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.

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.

We describe the first case of juvenile nasopharyngeal angiofibroma receiving blood supply from caroticotympanic artery which resulted in catastrophic bleeding intraoperatively. The patient was an 12-year-old boy with a Radkowski stage 3B JNA. We utilized preoperative embolization of feeders from external carotid artery though the surgeon failed to excise the tumor completely due to uncontrollable bleeding from the tumor. On retrospective analysis of angiography only the culprit feeder found. Tumor was excised in the second surgery conducted a week later. Although selective embolization of ICA branches are described in literature the data are sparse and so is the management of such cases.

The detection of androgen receptors within Juvenile Nasopharyngeal Angiofibroma (JNA) has prompted investigation of the role of Flutamide. The aim of this review is to evaluate Flutamide as a possible neo-adjuvant treatment for JNA. Literature searches were conducted using MEDLINE, EMBASE and Web of Science. The Joanna Briggs Institute (JBI) checklist was used to assess risk of bias. The Oxford Centre of Evidence-Based Medicine (OCEBM) Levels of Evidence was used to stratify the evidence level. Literature searches were conducted using MEDLINE, EMBASE and Web of Science. Flutamide as neo-adjuvant treatment potentially causes a reduction in JNA tumor volume by ≥ 25%. Based on the current limited evidence, Flutamide has a limited role in JNA management and further research is required. Its utilization should only follow discussion with the patient, their families, and within the multidisciplinary team.

Juvenile Nasopharyngeal Angiofibroma (JNA) is an unusual benign, although destructive fibrovascular tumor of the nasopharynx which rarely present itself at other sites. Maxilla is the most frequent extranasopharyngeal site of origin. We report a case of Extranasopharyngeal Angiofibroma (ENA) arising from maxillary sinus in an adult patient which was excised into successfully.

Endoscopic and open microsurgical approaches for pediatric patients are useful for a wide variety of skull base pathologies. A hybrid, cranioendoscopic approach may be beneficial in improving surgical resection for complex lesions. A case of a 13-year-old boy with a large juvenile nasopharyngeal angiofibroma extending through the nasopharynx and pterygopalatine fossa into the maxillary, sphenoid, and cavernous sinuses is demonstrated via an endoscopic, transnasal and frontotemporal, extended middle cranial fossa microsurgical approach. Management of a large pediatric tumor via narrow nasal passages, safe surgical resection around critical neurovascular structures, and complication avoidance is demonstrated. The video can be found here: https://youtu.be/1WqvsOnQCxs.

Objectives  The aim of the study is to evaluate the effect of preoperative vascular embolization (PVE) on juvenile nasopharyngeal angiofibroma (JNA) surgical outcomes using a national pediatric hospitalization database. Methods  The health care cost and utilization project Kids\' Inpatient Database was queried for all cases of operative management of JNA between the years of 1997 and 2016. Cases were stratified based on whether the patient received PVE. A multiple linear regression was used to predict the effect of PVE on hospital length of stay (LOS) and total cost while controlling for patient demographic factors and comorbidities. The odds ratio (OR) of receiving a perioperative blood transfusion was computed using a binary logistic regression for PVE patients. Results  A total of 473 patients who underwent JNA surgical resection in this time period were identified. The use of PVE has increased from 0% in 1997 to 66% of all cases by 2016. PVE was found to decrease LOS by 1 day ( p  = 0.036) and decrease the odds of needing a perioperative blood transfusion (OR = 0.511, p  = 0.041). Patients receiving PVE were charged an additional $35,600 ( p  < 0.001), but recent data in 2016 indicate that hospital costs for PVE are decreasing. Conclusion  PVE of JNA is becoming increasingly prevalent. Embolization results in decreased hospital LOS and lower odds of needing blood transfusions. While embolization increases the cost of management, this trend should be re-evaluated as this procedure is becoming more widespread.

Juvenile nasopharyngeal angiofibroma (JNA) is a benign tumor of the nasal cavity that predominantly affects young boys. Surgical removal remains the gold standard for the management of this disease. Preoperative intra-arterial embolization (PIAE) is useful for reductions in intraoperative blood loss and surgical complications. In our series of 79 patients who underwent preoperative embolization from 1999 to 2020, demographics, procedural aspects, surgical management and follow-up outcome were analyzed. Embolization was performed in a similar fashion for all patients, with a superselective microcatheterization of external carotid artery (ECA) feeders and an injection of polyvinyl alcohol (PVA) particles, followed, in some cases, by the deployment of coils . Procedural success was reached in 100% of cases, with no complications such as bleeding or thromboembolic occlusion, and surgical intraoperative blood loss was significantly decreased. In conclusion, PIAE is a safe and effective technique in JNA treatment, minimizing intraoperative bleeding.

Somatostatin receptors (SSTRs) are highly expressed in neuroendocrine tumors and is exploited for its imaging and treatment. SSTRs expression is also demonstrated in diverse benign and malignant tumor cell types and proliferating peri-tumoral vessels. Similarly, Juvenile Nasopharyngeal Angiofibroma (JNA) expresses different SSTRs and may be utilized for its imaging and treatment using DOTA, 1-Nal3-octreotide (DOTANOC)-PET/CT scan.
Prospective cohort.
Nineteen clinico-radiologically diagnosed primary JNA patients underwent a 68 Ga-DOTANOC PET-CT scan. Using a dedicated PET/CT scanner, a low-dose head and neck spot CT scan was performed after 45 to 60 minutes of intravenous injection of 2 to 3 mCi(74-111 MBq) of DOTANOC. The primary objective was to assess the intensity and pattern of DOTANOC uptake in these patients.
DOTANOC expression was noted in all cases (n = 19) of primary JNA (100%). The mean (SD) DOTANOC SUVmax ratio of tumor and background was 6.9+/-1.4(range, 3.8-9.5). Intra-cranial extension in all 13/19 patients was prominently visualized due to the absence of DOTANOC uptake in the brain. Compared to the background all stages of JNA showed significant DOTANOC uptake (P < .0001). No difference in uptake between advanced-stage tumors and early tumors was noted (P = .47). A statistically non-significant negative trend was noted for decreasing uptake with increasing age (Spearman correlation coefficient, r = -0.19).
This first study of 68 Ga-DOTANOC-PET/CT scan in JNA demonstrates consistent and reliable uptake activity in all patients irrespective of age and stage. This opens up possibilities to physiological diagnostic imaging with a promise of greater specificity and sensitivity and may have applications in ambivalent diagnostic situations such as the detection of recurrence.
3 Laryngoscope, 131:1509-1515, 2021.

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.