Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Juvenile Nasopharyngeal Angiofibroma Juvenile nasopharyngeal angiofibroma (JNA) is a histologically benign yet locally aggressive vascular head and neck tumor. JNA affects almost exclusively adolescent boys, but has been reported in women and elderly patients on rare occasions. JNA is an uncommon tumor, with reported incidence between 1 in 5000 and 1 in 60,000 otolaryngology patients. It is estimated to account for only 0.5% of all head and neck neoplasms, but is nevertheless considered the most common benign neoplasm of the nasopharynx. The histogenesis and pathogenesis of JNA are unclear. Popular theories include abnormal growth of embryonal chondrocartilage, testosterone acting on a hamartomatous nidus of inferior turbinate tissue mislocated in the nasopharynx, and tumor growth from normal nasopharyngeal fibrovascular stroma. Other suggested etiologies include trauma, inflammation, infection, allergy, and heredity. The site of origin of JNA is usually broad-based, on the posterolateral wall of the nasal cavity, where the sphenoidal process of the palatine bone meets the horizontal ala of the vomer and the root of the pterygoid process of the sphenoid. This area forms the superior aspect of the sphenopalatine foramen, and the posterior aspect of the middle turbinate. From its origin, tumor spreads into the nasal cavity and nasopharynx, displacing the soft palate inferiorly and sometimes becoming visible through the mouth. At the same time, the tumor extends laterally through the sphenopalatine foramen into the pterygomaxillary fossa. From there the JNA exerts pressure on the surrounding bony walls. Anteriorly, it pushes forward the posterior wall of the maxillary sinus, creating the classic "antral bowing sign" visible by x-ray. Posteriorly, it disrupts the root of the pterygoid plates. Superiorly, tumor expands into the orbit via the inferior orbital fissure, continuing eventually into the superior orbital fissure and middle cranial fossa. As tumor squeezes through the superior fissure, it widens the fissure's lower lateral margin, another sign commonly seen radiographically. With further lateral expansion, the tumor will pass through the pterygomaxillary fissure into the infratemporal fossa, often creating a bulging of the cheek. If it reaches the temporal fossa, the tumor can create a bulge above the zygoma. The ultimate danger of unchecked growth by JNA is intracranial extension. The tumor reaches the cranial vault through three paths. The two lateral paths are through the superior orbital fissure and directly through the greater wing of the sphenoid bone from the pterygomaxillary and infratemporal fossae. These paths bring JNA up lateral to the carotid artery and cavernous sinus. The medial path, which can bring tumor into contact with the pituitary and optic chiasm, leads directly through the sphenoid sinus and sella turcica, medial to the carotid and cavernous sinus. Tumor in this area can be extremely difficult or impossible to resect without unacceptable morbidity. Fortunately, this pathway is less common than the lateral pathways. Grossly, the JNA is a lobulated, firm, non-encapsulated mass, usually pink-gray or purple-red. The tumor base may be sessile or pedunculated, but the tumor often has numerous secondary attachments, complicating resection in continuity. Microscopically, the tumor is composed of thin-walled vessels of varying caliber in a mature connective tissue stroma. The vessels typically have a single endothelial cell lining without a muscularis layer, which probably explains the tumor's propensity for hemorrhage. The diagnosis of JNA is based on history, physical exam, and radiographic studies. The differential must include other benign and malignant lesions of the nasopharynx; among these are choanal polyp, angiomatous polyp, chordoma, nasopharyngeal carcinoma, rhabdomyosarcoma, nasopharyngeal cyst, and pyogenic granuloma. Biopsy of these tumors is generally condemned as both unnecessary and hazardous. The average age at onset of symptoms is 14 or 18 years, depending on the series quoted. A typical age range is between 7 and 21 years. Patients are almost always male. The most common presenting symptoms are nasal obstruction and epistaxis. Symptoms have usually been present for several months before the patient is seen. Other less common symptoms include diplopia, blindness, hearing loss, otitis media, rhinorrhea, anosmia, nasal speech, noisy sleep, mouth breathing, eye pain, and headache. On exam, virtually all patients will have a nasopharyngeal mass, usually pink-to-purple and nodular. Other signs which may be evident include proptosis, palatal bulge, or swelling of the cheek or over the zygoma. As discussed, proptosis and lateral facial swelling are ominous indications of extensive tumor spread. Unfortunately, symptoms are a relatively late development in the growth of JNA; at the time of presentation most patients will have tumor extension well beyond the nasopharynx. JNA has several characteristic radiographic features. CT scanning is currently the mainstay of diagnosis for JNA. Recent articles have explored the merits of MRI, and some authors consider MRI superior to CT in delineating the margins of tumor and in revealing tumor vascularity. Anterior bowing of the posterior wall of the maxillary sinus, the "antral bowing" sign, can be seen in most JNA patients. Other commonly seen radiographic changes include widening of the inferolateral aspect of the superior orbital fissure, distortion of the roots of the pterygoid plates, erosion of the hard palate, erosion of the medial wall of the maxillary sinus, and displacement of the nasal septum. Of course, the tumor itself will be evident as a soft tissue mass extending into these bony areas. The tumor has a characteristic angiographic appearance in the arterial phase of excessive numbers of dilated, tortuous vessels. In the capillary phase, a homogenous, dense stain is seen. The predominant blood supply of most JNA's is the ipsilateral internal maxillary artery. As it grows, the tumor may parasitize bilateral arterial supply from any nearby vessel. Therefore, bilateral internal and external carotid arteriography is indicated in most patients. In patients who have had previous attempts at surgical resection or who have had previous external carotid ligation, supply from as far away as the vertebrals and thyrocervical vessels has been demonstrated. Arterial embolization has been shown both to decrease intraoperative hemorrhage and to lower rates of tumor recurrence in JNA. Several materials have been tried over the years, including non-absorbable Silastic spheres, Gelfoam, dura mater, and polyvinyl alcohol particles. Absorbable Gelfoam was used at The Methodist Hospital several years ago, but non-absorbable polyvinyl alcohol particles are now preferred. Tumor staging has long been considered an important framework within which to evaluate the efficacy of therapy. Sessions et al from Baylor devised the first staging system for JNA in 1981, based on CT findings. Today, intracranial extension is the criterion most commonly used to divide patients into prognostic and therapeutic groups. The two primary therapeutic modalities for JNA over the years have been surgery and radiotherapy. Several adjunctive measures have been tried, including embolization, hormonal therapy, and chemotherapy. The main concerns with radiotherapy, which is delivered by external beam, are malignancies in the irradiated field and the danger of inhibiting normal facial growth in younger patients. Cummings found that, although symptomatic relief is fast, regression of tumor with radiation is slow. In his study, 50% of patients treated with primary radiotherapy had visible tumor in the nasopharynx or nasal cavity 12 months after treatment. Fifty percent of patients who had visible tumor at two years suffered eventual recurrence. Most recurrences after radiotherapy have been blamed on geographic misses, but some authors have blamed inadequate dosing. The proper dosage of radiotherapy is debated. Cummings et al declared 30 Gy for three weeks adequate for tumor control, and found no greater control at higher dosages. Economou et al from UCLA found doses of more that 36 Gy to be required for adequate tumor control. Some have suggested 30 to 35 Gy for moderately-sized tumors, with larger doses for extensive tumors. McGahan et al from Baylor recommend primary irradiation for intracranial tumor using 40 to 46 Gy with 180 cGy fractions. Most authors prefer surgical treatment in all patients with extracranial disease, and reserve radiation for "unresectable" intracranial tumors or as post-operative adjunctive therapy when residual tumor has to be left behind. Concerns with surgical management include intra-operative death secondary to exsanguination, tumor recurrence, and intra-operative injury to vital structures during attempted resection. In the literature, tumor control rates with a single surgical procedure generally range from 70% to 90%. Many surgical approaches have been used against JNA. The decision regarding approach is usually made after reviewing radiographic studies to assess tumor extent, blood supply, and presence or absence of intracranial extension. Midfacial degloving provides good exposure to the target area with excellent cosmesis. Techniques have been combined as necessary to provide full exposure for most tumors. In the case of JNA with extreme lateral extension, a transzygomatic infratemporal approach has been used. Intracranial JNA is estimated to occur in 20% to 25% of cases. Operative complication, recurrence, and mortality are all closely linked to intracranial extension. Invasion lateral to the cavernous sinus is generally considered resectable, while invasion of the sinus itself or medial penetration, with involvement of chiasm and/or pituitary, is generally deemed unresectable and treated with radiotherapy. Twenty male patients with JNA were seen at The Methodist Hospital between 1981 and 1993. The average age was 14.65, and ages ranged from 9 to 21 years. Sixteen patients underwent surgery, and four received radiation therapy. Eighteen patients were treated primarily, and two were transferred from outside institutions after unsuccessful treatment. Seven patients presented with epistaxis and obstruction respectively, and five patients presented with these two symptoms together. One patient presented with rhinorrhea. The most common tumor stage using the Sessions staging system was 2B. All patients underwent arteriogram; surgical candidates were embolized, with Gelfoam in the early part of the series, and with PVA for the latter patients. Several different approaches were used; midfacial degloving is the most popular approach currently. All irradiated patients received 46 Gy dosing. There were five recurrences: four surgical, one radiotherapy. Recurrences came with virtually every surgical approach, equally with both kinds of embolic materials, and with both early and late stage patients. Average estimated intraoperative blood loss was about one liter, with six patients requiring transfusions. Our experience at Baylor suggests that most extracranial tumors can be controlled in one procedure with a carefully planned approach, but that recurrences can occur at all tumor stages. Likewise, most intracranial tumors can be controlled with 40 to 46 Gy of radiotherapy with low morbidity. Case Presentations Case 1 A 9-year-old boy presented to the otolaryngology service with a history of several months of nasal congestion and a one-month history of occasional epistaxis. On examination the patient had a large fleshy mass completely filling the nasopharynx. CT scanning on the day prior to admission revealed a nasopharyngeal soft tissue mass with extension into the paranasal sinuses and bony destruction. Bilateral internal and external carotid arteriograms showed primary feeding vessels from pterygomaxillary branches of the right internal maxillary artery, with no significant contributions from the right internal carotid or from the left carotid system. The patient had polyvinyl alcohol particle embolization of the right internal maxillary feeders, and on the next day underwent resection of tumor through a right lateral rhinotomy approach. Estimated blood loss was 1250 cc, and the patient received one unit of packed red blood cells. He tolerated the procedure very well and was discharged on the sixth post-operative day. Case 2 A 20-year-old white man presented to the otolaryngology service with recurrent epistaxis and nasal obstruction. He had been diagnosed in 1986 with juvenile nasopharyngeal angiofibroma (JNA) with intracranial extension, and had been treated at that time with 4600 cGy of radiotherapy. He had an excellent clinical response with resolution of his symptoms, and subsequently had been asymptomatic until a few months prior to this clinic visit. Examination revealed a nasopharyngeal mass, and CT scanning of the head and sinuses revealed a soft tissue mass occupying the nasopharynx, left maxillary sinus, sphenoid sinus, and left pterygomaxillary fossa. Bilateral external and left internal carotid arteriography revealed that the primary feeding vessels to the tumor were branches from the left internal maxillary artery, with small contributions from a branch of the cavernous segment of the left internal carotid artery and the right internal maxillary artery. 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Arch Otolaryngol Head Neck Surg 1991;117:796-799. Wilson GH, Hanafee WN. Angiographic findings in juvenile nasopharyngeal angiofibroma. Clin Radiol 1970;21:279-284. Witt TR, Shah JP, Sternberg SS. Juvenile nasopharyngeal angiofibroma: a 30 year clinical review. Am J Surg 1983;146:521-525. Grand Rounds Archive | Department Home page BCM Public | BCM Intranet | Privacy Notices | Contact BCM | BCM Site Map | ©2001-2006 Baylor College of Medicine
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