Juvenile Nasopharyngeal Angiofibroma (JNA)
JNA’s are well-vascularized benign lesions typically occurring in adolescent males. These tumors are seen in 0.05% of all head and neck tumors with a frequency of 1:5000-1:60,000 in otolaryngology patients. They are exclusively found in males and if diagnosed in a female patient, genetic testing should be performed. The exact etiology is unknown but it is believed to be hormonally derived as the lesions tend to occur in adolescent males.The most common age of presentation in 7-19 years and these tumors are rare in adults.
Although they are benign tumors, they often behave in a locally invasive manner. The tumor generally originates at the sphenopalatine foramen and spreads both into the nasophrarynx and nasal cavity as well as the pterygopalatine fossa. The tumor can spread into all the paransal sinuses, the greater wing of the sphenoid, the infratemporal fossa, orbital fissures and into the nerves including the vidian and branches of the trigeminal nerve through the foramen rotundum.
The most common presentation is nose bleeds and/or nasal obstruction. Additionally, patients may present with headaches, facial swelling, nasal discharge, decreased or loss of smell, or swelling of the soft palate.
Initial evaluation is through an endoscopic examination of the nose and sinuses. This is followed by both a CT and an MRI with contrast. An angiography can be done to see the blood vessels feeding the JNA tumor, but this is typically only done prior to surgery if an embolization is planned (Embo Figures).
Treatment is primarily surgical. Previous these tumors were resected with an open craniofacial incision (facial incisions) for resection. Currently, centers with significant experience perform these completely endoscopically (through the nose only). For larger tumors, patients are often admitted prior to surgery for angiography and emobolization of blood vessels supplying the tumor (this allows a less bloody surgery during resection). Although previously these tumors where often resected in multiple surgeries due to excessive blood loss, currently they usually require only a single operation for complete excision. Although other treatment options have been discussed including cryotherapy, chemotherapy, radiation therapy, and hormonal manipulation, these are generally discussed if surgery is not feasible.
Case Example 1:
17 year old male with profuse right sided nose bleeds during soccer practice. Initially he was seen in the ER and a nasal pack was placed. This was removed after 3 days. 1 day after removal, he again presented with nose bleeds. This time a CT and subsequent MRI was performed demonstrating the JNA (Figures 1-4). The patient was taken in for preoperative embolization the day before surgery to reduce bleeding and subsequently underwent an endoscopic resection avoiding facial incisions. The patient was in the hospital for 2 days following surgery and then discharged without issues. His postoperative imaging demonstrates no evidence of residual tumor or any recurrence (Figures 5-7). He is followed routinely with endoscopic evaluation and MRIs to ensure no further recurrence
Embo Figure 1.This is an angiography demonstrating the vasculature of the left common carotid artery with subsequent bifurcation into the external carotid artery feeding the internal maxillary artery. The tumor (circle) is highly vascular (typical for a JNA). View Image
Embo Figure 2. This is the same tumor following embolization of the internal maxillary artery. Note the decrease in vascularity (same area circled) following embolization. This procedure is typically performed 24-48 hours prior to tumor resection to minimize bleeding during surgical resection. View Image
Case Example 2:
15 year old male with recent history of “allergies” and sinus infections treated with multiple courses of antibiotics as well as oral antihistamines and nasal steroids without relief. Seen by local otolaryngologist who identified a nasal mass consistent with a JNA in the right nasal cavity. Imaging also consistent with JNA (Figure 8,9). This tumor was removed completely endoscopically following embolization 24 hours prior to resection (Figure 10,11). The patient is 2 years post surgery with no evidence of recurrence on routine surveilience MRI.
How Surgery is Performed
Recommended Further Reading
Adappa ND and O’Malley BW. Approach for Juvenile Nasopharyngeal Angiofibroma. Atlas of Endoscopic Sinus and Skull Base Surgery. Editors Palmer JN, Chiu AG. Elseveir Saunders 2013; 219-226. View Textbook
Bleier BS, Kennedy DW, Palmer JN, Chiu AG, Bloom JD, O’Malley BW Jr. Current management of juvenile nasopharyngeal angiofibroma: a tertiary center experience 1999-2007. Am J Rhinol Allergy 2009 May-Jun;23(3):328-30. View Article