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。.

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。.

OBJECTIVE: To determine the effects of blood supply from internal carotid artery (ICA) on the surgical outcomes of primary juvenile nasopharyngeal angiofibroma (JNA) after transarterial embolization (TAE).
METHODS: A retrospective analysis was performed on primary JNA patients who underwent TAE and endoscopic resection in our hospital between December 2020 and June 2022. The angiography images of these patients were reviewed, and then they were divided into ICA + external carotid artery (ECA) feeding group and ECA feeding group according to whether the ICA branches were part of the feeding arteries. Tumors in ICA + ECA feeding group were fed by both ICA and ECA branches, while tumors in ECA feeding group were fed by ECA branches alone. All patients underwent tumor resection immediately after ECA feeding branches embolization. None of the patients underwent ICA feeding branches embolization. Data on demographics, tumor characteristics, blood loss, adverse events, residual and recurrence were collected, and case-control analysis was performed for the two groups. Differences in characteristics between the groups were tested using Fisher\'s exact and Wilcoxon tests.
RESULTS: Eighteen patients were included in this study: nine in ICA + ECA feeding group and nine in ECA feeding group. The median blood loss was 700 mL (IQR 550-1000 mL) in ICA + ECA feeding group versus 300 mL (IQR 200-1000 mL) in ECA feeding group, with no significant statistical difference (P = 0.306). Residual tumor was found in one patient (11.1%) in both groups. Recurrence was not observed in any patient. There were no adverse events from embolization and resection in either group.
CONCLUSIONS: The results of this small series suggest that the presence of blood supply from ICA branches in primary JNA has no significant effect on intraoperative blood loss, adverse event, residual and postoperative recurrence. Therefore, we do not recommend routine preoperative embolization of ICA branches.
METHODS: Level 4, Case-control.

BACKGROUND: Juvenile nasopharyngeal angiofibroma (JNA) is a highly recurrent tumor after curative surgery.
OBJECTIVE: The purpose of this study was to evaluate heat shock protein 90 (HSP90) expression in JNA and its association with tumor recurrence.
METHODS: Immunohistochemistry was performed to assess HSP90 expression using tissue microarrays containing 70 JNA patients and 10 control subjects. The associations of HSP90 expression with clinicopathological features and tumor recurrence were analyzed.
RESULTS: Immunohistochemistry revealed high HSP90 expression in JNA compared with normal middle turbinate samples. High expression of HSP90, which correlated with MVD (P = .001), ER-α (P = .001), VEGF (P < .001) and JNA recurrence (P = .009), was an independent prognostic factor of time to recurrence (P = .017). The combination of HSP90 and ER-α had a better power to predict disease recurrence than other clinicopathological features (P = .008).
CONCLUSIONS: HSP90 may be an independent prognostic marker in JNA patients administered surgical treatment. Combination of HSP90 and ER-α expression may be the best predictor of tumor recurrence among all clinicopathological factors.

Juvenile nasopharyngeal angiofibroma (JNA) has a high recurrence rate after surgery. Cofilin overexpression is associated with increased tumor cell metastasis, and progression of various human cancers. However, studies on cofilin expression in JNA are rare. The purpose of this study was to investigate the expression and localization of cofilin in a tissue microarray (TMA) of JNA specimens. In addition, we also analyzed its correlation with clinicopathological features and recurrence.
Immunohistochemistry was performed to detect cofilin expression in a TMA of samples from 70 JNA patients and 10 control subjects. The association between clinicopathological variables and cofilin immunostaining was analyzed using Pearson\'s chi-square test. Kaplan-Meier survival analysis was used to calculate the disease-free survival rate, and investigate the effect of cofilin expression on time to recurrence (TTR) in JNA patients. The Cox regression model was used for multivariate survival analysis.
Cofilin was detected in irregular smooth muscle cells, pericytes, less differentiated stromal cells, and plump cells, but not in inactive fibroblasts and mature vascular endothelial cells of JNA specimens. The presence of cofilin in JNA was correlated with tumor stage (p = 0.012) and volume of intraoperative hemorrhage (p < 0.001). JNA patients with high cofilin expression had a higher recurrence rate than those with low cofilin expression (p = 0.012). Cofilin expression and patient\'s age were significant predictors of TTR, and cofilin was a better predictor for disease recurrence (area under the receiver operating curve [AUROC; 0.711; p = 0.005) than other clinicopathological features.
Cofilin is an independent prognostic marker for JNA patients who have undergone surgical treatment and may represent a novel therapeutic target for extensive JNA.

OBJECTIVE: Juvenile nasopharyngeal angiofibroma (JNA) is non-metastasizing but potentially locally destructive tumor of the nasopharynx. It can destroy the skull base and invade into the cerebrum. Surgical management is the primary standard but residual disease is always a risk factor. We aimed to determine the risk factors for residual disease and usual sites for these residual tumors.
METHODS: The medical records of 131 patients (mean age 17.6 ± 6.8, range 9-71 years) with histologically proven JNA were retrospectively analyzed. The surgeries were all nasal endoscopic approaches, with or without assistant incision.
RESULTS: The prevalence of residual disease was 16.8%. Risk factors associated with JNA recurrence included tumor stage, intraoperative bleeding, and the year in which the operation was performed. The pterygoid canal, pterygoid process, and pterygopalatine foramen were the most frequent locations for residual tumor.
CONCLUSIONS: Surgical management should take particular care for the pterygoid canal, petrygoid process, and pterygopalatine foramen. Contrast-enhanced CT and MRI are effective tools to evaluate complete JNA excision in the first two days after primary surgery. Careful exploration of these areas may be the key to avoid residual JNA.

OBJECTIVE: To examine the expression of hypoxia-inducible factor-1α (HIF-1α) and its related molecules (cellular repressor of E1A-stimulated genes [CREG], osteopontin [OPN], proto-oncogene tyrosine-protein kinase Src [c-Src], and vascular endothelial growth factor [VEGF]) in juvenile nasopharyngeal angiofibroma (JNA) and explore the correlation between clinical prognosis and HIF-1α expression.
METHODS: The study performed a retrospective review of the clinical records of patients with JNA treated between 2003 and 2007. Specimens were analyzed by immunohistochemistry for HIF-1α, CREG, OPN, c-Src, and VEGF expression, and microvessel density (MVD) was assessed by tissue microarray. The correlation between expression levels and clinicopathological features including age, tumor stage, intraoperative blood loss, and recurrence was analyzed.
RESULTS: HIF-1α, CREG, OPN, c-Src, and VEGF were upregulated in endothelial cells (ECs) of patients with JNA, and strong correlations in the expression of these molecules were observed. HIF-1α expression was higher in young patients ( P = .032) and in recurrent cases ( P = .01). Survival analysis showed that low HIF-1α levels in ECs predicted longer time to recurrence (log rank test P = .006). Receiver operating characteristic curve analysis showed that HIF-1α was a prognostic factor for recurrence (area under the curve = 0.690, P = .019). No correlation was found between the expression of molecules and Radkowski stage or intraoperative blood loss.
CONCLUSIONS: In cases of JNA treated surgically, HIF-1α expression in ECs is a useful prognostic factor for tumor recurrence.

To determine the learning curve with cumulative sum analysis for endoscopic resection of juvenile nasopharyngeal angiofibroma (JNA) and investigate whether the surgeon\'s expertise is a risk factor for recurrence.
We reviewed the medical records of patients with JNA who underwent endoscopic or endoscopic-assisted surgery between 2006 and 2015. We used cumulative sum (Cusum) analysis to plot the learning curve for operation time versus chronological sequence, and verified the Cusum curve by risk-adjusted Cusum (RA-Cusum) analysis. We identified three phases of expertise. The recurrence rate was analyzed using the Kaplan-Meier method and log-rank tests. A multivariable Cox regression analysis was performed to identify the independent risk factors for recurrence.
We included 154 JNA patients with a median age of 16 years. The surgeon overcame the learning curve after case 80 with increasing surgical efficiency and competence. The learning curve plotted by Cusum analysis divided the cases into three phases: phase 1, accumulation of initial experience (cases 1-41); phase 2, further accumulation of experience (cases 42-117); and phase 3, mastering the procedure (cases 118-154). Pearson\'s χ2 tests showed that tumor stage (P = 0.021), blood loss (P = 0.001), operation time (P < 0.001), and phase (P < 0.001) were associated with recurrence. The log-rank test showed that time to recurrence was significantly shorter in phase 1 than in phases 2 and 3. Blood loss and phase were independently prognostic factors for time to recurrence, with P values of 0.023 and 0.009, respectively. The RA-Cusum analysis identified two inflection points of the curve at case 44 and 83, and verified the results of Cusum analysis.
Surgical experience and competence with endoscopic resection affect the recurrence rate in JNA patients.
4.

OBJECTIVE: The purpose of this study was to report on a series of 162 patients presenting with juvenile nasopharyngeal angiofibroma in a single academic hospital during the past 17 years, in an effort to compare outcomes between open and transnasal endoscopic approach, and to define an ideal treatment strategy.
METHODS: Patients who received either open or endoscopic surgery with a minimum follow-up of 6 months were selected. Local control and complications were compared between groups.
METHODS: Retrospectively, clinical data, surgical reports, pre- and postoperative images, and follow-up information were reviewed and analyzed.
RESULTS: All patients were male subjects from 8 to 41 years old. Ninety-six patients were treated by transpalatal or transmaxillary approach, and the remaining 66 patients were treated using transnasal endoscopic approach with/without labiogingival incision. When compared to the open surgery group, the endoscopic surgery group showed a lower median intraoperative blood loss (800 vs. 1100 mL, P = .017) and a lower number of postoperative complications (one vs. 10). In addition, recurrence statistically correlated with Radkowski\'s classification and patient age.
CONCLUSIONS: Transnasal endoscopic approach can be successfully used for Radkowski\'s stages I-IIb tumors and selective IIc-IIIb lesions, allowing for less blood loss, fewer postoperative complications, and a lower percentage of recurrence in comparison to open surgery. The management of recurrent tumor is complex, should be individually tailored, and should take into account tumor location, patient age, complications of treatment, and the possibility of spontaneous involution, to better define treatment strategy.