A rare and locally aggressive vascular tumor, juvenile nasopharyngeal angiofibroma (JNA) mostly affects male teenagers. This paper describes a 14-year-old male patient who presented with lethargy and recurrent nasal bleeding, which are symptoms of JNA. CT and MRI scans confirmed a vascular mass with a significant local invasion originating from the sphenopalatine foramen. After a CT angiography, which revealed the tumor\'s large blood supply and helped with efficient excision, a focused surgical strategy was designed. Histopathology verified the benign nature of the tumor, and the operation was successful and the patient had a smooth recovery. This case adds to the little literature on JNA. It highlights the need for healthcare professionals to be aware of the requirement of early identification and careful presurgical preparation in managing the illness.

Background Surgical excision is the primary treatment for juvenile nasopharyngeal angiofibroma (JNA), but this procedure is challenging due to its high vascularity and local aggressiveness. Moreover, preoperative embolization is a subject of debate. Objective The objective of this study is to assess the efficacy, safety, and feasibility of endoscope-assisted excision as a surgical intervention for non-embolized advanced JNA. Materials and methods This case series involved six male patients (mean age: 16 years) with JNA, classified as stages Ⅱc to Ⅲb according to the Radkowski classification. None underwent preoperative embolization. Results Two stage Ⅱc cases underwent total endoscopic endonasal excision. One patient with stage Ⅲa and another with stage Ⅲb underwent surgery via an endoscope-assisted sublabial approach. Two patients, one with stage Ⅱc JNA and another with Ⅲb, underwent a two-stage procedure. Postoperative CT scans showed no residual disease at the six-month mark. On average, each procedure required 1.5 units of blood transfusion. One patient experienced intraoperative bleeding, whereas the remaining patients were free of any major complications. The mean operation duration was 175 minutes per procedure. The mean length of stay at the hospital was 3.75 days per procedure. Conclusion Endoscope-assisted or purely endoscopic approaches can be safely and effectively employed for the complete excision of non-embolized advanced JNAs.

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。.

Onyx is a safe and effective embolic agent to utilize in the treatment paradigm of JNA. We present a tandem approach that combines trans-arterial embolization (TAE) with direct puncture embolization (DPE) with Onyx to limit blood loss and facilitate safe resection. Laryngoscope, 134:3568-3571, 2024.

Surgery for excision of juvenile nasopharyngeal angiofibroma (JNA) carries the possibility of massive life-threatening haemorrhage. Anaesthetic management aims to maintain haemodynamic stability and reduce blood loss. This case series describes the application of the bundled approach as a multimodal blood loss prevention bundle (MBLPB). Twenty patients underwent 23 surgeries with MBLPB. The blood loss and the number of units of blood transfused were recorded. The surgeon satisfaction score was assessed. The median [interquartile range (IQR)] estimated blood loss was 1300 (650-2350) ml. Patients with tumours in stages I and II had a median (IQR) blood loss of 550 (270-750) ml compared to patients with higher grades of tumours (stages III, IV) with a median (IQR) blood loss of 2100 (1300-2500) ml. Median (IQR) units of packed red cells transfused was 1 (0-3). The surgeon\'s satisfaction score was high when MBLPB was applied for JNA. However, it does not appear to reduce blood loss markedly.

BACKGROUND: Juvenile Nasopharyngeal Angiofibroma (JNA) is a fibrovascular tumor of the nasopharynx that classically presents in adolescent males. The reported mean age of onset is between 13 and 22 years old [1-6]. Significant androgen stimulation is hypothesized to explain the strong predisposition for JNA to present in young adolescent males. However, considerable variability in age at diagnosis exists with rare involvement of very young patients incongruent with typical male pubertal growth patterns.
OBJECTIVE: The purpose of this systematic review is to identify cases of early-onset JNA (EOJNA), (defined as age < 10 years) in the literature and to examine the disease characteristics and treatments used in this patient group. A case of a 7 year old boy with EOJNA at our institution is also described and presented.
METHODS: We searched Embase, Cochrane database and MEDLINE from 1996 to February 2021 for studies that reported cases of EOJNA. Relevant clinico-demographic data, disease severity and treatment outcomes were recorded and analyzed using descriptive statistics. We compared our findings with reported means for JNA in all ages.
RESULTS: We identified 29 studies containing a total of 34 cases of EOJNA. The vast majority (31/34) of patients were males and the mean age of diagnosis was 8.15 years old. The most common presenting symptoms were nasal obstruction (65.2%) and epistaxis (60.9%). Patients were most commonly Radkowski stage II (39.4%) and III (39.4%). Primary treatment modalities included open surgery (66.7%), endoscopic surgery (24.2%), and radiotherapy (9.1%). Recurrence was evident in 30%. Radkowski stage and type of treatment did not differ significantly within the EOJNA group (p = 0.440 and p = 0.659, respectively).
CONCLUSIONS: This systematic review suggests that rare cases of EOJNA have distinct disease characteristics. Patients in this cohort appeared to have more advanced disease and higher recurrence rates when compared with reported averages. We hope that this review prompts increased clinical awareness of this potentially more aggressive subtype of JNA. As more cases of EOJNA are reported, a more powered statistical analysis of this cohort would be feasible.

Subglottic stenosis (SGS), the narrowing of the upper trachea, can be an acquired condition in pediatric patients. Presenting with varying degrees of dyspnea and stridor, acquired SGS is most commonly due to intubation. Airway stenosis is often not considered a surgical complication, and no literature on acquired SGS after endoscopic sinus surgery exists. We present a unique case of a 13-year-old male with juvenile nasopharyngeal angiofibroma (JNA), who developed SGS in the setting of progressive dyspnea six weeks after endonasal resection of his mass. He required urgent intubation prior to preoperative embolization and endonasal surgery, which prolonged his total intubation period. After the patient was found to have acquired SGS, he eventually required serial dilation to treat his stenosis. The presentation and operative course of this patient, along with images and pathologic findings, are discussed. Based on an extensive literature review of PubMed, Medline, and Google Scholar, there have been no cases discussing SGS development post-intubation after endonasal surgery or in association with JNA. Acquired SGS can present as a life-threatening airway obstruction in pediatric patients. With the rise of endoscopic skull base surgery and the prevalence of JNA, this case study sheds light on the detection and management of SGS post-operatively.

A 54-year-old female presented to the otolaryngology (ENT) outpatient department with an eight-month history of unilateral nasal obstruction and headache. There was no change in the sense of smell, rhinorrhoea, facial pain, or associated epistaxis. On examination, there was a large, erythematous mass in the superior aspect of the right nasal cavity, filling the space between the nasal septum, middle, and superior meatus. The rest of the ENT examination was normal. Vital signs were all within the normal range. There was no significant past medical history, and she had tried steroid nasal spray without any benefit. She had a complete resolution of symptoms from surgical intervention, and the mass was confirmed to be an angiofibroma through histopathology. This case report discusses the importance of considering nasopharyngeal angiofibroma as a differential diagnosis for patients presenting with unilateral nasal masses, including female patients, regardless of age.

Lifestyle factors, including smoking, have been linked to neoplastic diseases, and reports suggest an association between smoking and overexpression of FGFR (fibroblast growth factor receptor) in certain neoplasms. This study aims to assess the expression of FGFR3 and FGFR4 genes in patients with and without a history of smoking.A total of 118 participants were recruited, including 83 Juvenile Nasopharyngeal Angiofibroma (JNA) patients and 35 healthy participants, the JNA patients were further stratified as smokers and nonsmokers. Total RNA was extracted from the blood & saliva sample by using TRIzol reagent, and quantified using a Nanodrop, and then subjected to gene expression analysis of FGFR3/4 using RT-PCR. Immunohistochemistry analysis was employed using fresh biopsies of JNA to validate the findings. All experiments were performed in triplicates and analysed using the Chi-Square test (P < 0.05). Smokers exhibited significantly lower total RNA concentrations across all sample types (P < 0.001). The study revealed significant upregulation of both FGFR3/4 genes in JNA patients (P < 0.05). Moreover, FGFR3 expression was significantly higher among smokers 66% (95% CI: 53-79%) compared to non-smokers 22% (95% CI: 18-26%). Immunohistochemistry analysis demonstrated moderate to strong staining intensity for FGFR3 among smokers. The study highlights the overexpression of FGFR3/4 genes in JNA patients, with a stronger association observed among smokers. Furthermore, medical reports indicated higher rates of recurrence and bleeding intensity among smokers. These findings emphasize the potential role of FGFR3 as a key molecular factor in JNA, particularly in the context of smoking.

Objective:To investigate the surgical approach for the resection of juvenile nasopharyngeal angiofibroma（JNA） under nasal endoscopy. Methods:The clinical data of 87 patients undergoing endoscopic resection of nasopharyngeal fibroangioma were retrospectively analyzed. We classified JNA according to tumor site, size, invasion scope and anatomic position relationship between tumor and midline of pupil. Three endoscopic surgical approaches were selected according to the classification, and the postoperative symptoms, complications and recurrence were investigated and analyzed. Results:The tumor resection rate of 87 cases by nasal endoscopic surgery was 100%. Thirty-five cases were approached through the middle nasal passage（small tumors located in the nasal sinuses and pterygopalatine fossa）, forty-five cases were approached through the lateral wall of the nasal cavity（tumor invaded the pterygopalatine fossa but did not exceed the midline of the pupil） , and seven cases were approached via the lateral wall of nasal cavity + ipsilateral anterior wall of maxillary sinus（tumor invaded the infratemporal fossa beyond the midline of pupil or invaded the cavernous sinus and the middle cranial fossa epidural）, Postoperative patients with nasal congestion, nasal bleeding, headache, dizziness, vision loss and other symptoms showed varying degrees of improvement. No surgical death or intracranial infection occurred. The postoperative follow-up was 6-78 months, and the recurrence rate was 3.44%. Conclusion:Endoscopic resection of nasopharyngeal fibroangioma is the main treatment method for JNA. Selecting suitable endoscopic approach to resect JNA, To maximize the advantage of nasal endoscopic equipment according to the inherent anatomical space of the human nasal cavity, In order to achieve the purpose of JNA resection, reduce intraoperative and postoperative complications, reduce the recurrence rate and improve the prognosis.
目的：探讨经鼻内镜手术入路切除鼻咽纤维血管瘤（juvenile nasopharyngeal angiofibroma，JNA）的手术经验及效果。 方法：回顾性分析87例行鼻内镜下切除JNA的患者的临床资料。根据肿瘤的部位、大小、侵犯的范围及肿瘤与瞳孔中线的解剖位置关系对JNA进行分类，根据分类选择了3种内镜手术入路方式，并对术后症状、并发症和复发情况进行调查分析。 结果：87例经鼻内镜手术肿瘤切除率100%，经中鼻道入路35例（肿瘤较小，位于鼻腔鼻窦及翼腭窝），经鼻腔外侧壁入路45例（肿瘤侵犯翼腭窝但未超过瞳孔中线），经鼻腔外侧壁+同侧上颌窦前壁入路7例（肿瘤超过瞳孔中线侵及颞下窝或侵犯海绵窦、中颅窝硬脑膜外）。术后患者鼻塞、鼻腔间断流鼻血、头痛头晕、视力下降等症状得到不同程度改善。无手术死亡、颅内感染病例。术后随访6~78个月，复发率3.44%。 结论：经鼻内镜切除JNA手术是治疗JNA的主要手段，术前是否选择行肿瘤营养血管栓塞术可根据肿瘤的位置和分期以及术者的临床操作水平综合考虑；选择合适的鼻内镜入路切除JNA，能够根据人体鼻腔固有解剖空间最大限度的利用鼻内镜设备的优势，以达到切除JNA、减少术中术后并发症、减小复发率、提高预后的目的。.