Multidisciplinary management of juvenile nasopharyngeal angiofibroma

Background Juvenile nasopharyngeal angiofibroma (JNA) is a rare benign, but the locally aggressive, vascular tumor that occurs almost exclusively in young men. Although this tumor accounts for only about 0.5% of head and neck tumors, it is by far the most common benign nasopharyngeal neoplasm. It is presented typically with epistaxis, obstructive symptoms, and chronic otomastoiditis. The examination of this tumor reveals pale reddish-blue mass. The tumor is seen on imaging as vividly augmenting soft-tissue mass centered on the sphenopalatine foramen. The treatment of choice is usually surgical resection; either open or endoscopic. Pre-operative embolization is usually done to help with hemostasis. We aim to address the efficiency and the safety of endovascular modality in JNA. Methods In the period between January 2012 to December 2017, 20 male patients with age ranging from 6 to 20 years were referred to the Endovascular Unit, at Departments of Neurosurgery in Sohag, Alexandria, and Tanta University Hospitals with JNF. Preoperative trans-arterial embolization was done, followed by endoscopic surgical resection by our ENT surgeons. All patients were subjected to clinical and radiological Imaging evaluation pre embolization, post embolization, and post endoscopic resection. All patients had clinical and radiological follow-ups for at least 1 year. Results 20 male patients with JNA underwent preoperative super-selective trans-arterial embolization. The average age at presentation was 13 years. All patients underwent successful embolization with total obliteration of vascular blush of the tumor in 15 patients, and near-total obliteration of blush in 5 patients. The dramatic effect of preoperative embolization was observed on limiting intraoperative blood loss (average 200 ml) and decreasing the time of surgery (average 2.5 h). There was no permanent morbidity or mortality related to either embolization or endoscopic resection in all patients. Conclusions Trans-arterial embolization is strongly recommended in patients with JNA, it offers a good assist to the surgeon, reduces blood loss, decreases the amount of transfusion, improves the degree of resection, reduces operative time, and decreases the incidence of recurrence.

JUVENILE NASOPHARYNGEALANGIOFIBROMA: OUR EXPERIENCE AND LITERATURE REVIEW

ABSTRACT Introduction: Juvenile nasopharyngeal angiofibroma is a benign vasculartumor.It is commonly found in teen age males. Its site of origin is sphenopalatine foramen. Exact pathogenesis of angiofibroma is not known. It has predictable natural history and growth pattern. This tumor most often involves nasopharynx, nasal cavity, paranasal sinuses, pterygopalatine fossa and infratemporal fossa. It can also involve orbit and can spread intracranially. Its very important to diagnose this tumor very early on the basis of clinical examination and imaging. As early tumor confined to nose and sinuses can be removed exclusively with endoscope. It is very helpful to do angiography before surgery to ascertain itsblood supply and then embolization can be done to reduce intraoperative bleeding. Objective: To describe our experience of Juvenile Nasopharyngeal Angiofibroma cases in ENT Unit-I of Lahore General Hospital. Study Design: Descriptive Study with retrospective analysis after approval from Institutional Review Board (IRB) of LGH/PGMI/AMC Lahore. Methods: We studied 20 patients who underwent surgery in our department from October 2019 to October 2020. We analyzed following factors: age, gender, symptoms, staging, mode of surgery and need for intraoperative blood transfusion, hospital stay, complications and recurrences. Results: Range of patient’s age was 12 to 25 years. Eight patients underwent surgery with endoscope. Mean blood loss was about 400 ml and mean operating time was 140 minutes. All the cases were embolized preoperatively. Conclusion: Endoscopic surgery is a safe and effective method in early stage JNA patients. While patients with advance stage tumors should be managed with combined endoscopic and conventional open approaches. KEYWORDS: juvenile nasopharyngeal angiofibroma, JNA, endoscopic surgery

Abstract 1122‐000193: Intracranial Bleeding in a Juvenile Nasopharyngeal Angiofibroma Stage IVb

Introduction : The trigeminocardiac reflex has been reported in craniofacial, neurosurgery, ophthalmological surgeries, and recently at endovascular procedures. Therefore, it has been called by other names also as trigeminal depressor reflex, reflex vagal trigeminal, or oculocardiac reflex. It is provoked by the stimulation of branches of the trigeminal nerve and presents cardiovascular alterations such as hypotension, bradycardia, cardiac arrhythmias, which can lead to asystole. This reflex originates at the brainstem and occurs as a rare autonomic dysfunction triggered by the stimulation of baroreceptors. Some factors predispose the appearance of this type of reflex, such as hypercapnia, hypoxemia, superficial anesthetic depth, and acidosis, among others. During these procedures is recommended continuous monitoring of the ECG and PAM. It is always essential to know the patient and modify the risk factors, or even stop the stimulus notifying the surgeon, if there is no adequate response, anticholinergic therapy, such as atropine, and the use of vasopressors should be applied. Methods : We report a clinical case of an 18‐year‐old male with a history of 3 years of recurrent epistaxis diagnosed with a Juvenilenasopharyngeal angiofibroma stage IVB, who underwent diagnostic cerebral angiography for surgical planning. Results : Angiography was performed under conscious sedation. When we placed the JB2 diagnostic catheter in the external carotid artery, the patient presented bradycardia of 40bpm. The catheter was removed, and the heart rate improved; we made a second attempt again with bradycardia, for which atropine was administered, and continued with the procedure without incident. We evaluated the vascular supply to the tumor and ruled out the involvement of the ipsilateral internal carotid artery. An occlusion test was also performed, which was positive. No aneurysms were found during angiography. At the end of the angiography, the patient presented anisocoria and left hemiparesis, so due to the suspicion of a thromboembolic event, a new femoral approach was performed to assess the intracranial circulation we found adequate patency. A non‐contrast head CT was performed, a subarachnoid hemorrhage in the prepontine and the interpeduncular cistern was observed. Medications used for sedation were discontinued to assess his neurological status at that time with GCS of 12. 48 hrs later, the patient was neurologically intact and without sequelae. In the literature review, we did not find reports of intracranial hemorrhage as complications in nasopharyngeal angiofibroma with intracranial extension or secondary to the presentation of the trigeminocardiac reflex. However, we suspected that it could result from a transient elevation of arterial hypertension due to the administration of anticholinergic therapy. Conclusions : Neuroanesthesiologists and endovascular surgeons must be aware of its manifestations and management to avoid complications due to the presentation of this reflex.

Master Regulators of Epithelial-Mesenchymal Transition and WNT Signaling Pathways in Juvenile Nasopharyngeal Angiofibromas

Juvenile nasopharyngeal angiofibroma (JNA) is a rare fibrovascular benign tumor showing an invasive growth pattern and affecting mainly male adolescents. We investigated the role of epithelial–mesenchymal transition (EMT) and WNT signaling pathways in JNA. Gene expression profiles using nine JNA paired with four inferior nasal turbinate samples were interrogated using a customized 2.3K microarray platform containing genes mainly involved in EMT and WNT/PI3K pathways. The expression of selected genes (BCL2, CAV1, CD74, COL4A2, FZD7, ING1, LAMB1, and RAC2) and proteins (BCL2, CAV1, CD74, FZD7, RAF1, WNT5A, and WNT5B) was investigated by RT-qPCR (28 cases) and immunohistochemistry (40 cases), respectively. Among 104 differentially expressed genes, we found a significantly increased expression of COL4A2 and LAMB1 and a decreased expression of BCL2 and RAC2 by RT-qPCR. The immunohistochemistry analysis revealed a low expression of BCL2 and a negative to moderate expression of FZD7 in most samples, while increased CAV1 and RAF1 expression were detected. Moderate to strong CD74 protein expression was observed in endothelial and inflammatory cells. A significant number of JNAs (78%) presented reduced WNT5A and increased WNT5B expression. Overall, the transcript and protein profile indicated the involvement of EMT and WNT pathways in JNA. These candidates are promising druggable targets for treating JNA.