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.

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.

Giant cell angiofibroma in the vocal cord

Giant cell angiofibroma （GCA） was first described in 1995, and it is a rare tumor in soft tissues,1 Since the initial report of the disease in orbital tissue, GCA has been reported in a variety of other soft tissues.2,3 The morphological hallmark is richly vascularized, patternless spindle cell proliferation in the presence of pseudovascular spaces and multinucleated giant cells. The pathological diagnosis of GCA is mainly based on positive staining of CD34. GCA is usually considered to be a benign tumor. In clinic, GCA needs to be distinguished from other tumors in soft tissues. Herein,we report two rare cases of GCA in the vocal cord and describe its histological features.

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。

Topical rapamycin for angiofibromas in patients with tuberous sclerosis:how does it work in clinical practice?

Aim:Topical rapamycin for angiofibromas has been reported to be a new promising treatment.This study aims to report the outcome in clinical practice.Methods:A retrospective clinical follow-up on twenty-three patients who had been prescribed an oral solution of 0.1%rapamycin,to be applied on facial lesions once a day.Results:Seventeen of 23 patients continued the treatment.Papules and nodules were improved in 8 patients(47%)and erythema in 12(70%).Side effects,such as stinging and redness were reported in 35%of patients.Blood samples were taken from 5 patients and no rapamycin could be detected.All patients who paused the treatment relapsed.Conclusion:Topical rapamycin has a positive effect on angiofibromas with improvement in both erythema and papules even if only applied every second to third day,but continuous treatment is needed.

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。

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。

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。

低温等离子联合彭氏多功能手术解剖器治疗鼻咽纤维血管瘤的临床应用

目的初步探讨低温等离子联合彭氏多功能手术解剖器(PMOD)治疗鼻咽纤维血管瘤的临床疗效。方法回顾性分析广西中医药大学附属瑞康医院耳鼻咽喉头颈外科鼻内镜引导下应用低温等离子联合PMOD对11例鼻咽纤维血管瘤患者行手术治疗的临床资料。结果所有患者均一次完成手术,术中出血量100~1200 ml,平均出血量500 ml,术后病理均确诊为鼻咽纤维血管瘤。术后1例患者出现复视,眼球外展困难,予以激素及对症保守治疗,眼球外展稍改善出院,术后3个月复查复视消失,眼球活动正常。1例术后随访1年时局部复发,予以再次手术治疗并随访1年未见复发,余病例术后随访12~48个月均未见肿瘤复发。结论鼻内镜引导下低温等离子技术联合PMOD可有效的切除鼻咽纤维血管瘤,手术安全性较高,并发症相对较小,是目前治疗鼻咽纤维血管瘤较好的手术方式,值得临床推广及应用。

鼻内镜下切除鼻咽血管纤维瘤临床探讨

青少年鼻咽血管纤维瘤（juvenile nasopharyngeal angiofibroma，JNA）的起源部位深在、血管供血丰富、周围解剖关系复杂，一度被视为鼻内镜手术的禁忌。而随着鼻内镜外科技术的成熟和影像介入技术的广泛开展，鼻内镜下切除鼻咽血管纤维瘤已经取得了一些成功经验。自2004年6月-2006年2月我科开展4例经鼻内镜鼻咽血管纤维瘤切除手术，获得满意疗效，现报道如下。

鼻腔纤维血管瘤合并灭鼠药误服致复杂出血1例

鼻出血是耳鼻咽喉科最常见的急症之一,病因多样,可突发于各年龄段,出血量多少不一,轻者仅表现涕中带血,重者可引起失血性休克,临床明确病因,及时诊疗处理非常关键。2013年3月我院收治1例复杂的急性鼻出血,现报道如下。1临床资料患儿,男,13岁,因尿红5 d,于儿童肾内科收入院。入院查体：体温36.9℃,脉搏111次/分,呼吸20次/分,血压126/87 mm Hg,双肾区叩击痛阳性,左侧胫骨及腘窝处轻触痛,无肿胀,余四肢关节无明显触痛,尿道口无充血,余无异常。血常规：红细胞（RBC）4.2×10^12/L,血红蛋白（HB）116 g/L,白细胞（WBC）8×10^9/L,血小板（PLT）269×10^9/L。

鼻咽血管纤维瘤

鼻咽血管纤维瘤好发青春期男性,是高度血管化良性肿瘤,手术治疗为主。我们应用头颈部血管瘤介入技术和鼻内镜及其延伸技术,对我院2003年1月～2006年1月鼻咽血管纤维瘤患者分析如下。

误诊为鼻咽纤维血管瘤的翼腭窝神经鞘瘤1例

1临床资料患者女,19岁,主因左侧鼻塞2年余于2011年2月入院。患者2年前无明显诱因出现左侧鼻塞,呈持续性并渐加重,抗炎治疗无效（具体药物不详）,无鼻出血、面部麻木、肿胀、头痛等症状。专科检查：左侧鼻腔后部可见红色肿块,表面光滑,周围少许脓性分泌物。影像学检查：CT扫描可见鼻腔后部、后组筛窦、蝶窦及翼腭窝区域软组织密度影,呈膨胀性生长,蝶窦下壁、蝶骨大翼、

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。