ORIGINAL ARTICLE

Bilateral juvenile nasopharyngeal angiofibroma: Report of a case Edward C. Wu, MD, MBA1 , Davin W. Chark, MD2 and Naveen D. Bhandarkar, MD2

Background: Juvenile nasopharyngeal angiofibroma (JNA) is the most common benign neoplasm of the nasopharynx. Almost always unilateral on diagnosis, JNAs are locally invasive and may extend across the midline, giving a false bilateral appearance; as such, true bilateral JNA is exceedingly rare. We present a recent case of true bilateral JNA. Methods: Single case report of a patient with bilateral JNA, including clinical presentation, diagnosis, and management. Results: The patient presented with unilateral nasal obstruction and recurrent epistaxis. Computed tomography and magnetic resonance imaging demonstrated bilateral, noncontiguous masses. Angiography revealed independent vascular supplies from each respective side with no bilateral supply noted. The patient underwent preoperative embolization followed by endoscopic surgical removal of the

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uvenile angiofibroma is a rare, benign vascular tumor that accounts for less than 0.5% of neoplasms in the head and neck.1, 2 Upward of 98% of tumors primarily involve the nasopharynx,3 and are thus termed juvenile nasopharyngeal angiofibroma (JNA), though extranasopharyngeal presentations (eg, maxilla, nasal septum, ethmoid) have also been reported.3, 4 It uniquely presents in adolescent males with unilateral nasal obstruction and recurrent epistaxis. JNA typically arises in the pterygoid canal within the pterygopalatine fossa,5 and, in its early stages of growth, extends into the nasopharynx and nasal cav1 Department

of Head and Neck Surgery, University of California, Los Angeles (UCLA) Medical Center, Los Angeles, CA; 2 Department of Otolaryngology–Head and Neck Surgery, University of California, Irvine Medical Center, Orange, CA

Correspondence to: Naveen D. Bhandarkar, MD, Department of Otolaryngology–Head and Neck Surgery, University of California, Irvine, 101 The City Drive South, Bldg. 56, Suite 500, Orange, CA 92868; e-mail: [email protected] Potential conflict of interest: None provided. Presented as a poster at the 2012 Combined Otolaryngological Spring Meetings, American Rhinologic Society section, April 19, 2012, San Diego, CA. Received: 3 October 2013; Revised: 21 December 2013; Accepted: 29 January 2014 DOI: 10.1002/alr.21322 View this article online at wileyonlinelibrary.com.

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larger mass; no complications were noted. Follow-up at 2 years demonstrated no recurrence or growth. Conclusion: The vast majority of JNAs are unilateral, though invasive growth to the contralateral side may appear “bilateral” in presentation. Proper identification of true bilateral JNA is helpful in guiding management, wherein excision of both tumors may not be necessary.  C 2014 ARS-AAOA, LLC.

Key Words: epistaxis; radiology; imaging; angiofibroma; bilateral How to Cite this Article: Wu EC, Chark DW, Bhandarkar ND. Bilateral juvenile nasopharyngeal angiofibroma: report of a case. Int Forum Allergy Rhinol. 2014;00:X–XX.

ity through the sphenopalatine foramen. Though benign in nature, JNAs have the potential to grow and expand into local structures, with advanced tumors extending into the paranasal sinuses, orbital cavity, infratemporal fossa, or intracranial cavity. A review of the Western literature returned only 1 previously reported case of true bilateral JNA.6 We report here another case of true bilateral JNA.

Case presentation The patient is an 18-year-old male who reported worsening left nasal obstruction over 5 months and initially presented with severe left-sided epistaxis that required electrocautery in the emergency department. Nasal endoscopic exam subsequently demonstrated a large, vascular mass filling the left posterior nasal passage and nasopharynx. The mass extended across the midline of the nasopharynx to fill the superomedial portion of the right choana. The remainder of the head and neck exam and laboratory workup was within normal limits. The patient did not report any rightsided symptoms. Computed tomography (CT) of the paranasal sinuses demonstrated a large 3.4-cm × 2.9-cm × 1.3-cm left-sided posterior nasal and nasopharyngeal mass that was widening the sphenopalatine foramen, expanding the pterygopalatine

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FIGURE 1. Axial CT demonstrates large left-sided mass widening the left pterygopalatine fossa (7.0 mm compared to 4.7 mm on right), suggestive of JNA. CT = computed tomography; JNA = juvenile nasopharyngeal angiofibroma.

fossa, and extending into the left vidian canal and sphenoid sinus, highly suggestive of a JNA (Fig. 1). Although a mass was not visualized on the right side endoscopically, CT demonstrated a mass within the medial pterygopalatine fossa on the right side with widening of the sphenopalatine foramen and vidian canal, with no contiguous extension to the left-sided mass, suggesting possible bilateral masses. Magnetic resonance imaging (MRI) of the face and paranasal sinuses confirmed bilateral, noncontiguous, contrast-enhancing lesions with identical radiographic appearance originating from each respective pterygopalatine fossa, with the right-sided 1.4-cm × 1.0-cm × 0.9-cm lesion extending deeper into the pterygopalatine fossa and vidian canal (Fig. 2). Bilateral angiography traced the vascular supply of the left-sided mass to the left sphenopalatine and ethmoidal branches of the left maxillary artery, and that of the right-sided mass to the right vidian artery (branch of the right internal carotid artery) and right sphenopalatine artery (branch of the right maxillary artery) (Fig. 3). Informed consent was obtained for preoperative embolization and endoscopic, endonasal surgical removal of the left-sided mass, with the patient and family electing to observe the right-sided mass. The procedure and postoperative course were uncomplicated. Histologic analysis of the excised left-sided mass confirmed the diagnosis of JNA. A repeat MRI at 6 months from the prior study revealed no evidence of persistent or recurrent tumor on the left, and no growth of the right-sided mass (Fig. 4). Thus, the patient has elected for continued observation with serial MRIs. A repeat MRI at 22 months postoperatively demonstrated no significant change from the prior study, with the patient remaining asymptomatic.

FIGURE 2. Axial T1-weighted post-gadolinium MRI demonstrates enhancement of the bilateral JNAs. JNA = juvenile nasopharyngeal angiofibroma; MRI = magnetic resonance imaging.

FIGURE 3. Reconstructed image of bilateral external carotid angiography demonstrating bilateral JNA supplied by branches of each respective maxillary artery. Arrows point to JNAs on each respective side. JNA = juvenile nasopharyngeal angiofibroma.

Discussion In the vast majority of cases, JNAs occur in a characteristic manner: unilaterally in adolescent males. Rarely, deviations from this classic presentation have been reported in the literature. To our knowledge, there has been only 1 case of true bilateral JNA in a 21-year-old male.6 We present here another case of true bilateral JNA in an 18-year-old male patient. Aside from direct visualization of the nasopharyngeal mass on nasal endoscopy, the diagnosis and staging of

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FIGURE 4. Axial T2-weighted MRI at 4 months postoperatively shows postsurgical changes in left nasopharynx and right-sided JNA with no noted change in size. The patient reports no right-sided symptoms. JNA = juvenile nasopharyngeal angiofibroma; MRI = magnetic resonance imaging.

JNA can usually be made through CT and MR imaging of the paranasal sinuses.7, 8 CT demonstrates a soft tissue mass originating at the sphenopalatine foramen margin that demonstrates enhancement if contrast is administered, whereas MRI demonstrates a heterogeneous, intermediateto-high signaled, contrast-enhancing mass in the same location. This patient initially presented only with left-sided symptoms (unilateral nasal obstruction and recurrent epistaxis) with a mass readily visible on nasal endoscopy. The patient’s right-sided mass was not directly visualized during endoscopy given that it was completely submucosal, and was only detected once radiographic imaging was reviewed. Oftentimes, imaging reveals widening of the pterygomaxillary fissure with anterior bowing of the posterior maxillary sinus wall (Holman Miller sign). Though classically associated with JNAs, this radiologic finding is unspecific,9, 10 and was not present in this patient. Carotid angiography is often used to identify the vascular supply of the JNA. Traditionally, the majority of JNAs originate from 1 or more of several ipsilateral vascular networks, usually branches of the maxillary and/or ascending pharyngeal arteries. Bilateral vascular supply may be underappreciated (nearly 40% of cases).11 In order to minimize the risk of profuse intraoperative hemorrhage, there is potential value in systematically evaluating for bilateral vascular supply. In addition, although JNAs may extend into the opposite nasopharynx by direct extension, true bilateral JNAs, characterized by 2 masses with distinct vascular supplies originating from each respective side, are exceed-

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ingly rare, with only a single previously reported case.6 In this case, angiography was used not only for identification of vascular supply and embolization, but also served to confirm the separate vascular supply of the right-sided lesion. Given similar radiographic and angiographic characteristics when compared to the left side, we presumed a diagnosis of JNA in the absence of histologic confirmation. Typically, diagnosis of JNA through biopsy is avoided to prevent severe hemorrhage. There have, however, been rare reports of diagnoses of JNA made on clinical examination and imaging studies, but that on histologic analysis revealed a different tumor type, such as embryonal rhabdomyosarcoma12, 13 or lymphoepithelioma.9 The clinician must therefore be wary of any atypical clinical presentations (eg, constitutional symptoms) or deviations from classical diagnostic imaging findings, and consider histologic diagnosis as a possible option. If the clinician decides to biopsy the mass, it is best performed in the operating room under general anesthesia given the highly vascular nature and risk of bleeding. In this case, as the patient’s demographic data (ie, age and gender), physical exams (ie, general and endoscopic), and imaging results (ie, CT, MRI, and angiography) were all consistent with the diagnosis of JNA, biopsy was not performed for either mass. Management of JNA depends on tumor stage, but generally involves surgical removal of the mass through a variety of approaches. External beam radiation therapy may be considered for residual tumors, nonoperative patients, or intracranial extension, with local tumor control reported to be greater than 80%.14 Preoperative embolization has been shown to be effective in reducing intraoperative blood loss and was performed for this patient.15, 16 If true bilateral JNA were bilaterally symptomatic, the surgeon may consider a simultaneous procedure, but should be prepared for a staged procedure due to the possibility of significant hemorrhage from 2 simultaneous surgical sites and increased operative time. In the latter instance, accurate identification of separate tumors is important to avoid unnecessary embolization of a tumor not planned for resection. As this patient’s right-sided JNA was completely asymptomatic, the decision was made to not embolize vascular supply from the right and to clinically monitor the mass with consideration of future surgical management if necessary. Of note, JNAs are rarely reported to undergo malignant transformation unless subjected to radiotherapy, and we do not expect this to occur in this patient.17 When left alone, JNAs have, in a few cases, been reported to spontaneously regress over time with increasing age.18, 19 Though poorly understood, spontaneous regression of JNAs is thought to be related to postpubertal hormonal changes that deprive the tumor of trophic factors.18, 19 Stansbie and Phelps20 reported a case of gradual tumor involution following partial resection of a massive JNA, suggesting that disruption of the tumor architecture and vasculature may in fact prevent further growth and incite clinical regression. Though complete surgical resection remains the goal whenever possible, tumor debulking

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(for large, difficult-to-access tumors) or observation (for small tumors) with close, long-term surveillance may thus be reasonable management options, especially in consideration of minimizing surgical morbidities. For this particular patient, observation of the right-sided tumor was elected with no interval change in size or symptoms. As most tumor recurrences tend to occur within 3 to 4 months postoperatively,21 it has been recommended that patients undergo scheduled surveillance visits every month for the first 6 months with an initial MRI at 6 months after surgery. If there are no signs of recurrence, visits can be extended to every 3 to 6 months with an MRI annually to monitor for recurrence.22 Though we fully support and follow these recommendations, it is unclear at this stage whether an annual MRI is necessary, particularly when several years out from tumor resection, in the absence of recurrent or new symptoms. Logically, if small tumors can be observed with no growth or perhaps regression in the

postpubertal period, fully resected tumors may not require the traditionally accepted frequency of radiographic imaging. Though our current data should not necessarily change current paradigms, further insight into this issue may be initially achieved by large-scale retrospective data analysis of patients in the follow-up period.

Conclusion JNA is a rare, typically unilateral, highly vascular tumor of the head and neck. Although large tumors extending to the contralateral side will appear bilateral and may be vascularized or symptomatic bilaterally, completely separate bilateral tumors with separate vascular supplies are exceedingly rare. Proper identification of true bilateral tumors is helpful in guiding management, wherein immediate excision of the asymptomatic tumor may not be necessary given reliable follow-up.

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16. Roberson GH, Biller H, Sessions DG, Ogura JH. Presurgical internal maxillary artery embolization in juvenile angiofibroma. Laryngoscope. 1972;82:1524– 1532. 17. Donald PJ. Sarcomatous degeneration in a nasopharyngeal angiofibroma. Otolaryngol Head Neck Surg. 1979;87:42–46. 18. Herman P, Lot G, Chapot R, Salvan D, Huy PT. Long-term follow-up of juvenile nasopharyngeal angiofibromas: analysis of recurrences. Laryngoscope. 1999;109:140–147. 19. Tosun F, Onerci M, Durmaz A, Ugurel S. Spontaneous involution of nasopharyngeal angiofibroma. J Craniofac Surg. 2008;19:1686– 1689. 20. Stansbie JM, Phelps PD. Involution of residual juvenile nasopharyngeal angiofibroma (a case report). J Laryngol Otol. 1986;100:599– 603. 21. Douglas R, Wormald PJ. Endoscopic surgery for juvenile nasopharyngeal angiofibroma: where are the limits? Curr Opin Otolaryngol Head Neck Surg. 2006;14:1–5. 22. Seiberling K, Wormald PJ. Benign sinonasal tumors. In: Kennedy DW, Hwang PH, eds. Rhinology: Diseases of the Nose, Sinuses, and Skull Base. New York: Thieme; 2012:668–687.

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Bilateral juvenile nasopharyngeal angiofibroma: report of a case.

Juvenile nasopharyngeal angiofibroma (JNA) is the most common benign neoplasm of the nasopharynx. Almost always unilateral on diagnosis, JNAs are loca...
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