Advanced Juvenile Nasopharyngeal Angiofibroma Research Articles

Helium Ion Therapy for Advanced Juvenile Nasopharyngeal Angiofibroma.

Helium ion therapy (HRT) is a promising modality for the treatment of pediatric tumors and those located close to critical structures due to the favorable biophysical properties of helium ions. This in silico study aimed to explore the potential benefits of HRT in advanced juvenile nasopharyngeal angiofibroma (JNA) compared to proton therapy (PRT). We assessed 11 consecutive patients previously treated with PRT for JNA in a definitive or postoperative setting with a relative biological effectiveness (RBE) weighted dose of 45 Gy (RBE) in 25 fractions at the Heidelberg Ion-Beam Therapy Center. HRT plans were designed retrospectively for dosimetric comparisons and risk assessments of radiation-induced complications. HRT led to enhanced target coverage in all patients, along with sparing of critical organs at risk, including a reduction in the brain integral dose by approximately 27%. In terms of estimated risks of radiation-induced complications, HRT led to a reduction in ocular toxicity, cataract development, xerostomia, tinnitus, alopecia and delayed recall. Similarly, HRT led to reduced estimated risks of radiation-induced secondary neoplasms, with a mean excess absolute risk reduction of approximately 30% for secondary CNS malignancies. HRT is a promising modality for advanced JNA, with the potential for enhanced sparing of healthy tissue and thus reduced radiation-induced acute and long-term complications.

Total Endoscopic or Endoscope-Assisted Excision of Non-embolized Advanced Juvenile Nasopharyngeal Angiofibroma: A Clinical Case Series.

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.

Preoperative Transarterial Embolization of Advanced Juvenile Nasopharyngeal Angiofibroma Using n-Butyl Cyanoacrylate: Case-Control Comparison with Microspheres

PurposeTo evaluate the efficacy and safety of transarterial embolization (TAE) with n-butyl cyanoacrylate (nBCA) for juvenile nasopharyngeal angiofibroma (JNA). Materials and MethodsA retrospective review was performed on patients with JNA who underwent TAE and endoscopic resection between 2020 and 2022. Patients embolized with nBCA were identified, and those embolized with microspheres were set as the control group. Data on demographics, symptoms, tumor characteristics, blood loss, adverse events, residual disease, and recurrence were collected, and case-control analysis was performed for the 2 groups. Differences in characteristics between the groups were tested using the Fisher exact and Wilcoxon tests. A generalized linear model (GLM) was used to analyze the univariate and multivariate influences on blood loss. ResultsTwenty patients were included in this study: 13 in the microsphere group and 7 in the nBCA group. The median blood loss was 400 mL (interquartile range [IQR], 200–520 mL) in the nBCA group and 1,000 mL (IQR, 500–1,000 mL) in the microsphere group (P = .028). The GLM confirmed lower blood loss in the nBCA group (relative risk, 0.58 [0.41–0.83]; P = .01). A residual tumor was found in 1 patient in each group (7.7% vs 14.3%; P = 1.000). Recurrence was not observed in any patient. None of the patients experienced adverse events during embolization. ConclusionsTAE of advanced JNA with nBCA glue is safe and effective and can significantly reduce intraoperative blood loss compared with microspheres.

Endoscopic endonasal transmaxillary ligation of a feeding artery and coblation plasma technology enables en bloc resection of advanced juvenile nasopharyngeal angiofibroma without preoperative embolization

Juvenile nasopharyngeal angiofibroma (JNA) is a hypervascular tumor and uncontrolled hemorrhage makes its removal very difficult. Although preoperative intravascular embolization of a feeding artery is recommended, serious complications such as iatrogenic thrombosis in the brain and insufficient decrease in blood flow to the tumor are concerns. Recently, coblation plasma technology has been reported to be useful for tumor removal with minimum hemorrhage under a clear surgical field. Here we report successful removal of advanced JNA without preoperative embolization, using intraoperative ligation of the maxillary artery and coblation plasma technology. The left nasal cavity of a 23-years-old man was closed by a JNA tumor at Radkowski stage IIC, which was 65mm in size and extended from the nasal cavity to the infratemporal fossa. MRA imaging showed the maxillary artery running along the posterior wall of the maxillary sinus. Therefore, the maxillary artery was first clipped using an endoscopic modified medial maxillectomy (EMMM) approach and endoscopic endonasal en bloc resection of the tumor was then completed using coblation technology with no need for blood transfusion. This case illustrates that preoperative embolization is dispensable in JNA surgery even at Stage IIC if the maxillary artery can be ligated during surgery and a coblation device can be utilized.

Case Report of Advanced Juvenile Nasopharyngeal Angiofibroma with Cavernous Sinus Involvement: Advantages of Advanced Radiotherapy Thechnique Volumetric Modulated Arc Therapy

Objectives: In very advance unresectable stages Juvenile nasopharyngeal angiofibroma (JNA)radiotherapy is treatment of choice. Usually JNA is in close proximity to different organ at risk, which necessitates accurate and precise radiation delivery. Series reported the application of IMRT (Intensity Modulated Radiotherapy) with good sparing of organ at risk, but the literature search found no report on VMAT (Volumetric Modulated Arc Therapy) treatment in case of JNA. Clinical case: The present publication report for first time a patient irradiated with VMAT and IGRT (Image guided radiotherapy) for JNA. The stage of the tumor based on CT and MRI was IIIB according to Radkovsi with cavernous sinus invasion. Moderate total radiation dose 36 Gy in 1.8 Gy per fraction was delivered. No early and late toxicity was recorded. The patient is followed up every six months and persistent residual tumor on MRI was seen, but no bleeding onphysical exam. At one year follow up this is accepted as good local control. Conclusion: VMAT and IGRT could be safely applied with reduction of the radiation dose at organ of risk. VMAT is promising in the treatment of juvenile angiofibroma with advantages over conventional radiotherapy, but as this is case report of only one patient, further experience with VMAT is needed in JNA

Management and Outcome in Patients with Advanced Juvenile Nasopharyngeal Angiofibroma.

Objective To report the management outcome in a series of patients with advanced juvenile nasopharyngeal angiofibroma (JNA). Design Retrospective study. Setting Tertiary care teaching hospital. Participants Forty-five patients classified as Radkowski stage IIIA or IIIB who presented to us over the past 10 years. Main Outcome Measures Surgical approaches used and disease free outcomes in patients with advanced JNA. Results Surgical access for the extracranial component included open (41.9%) and expanded endonasal approaches (58.1%). Craniotomy (16.3%), endoscopy-assisted open approach (7%), or expanded endonasal approach (20.9%) was performed to excise the skull base or intracranial component. Follow up ranged from 4 to 96 months (mean, 20.3 months). Of 35 patients who underwent imaging at the first postoperative follow up, 25 (71.4%) had negative scans. Three symptomatic patients with residual disease underwent endoscopic excision and had negative scans thereafter. Of two others who had radiation therapy, one was disease free and the other lost to follow up. Five others had stable, residual disease. Three patients (8.6%) with recurrent disease underwent surgical excision, of whom two had minimal, stable residual disease. At the last follow-up, 27 (77.1%) patients had negative scans, and 7 (20%) had stable residual disease with one (2.9%) patient lost to follow-up. Conclusions Advanced JNA may be successfully treated in most cases with expanded endonasal/endoscopy assisted ± craniotomy approach after appropriate preoperative evaluation. At follow-up, only symptomatic patients or those with enlarging residue require treatment; periodic imaging surveillance is adequate for those with stable disease.

Conformal radiotherapy for locally advanced juvenile nasopharyngeal angio-fibroma.

To assess the efficacy of radiation in the treatment of juvenile nasopharyngeal angiofibroma (JNA). Data were retrieved for JNA treated with radiotherapy from 1987-2012. The demographics, treatment and outcome data were recorded in predesigned proforma. Data of 32 patients were retrieved. Median age was 17 years (range: 12-33 years). All patients received radiation because of refractory, residual or unresectable locally advanced disease. All patients were planned with a three-dimensional conformal technique (3DCRT). The median radiation dose was 30 Gray (range: 30-45 Gray). Median follow-up was 129 months (range: 1-276 months). At the last follow-up, 13 patients were found to have a radiological complete response. Two patients progressed 38 and 43 months after completion of treatment and opted for alternative treatment. One patient developed squamous cell carcinoma of the nasal ale 15 years after radiation. Conformal radiotherapy shows promise as an alternative treatment approach for locally advanced JNA and confers long-term disease control with minimal toxicity.

A systematic review of surgical outcomes for advanced juvenile nasopharyngeal angiofibroma with intracranial involvement

Although transnasal endoscopic resection has become an established treatment for most juvenile nasopharyngeal angiofibroma (JNA), surgical management of JNA with intracranial extension remains challenging. This study systematically reviews the JNA literature to determine surgical outcomes. Systematic review. A systematic search of the PubMed was undertaken using a combination of MeSH terms: angiofibroma, nasopharynx. The search was limited to articles published in the English language between January 1990 and April 2012. Each article was reviewed to identify sufficient individual data on patients treated for JNA, defined as reporting on demographics, JNA stage, the specific surgical approach, complications, and length of follow-up for each patient. A total of 72 patients from 15 studies were included in this review. The mean age was 15.7 years. The mean follow-up period was 47 months. Most patients had a craniofacial procedure. The overall mean estimated intraoperative blood loss was 1,709 mL. Preoperative embolization resulted in significantly less blood loss. The most common complications were sinonasal and neurological. Facial paresthesia was reported in 16%, followed by ophthalmoplegia (12%) and intranasal crusting (12%). Recurrence was reported in 13 patients (18%), which were detected between 7 and 26 months during the follow-up period. Overall, 86% of the cohort was free of disease. Surgical management of JNA with intracranial extension is complex and requires an expert multidisciplinary team. Although craniofacial approaches appear to be the current standard of treatment, there is increased experienced-based evidence that endoscopic resection of large tumors or endoscopic-assisted resection is feasible in expert hands. 3a.

Endoscopic Coblation for the Treatment of Advanced Juvenile Nasopharyngeal Angiofibroma

We present 2 cases of advanced juvenile nasopharyngeal angiofibroma (JNA) to illustrate the advantages of endoscopic Coblation-assisted resection of intranasal extensions of these masses. Both patients-an 11-year-old boy and a 14-year-old boy-presented with a large, extensive mass (Radkowski stage IIIb and Fisch stage IVb in both cases). After embolization was performed on each patient, his JNA was partially ablated via an endoscopic approach with the Coblator II Surgery System with an EVac Xtra Plasma Wand in conjunction with an image-guided navigation system. Both patients experienced resolution of their nasal obstruction with removal of the intranasal extension of the tumor. Coblation allowed for a controlled debulking of the tumors with less blood loss and without the need for multiple instruments. To the best of our knowledge, our report is one of the first to describe image-guided endoscopic Coblation of advanced JNA tumors. Future studies in adequately sized populations are needed to determine the safety and effectiveness of Coblation-assisted endoscopic removal of both advanced and lower-stage JNAs.

Advanced Juvenile Nasopharyngeal Angiofibroma

Objective: The aim of this study was to describe a case of advanced juvenile nasopharyngeal angiofibroma from our practice and discuss the controversies of surgical treatment. Method: We present a 13-year-old boy who underwent external resection of advanced juvenile nasopharyngeal angiofibroma at the Gazi University medical faculty otorhinolaryngology department, in 2012. Results: No complication was seen after surgery. Postoperative radiotherapy was given for small residual tumor. Conclusion: Surgical treatment is the basic tactics in management of advanced juvenile nasopharyngeal angiofibroma. Stage of the tumor is the main factor contributing to the choice of the appropriate surgical approach.

Preoperative Direct Puncture Embolization of Advanced Juvenile Nasopharyngeal Angiofibroma in Combination with Transarterial Embolization: An Analysis of 22 Consecutive Patients

This study was designed to evaluate the clinical application of preoperative auxiliary embolization for juvenile nasopharyngeal angiofibroma (JNA) by direct puncture embolization (DPE) of the tumor in combination with transarterial embolization (TAE). The study included 22 patients. An 18-gauge needle was used to puncture directly into the tumor, and 20-25% N-butyl cyanoacrylate was injected under the guidance of fluoroscopy after confirming the placement of the needle into the JNA and no leaking into the surrounding tissue. Tumors were obstructed later via TAE. The supplying arteries of JNA were from branches of the internal carotid and external carotid arteries. Control angiography showed the obliteration of contrast stain in the entire tumor mass and the distal supplying arteries disappeared after DPE in combination with TAE. Surgical resection was performed within 4days after embolization and none of the patients required blood transfusion. The use of DPE in combination with TAE was a safe, feasible, and efficacious method. It can devascularize effectively the JNAs and reduce intraoperative bleeding when JNAs are extirpated.

Multisession Cyberknife radiosurgery for the treatment of advanced-stage intracranial extended Juvenile nasopharyngeal angiofibrom

Background: A case of a large advanced stage juvenile nasopharyngeal angiofibroma (JNA) treated with combined temporally and volume fractionated CyberKnife radiosurgery (CKRS) is presented. The optimal modality for treating advanced JNA continues to be controversial because of the complications associated with surgical resection and the potential delayed morbidity of radiotherapy (RT) in a pediatric age group. Clinical Presentation: CKRS treatment was delivered with a volume fractionation method. First, for the lower two-thirds of the tumor occupying the nasal cavity, pterygopalatine fossa, maxillary sinus, and ethmoid sinus, CKRS was administered in five fractionated treatments of 7.0 Gy to the 71.7% prescription isodose. This lower part of the tumor volume was 56.4cm 3 . Next, for the upper one-third of the tumor occupying the sphenoid sinus, infratemporal fossa and intracranial left cavernous sinus, CKRS was administered in five fractionated treatments of 6.2 Gy to the 72.1% prescription isodose. This upper part of the tumor volume was 25.2 cm 3 . Post-treatment, a gradual reduction in the whole tumor volume was observed continuously over the course of follow-up, and the patency of the internal carotid artery was restored. Radiation-related complications were limited to nasal mucosal crust formation. Conclusion: A case of a large advanced stage JNA treated with CKRS using combined temporal and volume fractionated CKRS is presented. Follow-up revealed reasonable continuous reduction in tumor volume and the normal patency of the internal carotid artery without any radiation-related complication except for nasal mucosal crust formation. Longer follow-up is needed to evaluate late morbidity and long-term disease control.

Advanced craniofacial juvenile nasopharyngeal angiofibroma. Description of surgical series, case report, and review of literature

Juvenile nasopharyngeal angiofibroma (JNA) is a rare benign tumor occurring almost exclusively in adolescent and young adult males. The tumor is characterized by slow progression, aggressive growth, high vascularization, and increased rate of persistence and recurrence. The aim of this study was to describe a case of giant JNA from our practice and discuss the controversies of surgical treatment of advanced JNA. A series of 29 consecutive male patients with JNA Fisch grade III and IV was surgically treated in Burdenko Neurosurgical Institute from 2000 until 2008. In the vast majority of cases, endovascular embolization and surgical removal via orbitozygomatic approach were applied. Gross total resection was achieved in 24 cases (83%). Complications were encountered in eight cases. No mortality was observed. In three patients, the diseases recurred. An illustrative case is described. Surgical treatment is the basic tactics in management of extensive JNA including endovascular embolization and resection of the tumor. We recommend using orbitozygomatic approach or its modifications in JNA. Radiation therapy may be recommended for patients with small residual tumor.

Adjuvant therapy with flutamide for presurgical volume reduction in juvenile nasopharyngeal angiofibroma

Although 2 studies totaling 11 cases have indicated some benefit of anti-androgen treatment with flutamide on juvenile nasopharyngeal angiofibroma (JNA), it is not part of contemporary practice. Our approach was through a prospective, single-arm, before-and-after study, in which 20 patients with advanced JNA (Radkowski stage IIB-IIIB) were administered flutamide (per oral: 10 mg kg(-1) day(-1) × 6 weeks) prior to surgical excision. Pretherapy and posttherapy tumor volume measurements were established by MRI. Periodic assessments were recorded of liver, kidney functions, testosterone levels, and secondary sexual characteristics. Prepubertal and postpubertal cases responded differently (p < .05). Prepubertal cases had inconsistent and minimal responses; 13/15 postpubertal cases demonstrated measurable volume reduction (mean, 16.5%; maximum, 40%). Two cases with optic nerve compression had visual improvement. Volume reduction correlated with serum testosterone level (r = .53; p < .05). No significant toxicity was noted, with the exception of transient breast tenderness. Prepubertal and postpubertal patients differ in their response to flutamide. In postpubertal patients, 6 weeks preoperative use is safe and leads to partial tumor regression. Tumor regression from adjacent vital structures may facilitate surgical excision and limit morbidity.

Conformal Radiotherapy in the Treatment of Advanced Juvenile Nasopharyngeal Angiofibroma With Intracranial Extension: An Institutional Experience

To describe the results of conformal radiotherapy in advanced juvenile nasopharyngeal angiofibroma in a tertiary care institution. Retrospective chart review was conducted for 8 patients treated with conformal radiotherapy between 2006 and 2009. The median follow-up was 17 months. All patients had Stage IIIB disease with intracranial extension. Radiotherapy was considered as treatment because patients were deemed inoperable owing to extensive intracranial/intraorbital extension or proximity to optic nerve. All but 1 patient were treated with intensity-modulated radiotherapy using seven coplanar fields. Median (range) dose prescribed was 39.6 (30-46) Gy. Actuarial analysis of local control and descriptive analysis of toxicity profile was conducted. Despite the large and complex target volume (median planning target volume, 292 cm(3)), intensity-modulated radiotherapy achieved conformal dose distributions (median van't Reit index, 0.66). Significant sparing of the surrounding organs at risk was obtained. No significant Grade 3/4 toxicities were experienced during or after treatment. Actual local control at 2 years was 87.5%. One patient died 1 month after radiotherapy secondary to massive epistaxis. The remaining 7 patients had progressive resolution of disease and were symptom-free at last follow-up. Persistent rhinitis was the only significant toxicity, seen in 1 patient. Conformal radiotherapy results in good local control with minimal acute and late side effects in juvenile nasopharyngeal angiofibromas, even in the presence of advanced disease.

Treatment outcomes of juvenile nasopharyngeal angiofibroma according to surgical approach

Objective This is a retrospective study to assess treatment outcomes according to stage and surgical approach in advanced juvenile nasopharyngeal angiofibroma (JNA). Methods We retrospectively evaluated 20 JNA patients diagnosed and treated at our hospital. We only enrolled advanced disease with Radkowski stages greater than I and with minimum follow-up of 1 year (range 1–8.5). Results Recurrence or remnants were observed in 7 patients out of 20 patients (35.0%) who underwent primary surgical resection of advanced JNA and the mean interval to recurrence was 15.6 months (range 6–38). A recurrence rate according to a different stage was as follows: 33.3% in stage IIa, 33.3% in stage IIb, 50.0% in stage IIc and no recurrence in stage III. An endoscopic approach was chosen in 4 patients among these patients, four were classified as stage IIb tumors, one as a stage IIc tumor, with a recurrence rate of 25.0%, but no recurrence found in stage IIa disease. A midfacial degloving approach was used in 7 patients, with a recurrence rate of 42.9% and maxillary swing approach was taken in 3 patients with complete control. Postoperative complications required interventions occurred in 14.8%, more in the invasive maxillary swing or infratemporal fossa approaches. Conclusions Although selecting minimal invasive or invasive approaches is equivocal, we recommend using the endoscopic approach or a midfacial degloving approach for the treatment of JNA extended to the pterygopalatine fossa. For stage III, aggressive surgery is preferable to guaranty a complete resection even if postoperative complications are more frequent. For a stage IIc, we could choose between a minimally invasive approach or a more aggressive one balancing between the possibility of salvage surgery in the future and the occurrence of postoperative healing problems.

Juvenile nasopharyngeal angiofibroma involving the cavernous sinus: Does surgery have a role?

Juvenile nasopharyngeal angiofibroma involving the cavernous sinus poses a difficult dilemma. In the UKJ practice, radiotherapy is often recommended but has significant implications due to tumour volume and patient age. We present two cases of JNA involving the cavernous sinus treated by surgical excision. The surgical approaches and literature are discussed. In these cases, it was possible to separate the disease from the cavernous sinus without neurological sequelae. In advanced juvenile nasopharyngeal angiofibroma involving the cavernous sinus, surgical treatment remains an important option.

Definitive Radiotherapy for Juvenile Nasopharyngeal Angiofibroma

To update our experience with definitive radiotherapy (RT) for juvenile nasopharyngeal angiofibroma (JNA). Twenty-two patients received definitive RT for advanced JNA between June 1975 and March 2003 and were followed from 2.0 to 30.2 years (median, 12.7 years). Total dose ranged from 30 to 36 Gy. Twenty of 22 patients (91%) remained locally controlled after RT; both patients who developed a local recurrence were salvaged so that the ultimate local control rate was 100%. No patient experienced a severe complication. Patients with advanced and/or recurrent JNA thought not to be amenable to complete resection with acceptable morbidity have a high likelihood of cure after RT with a low risk of complications.

Loss of Reflex Tearing: An Expected Consequence of Juvenile Nasopharyngeal Angiofibroma

To report the loss of reflex tearing after surgical treatment of juvenile nasopharyngeal angiofibroma (JNA). A retrospective case series of 8 patients with surgical extirpation of JNA from 1995 to 2003 in a major teaching hospital setting was studied for symptomatic xerophthalmia. One patient was lost to follow-up. Four of the remaining 7 patients reported a dry ipsilateral eye after surgical treatment of JNA. The location of the pterygopalatine ganglion and its associated fibers in the pterygopalatine fossa is directly adjacent to the location of origin of JNA. Lacrimal innervation passes through the pterygopalatine ganglion. Given the extensive nature of advanced JNA and mandate for complete surgical excision, lacrimal dysfunction should be considered an expected consequence of surgery. The loss of reflex tearing has not been reported as a consequence of JNA or its surgical treatment. C.

Intermittent esotropia as the initial presentation of juvenile nasopharyngeal angiofibroma

Juvenile nasopharyngeal angiofibroma is a vascular tumor primarily affecting the nasopharynx of adolescent boys. However, because of its capacity to expand locally, ocular manifestations are common and may result in exophthalmos and visual loss. Strabismus is reported in 2% of cases, but no reports have implicated eye-motility dysfunction as the initial presenting sign. 5 The case presented here illustrates the early manifestation of a left esotropia in a 9-year-old boy who developed severe vision loss from an advanced juvenile nasopharyngeal angiofibroma. The importance of the early recognition and diagnosis of this tumor is emphasized, thus demonstrating the potential role of the ophthalmologist in achieving good outcomes. CASE REPORT A 9-year-old boy was being treated by his pediatrician for a prolonged (3- to 4-month) upper respiratory illness characterized by nasal obstruction, rhinorrhea, frontal headache, and diplopia. After 2 months of treatment with allergy medications, an intermittent left esotropia was noted. He was referred to an ophthalmologist, who also noted a small left esotropia as well as normal visual acuity of 20/20 in the right eye (OD) but decreased visual acuity of 20/40 in the left eye (OS). The change in vision was attributed to strabismic amblyopia and spectacle correction was prescribed. Two weeks later, the patient suffered a soccer ball injury to the face, which resulted in heavy and prolonged epistaxis. His left eye showed a large constant esotropia. Computed axial tomography and magnetic resonance imaging (MRI) studies of the skull base and nasopharynx were obtained. The films showed a large vascular-appearing tumor with extensive expansion into the orbital apex and the cavernous sinus (Figure 1). Otolaryngologic examination, including rigid nasal endoscopy, revealed the presence of an obstructing vascular and rubbery-appearing mass me