌动脉栓塞的临床价值

目的 探讨鼻咽纤维血管瘤术前栓塞的价值。方法 回顾性分析 13例病理证实的鼻咽纤维血管瘤患者 ,采用 4 .1F或 5FHead Hunter导管行双侧颈动脉造影 ,了解瘤体供血情况后 ,依据供血动脉的粗细 ,选择不同大小的钢圈行纤维血管瘤供血动脉术前栓塞治疗 ,术中以出血量的多少来判断瘤体栓塞效果。结果 钢圈术前栓塞后 ,13例患者均无明显并发症 ,栓塞距手术时间 1～ 4d ,平均 2d行外科手术治疗 ,术中平均出血量为 (5 84 .6± 379.4 )ml(2 5 0～ 15 0 0ml) ,瘤体均完整切除。结论 鼻咽纤维血管瘤术前钢圈栓塞 ,能有效降低术中出血量 ,提高全切率 ,减少并发症。

鼻内窥镜引导下鼻咽血管纤维瘤切除术

目的鼻咽血管纤维瘤是一种较少见的难治性良性肿瘤，为避免常规手术的并发症，采用鼻内窥镜技术辅以动脉血管栓塞、控制性低血压麻醉等治疗８例。方法８例均为男性，平均年龄１６．６岁。全部患者术前均接受了ＣＴ和鼻内窥镜检查；６例在数字减影血管造影下进行了肿瘤供血动脉栓塞；７例全身麻醉术中采用了控制性低血压技术。结果全部患者无并发症；术中平均出血９００ｍｌ；８例平均随访８个月（１～２０个月），１例（１２．５％）术中未能完全切除。结论使用鼻内窥镜技术治疗鼻咽血管纤维瘤，并配合其他一些相关技术，对肿瘤局限在鼻腔、鼻咽腔、筛蝶窦和部分侵及翼腭窝的患者是适宜的。

鼻咽血管纤维瘤术中出血与复发的多因素分析

目的 探讨青少年鼻咽血管纤维瘤的手术效果、影响出血量和复发的因素。方法 回顾性总结解放军总医院 1986～ 1999年收治的 34例经病理证实的青少年鼻咽血管纤维瘤 ,应用SPSSforWindows 10 .0统计软件 ,分析患者的年龄、病程、肿瘤分期、手术次数、围手术期处理和手术进路与手术效果、术中出血量及复发的相关关系。结果 完全切除 30例 ,平均随访 77个月 ,复发 5例 ,复发率为 16 .7%。平均复发时间为 3.2月 (1～ 6月 )。手术中出血量与患者的年龄、病程、肿瘤分期、是否采用术前血管栓塞或颈外动脉结扎及手术进路的选择有相关关系 (P <0 .0 5 ) ,而与手术次数无关 (P>0 .0 5 )。肿瘤复发与肿瘤分期有关 (P <0 .0 5 ) ,与患者的年龄、病程、是否采用术前血管栓塞或颈外动脉结扎、手术进路及手术次数无关 (P >0 .0 5 )。结论 对出血量有明显影响的因素有患者的年龄、病程和肿瘤分期。复发的主要原因是手术中未能彻底切除肿瘤。对于不能切除干净的肿瘤 ,或局限性肿瘤残留 ,小剂量放射治疗 (30Gy)。

鼻内镜下切除侵犯颅底与海绵窦的鼻咽纤维血管瘤二例临床分析

目的通过报道2例经鼻内镜下切除累及颅底、海绵窦的巨大鼻咽纤维血管瘤,探讨鼻内镜下切除该类肿瘤的可行性及手术方法。方法回顾性分析2例侵犯颅底、海绵窦的鼻咽纤维血管瘤患者的临床资料及治疗方案,结合相关文献分析该类型肿瘤的供血特点及鼻内镜的手术要点。结果 2例患者术前未行动脉血管栓塞,术中出血量分别为3 500 ml和3 000 ml,均获得一次性完整切除。分别随访15个月与11个月未见肿瘤复发。结论对于侵犯颅底、海绵窦及多重供血的鼻咽纤维血管瘤,可在充分的术前准备下行鼻内镜下手术切除。

超脉冲二氧化碳激光治疗结节性硬化症患者面部血管纤维瘤3例

报告3例结节性硬化症,评估超脉冲二氧化碳激光对其面部血管纤维瘤的治疗效果。方法:采用超脉冲二氧化碳激光治疗仪,波长为10 600 nm,输出功率10 W,输出时间600μs,由表皮向皮损深处逐层汽化去除病变组织至真皮浅层。每次治疗间隔2周。结果:3例患者对治疗反应较好,面部多数皮损均消退。未见瘢痕及其他不良反应。结论:超脉冲二氧化碳激光可作为结节性硬化症面部血管纤维瘤的一种有效且美容效果较好的治疗手段。

术前IADSA和栓塞术在鼻咽纤维血管瘤治疗中的应用

目的：研究分析术前经动脉数字减影血管造影（IADSA）和栓塞术在鼻咽纤维血管瘤治疗中的价值。方法：分析本院21例鼻咽纤维血管瘤患者的临床治疗资料，1例术后1年复发，再次手术治疗。22次手术中，术前行及未行IADSA和栓塞术的各11次。比较了术前未行和行IADSA和栓塞术的术中平均出血量和输血量。结果：术前未行和行IADSA和栓塞术的术中平均出血量和输血量分别为1136ml、836ml和677ml（P＜005）、400ml（P＜0.01）。结论：术前IADSA加动脉内栓塞可大大减少鼻咽纤维血管瘤术中出血量。

血管内栓塞在鼻咽血管纤维瘤的应用

目的探讨术前超选择性血管内栓塞对鼻咽血管纤维瘤的疗效和安全性。方法在术前3～5d,对21例鼻咽部血管纤维瘤进行DSA检查和微导管超选择栓塞肿瘤供血动脉,栓塞的材料包括PVA颗粒、明胶海绵和真丝线段等。栓塞后行手术切除肿瘤。结果鼻咽血管纤维瘤主要由同侧的颌内动脉供血19例,主要由咽升动脉供血2例,同侧或双侧的颈内动脉参与供血为9例。21例病人均栓塞成功,其中13例肿瘤染色完全消失,8例肿瘤染色大部分或部分消失。肿瘤全切除者19例,部分切除者2例,全切除率为90.5％。术中出血明显减少,术中及术后无严重并发症。结论鼻咽血管纤维瘤的术前栓塞有助于减少术中出血,提高手术安全性及全切率,是一种安全、有效的微侵袭方法。

鼻咽纤维血管瘤鼻内镜下微创手术治疗的临床探讨

目的 探讨鼻咽纤维血管瘤 (JNA)鼻内镜下微创手术治疗的几个临床问题。方法 结合文献复习 ,回顾性总结和分析 4例JNA鼻内镜下微创手术治疗的 ,陆床资料。结果 患者术中失血量与传统手术相近或略为减少 ,无任何围手术期并发症 ,术后随访疗效十分满意。结论 JNA鼻内镜下微创手术治疗安全可行。经颈部小切口颈外动脉暂时性阻断术操作简单 ,费用低廉 ,JNA切除在此手术后立即进行 ,对减少术中失血量效果最佳。充分的手术视野和准确的JNA根蒂定位是JNA鼻内镜下微创手术治疗的关键环节。该术式的适应证尚在继续完善之中。

CT和MRI诊断鼻咽纤维血管瘤术后复发

目的探讨CT和MRI诊断鼻咽纤维血管瘤(JNA)术后复发的应用价值。方法回顾性分析20例JNA术后复发患者的临床资料、CT及MRI表现。结果患者术后均未进行规范性随访,平均于2.4年后症状再次出现时诊为肿瘤复发。CT显示复发肿瘤多以蝶腭孔为中心,广泛累及周围结构,包括颅底及颅内结构。MRI能更准确地显示肿瘤范围。结论采用CT和MRI对JNA术后患者行规范性随访检查对早期发现肿瘤复发极为重要。

鼻咽纤维血管瘤术前血管造影及超选择栓塞治疗

目的探讨鼻咽部纤维血管瘤术前超选择栓塞的临床价值。方法 14例经CT和CTA诊断并为手术病理证实的鼻咽部纤维血管瘤患者,术前全部行双侧颈内、外动脉血管造影。肿瘤均为颈外系统供血,术前使用300~510μm多聚乙烯醇颗粒（PVA）及明胶海绵条超选择性栓塞治疗,栓塞后1~3天行手术切除。结果 14例患者,其中颌内动脉分支供血9例,颌内动脉和咽升动脉分支供血5例。所有患者颈外动脉供血分支用Cobra导管超选择性栓塞,均获得成功。栓塞后,13例肿瘤染色完全消失,1例肿瘤染色大部分消失。无临床并发症出现。结论术前血管内超选择栓塞供血动脉是治疗鼻咽部纤维血管瘤一种安全、有效的辅助方法。