| 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 So this morning I would like to talk about juvenile nasopharyngeal angiofibroma. This is a benign vascular neoplasm which presents primarily in adolescent males, although there have been anecdotal reports of this being found in females as well as adults. The mean age is approximately 14 years of age although reported age range has been between 9 and 40 years. There have been several investigations as to the underlying etiology of this lesion although it has not been definitively determined. The thinking is that there might be an androgen dependence related to this given the presentation in young males during the puberty age. Estrogen and progesterone receptors have been negative but dehydrotestosterone receptors have been found to be highly positive in these lesions, suggestion that JNA may, in fact, be androgen dependent. Just going over some of the historical perspective on this mass. According to Akuna the condition was first known and treated by *hypocrites; however, the first case was treated by medial maxillectomy was performed by Elistin in 1841. Telius in 1847 then described the fibrous nature of an intranasal lesion that occur around the time of puberty and in 1873 Gosalin emphasized the occurrence of a nasopharyngeal fibrous polyp that occurred primarily in young males as well. He stated that some of these lesions regressed as the patient reached adulthood, while others required surgical excision. In 1906 Shavot first introduced the term of juvenile nasopharyngeal fibroma and then in 1940 Friedburg suggested the name of angiofibroma. It has since been termed JNA, juvenile nasopharyngeal angiofibroma or juvenile nasopharyngeal fibroma. The classic presentation of JNA is usually this triad of nasal obstruction, epistaxis and an intranasal mass. Kids, however, can also present with cheek swelling, proptosis, visual changes, even otologic complaints such as oral fullness, tinnitus or hearing loss, or even cranial neuropathies. These more unusual findings, however, are more often suggestive of more extensive disease. In a large series of 150 patients by Bremer in 1986 in young age 7 to 29 he sort of broke down the symptoms. This was done at the Mayo Clinic and as you can see again, 70 to 90% presented with epistaxis, 80 to 90% presented with nasal obstruction, and the other symptoms were less commonly presented. The differential diagnosis of JNA also includes inflammatory polyps with neovascularization, hemangiomas, chordomas, well vascularized granulomas or pyogenic granulomas, nasopharyngeal cysts. It is also important to recognize neoplasms, malignant neoplasms such as rhabdomyoma sarcoma and nasopharyngeal carcinoma. The origin of JNA was classically described by Neal and this was noted to be off the posterolateral wall of the nasopharynx at the superior margin of the sphenopalatine foramen. This is located at the trifurcation of the sphenoid process of the palatine bone, the vomer and the root of the pterygoid and the process of the sphenoid. Based on this location Neal went on to described the classic patterns of growth for JNA. Based on the location it can grow inferiorly down into the nasopharynx and postnasal space. It can progress posteriorly and erode through the anterior space of the sphenoid sinus and back and through and disrupt the roots of the pterygoid plates. It can grow anteriorly and into the nasal fossa. It can expand and become continuous with the inferior or middle turbinate or push forward into the posterior wall of the maxillary sinus. It can also grow laterally into the pterygopalatine fossa or the infratemporal fossa or even through the orbital fissures and dura of the middle cranial fossa. The gross appearance of these tumors are often unremarkable. They are often, pale red or blue; smooth, often lobulated but nonencapsulated. They can be pedunculated or sessile. Going over the histology of these lesions, they are usually a rich vascular network and buried in a very thick fibrous stroma composed of spindle shaped cells in a dense collagen matrix. The unique finding here is that there is no surround smooth muscle around these vascular channels, making these more likely to bleed briskly. There have been multiple classification systems that have been established regarding JNA. One of the earlier ones came out of Baylor in 1981 by Sessions based on radiographic CT findings and this is his classification system. He defined the grade 1 as being limited to the nose or the sinus, grade 2 being extension into the pterygomaxillary fossa or into the intertemporal and grade 3 with intracranial extension. Dr. Radkowski in 1996 published a report out of archives which modified this classification system with further 3A and 3B regarding extradural versus intradural intracranial extension. The other commonly used classification system was established by Fish. This came out in Laryngoscope in 1989 and he sort of spent more emphasis on delineating the infratemporal fossa and intracranial extent of the tumors with grade 1 also being confined to the nasopharynx, 2 being into the pterygomaxillary fossa or into the sinuses; grade 3 being into the infratemporal with and without intracranial involvement and grade 4 being intracranial intradural involvement. There have been classic radiographic findings regarding JNA traditionally based on plain films. This was described by Holman and Miller in 1965 and then by Dr. Session and Dr. Alford in 1975. On the common, plain radiographic findings are the presence of a nasopharyngeal mass, anterior bowing of the posterior wall of the antrum. There can be erosion of other sinus walls including the sphenoid bone or the medial wall of the maxillary sinus. There can be displacement of the nasal septum or erosion into the hard palate. The anterior bowing of the maxillary antrum is considered to be pathognomonic for angiofibroma. This slide came out of Dr. Sessions and Dr. Alford’s paper with the control being the normal on left-lateral view versus the bowing of the JNA lesion. Today, CT scanning and MRI are the most commonly used modalities for imaging of JNA. The diagnosis is usually based on the imaging and the physical examination findings. Because of its vascular nature biopsy of these is contraindicated. The site of the mass, as I stated, is often in the nose or often into the pterygopalatine fossa. You can see erosion of the sphenopalatine foramina at the root of the pterygoid plate. CT scan findings are often very good to delineate the extent of the bony erosion. MRI scanning may also be helpful. This is classically more helpful in delineating the soft tissue margins or the soft tissue extent of the tumor. For larger lesions it may be helpful in delineating the intracranial extent or intracranial involvement of the tumor. The classic MRI findings are the presence signal flow voids and strong enhancement following gadolinium contrast. Angiography is primarily reserved for preoperative embolization although it may assist in the diagnosis. It can define the arterial source of the tumor which is often from the ipsilateral internal maxillary artery. There have been multiple different kinds of materials that have been used for embolization including Gelfoam. Non-absorbable polyvinyl alcohol particles are now the preferred materials that are used today. Interestingly to note, while most authors continue to recommend preoperative embolization, there have been some reports by McComb and others who have suggested that while preoperative embolization is valuable in minimizing intraoperative blood loss, it shrinks the tumor and therefore makes it difficult to identify the full extent of the tumor margins, thereby increasing the theoretically potential for recurrence. The management for JNA is primarily surgical. Due to advancements in preoperative embolization, improved skull base surgical approaches and microsurgical techniques, surgery is usually still the main stay for initial therapy; however, radiotherapy and other modalities have been described and we will just briefly go over these today. Fagan, in 1997, went through an algorithm as to how to decide the surgical approach of these tumors. In deciding the surgical approach we basically need to look and consider each patient individually depending on the extent of the disease and the primary location of the tumor. We will sort of briefly go over these. For smaller tumors confined to the nasopharynx, nasal cavity, ethmoids or sphenoids an endoscopic approach may be attempted. Transpalatal approaches have been described. You can do a transfacial or mid-faced degloving approach and this can even be extended using Le Fort I osteotomies. You can do a lateral rhinotomy, medial maxillectomy approach or infratemporal fossa and facial translocation approach as well. For more extensive lesions involving the infratemporal fossa, the medial cavernous sinus, middle cranial fossa, more extensive approaches including the infratemporal fossa approach and the facial translocation approach or an extended Le Fort I, mid-faced degloving approach has been described. So we will sort of briefly just try and go through some of these. The transnasal endoscopic excision approach has become increasingly popular for small tumors, particularly those confined to the nasopharynx or class I or II lesions. This came out of one of the reviews on the endoscopic approaches where he defined the tumor extent that are amenable to endoscopic procedures; tumors that extend intranasally with minimal extension into the infratemporal fossa or just into the anterior portion of the sphenoid. Again, preoperative embolization is essential for these lesions and also requires a very skilled endoscopic surgeon. To do these endoscopic approach it is important to understand some of the basic surgical endoscopic techniques requiring wide maxillary antrostomies, anterior ethmoidectomies, removal of the middle turbinate for improved exposure, and removal of the posterior wall of the maxillary antrum to gain access into the pterygomaxillary fossa. Other advances in endoscopic surgery that have proven to be beneficial include improvements in instrumentations, use of suction irrigation instruments. There have been described reports of using KTP laser to help with minimizing blood loss as well as using 3-dimentional intraoperative imaging to assist with the excision. For tumors that are not amenable to endoscopic procedures, stages I through III the most commonly used approaches include the transpalatal approach, the mid-faced degloving approach. This can be extended with Le Fort I osteotomies or the lateral rhinotomy approach. The transpalatal approach begins sort of from the same position as the tonsillectomy with insertion of a mouth gag and then requires division of the soft palate and overlying soft tissues, retraction of these soft tissues and then removal of the bony portions of the hard palate. You then remove the floor of the nasal cavity to improve your exposure and this permits access to the sphenoid sinuses, the sphenoid bone. You have your intersinus septum for a landmark and the nasal septum and the hard palate. The advantages of a transpalatal approach is that it permits good exposure into the nasopharynx and posterior nasal cavity. The complications or drawbacks of this approach are the development or risks for development of an oral nasal fistula, resultant velopharyngeal dysfunction and possibilities for eustachian tube dysfunction. One approach that is becoming more popular is the mid-faced degloving approach. This approach begins with a gingival mucosa incision from canine to canine and a transfixion incision with separation of the cartilage from the bony nasal pyramid. Then you can extend this excision by removing the anterior maxillary wall. This permits access into the nasal cavity, the paranasal sinuses and the anterior base of the skull. As I stated, the extensions of this approach can include Le Fort I osteotomies to lower the palate and provide increased exposure into the nasopharynx, the posterior nasal cavity and the clivus. The osteotomies would be performed from the lateral piriform rim to the pterygomaxillary fissure with care taken to avoid tooth roots. Here is an example of a mid-faced degloving approach and you can see, the tumor is being delivered through the anterior maxillary sinus. The advantages of this approach include that there are no permanent, external visible scars and it does provide a reasonable exposure as well. The drawbacks of this, or the risks of complication can include scar contracture of the oral vestibule, injury to the infraorbital nerve, nasal vestibular stenosis and cartilage or nasal deformities or tip damage. Should Le Fort I osteotomies be performed, it is important to recognize that these would be osteotomies performed in an adolescent age group and that you need to consider the mid-facial growth and any alterations that might be resultant from this. Another popular approach for accessing stage I through stage III lesions includes the lateral rhinotomy approach. This is often the popular workhorse with approach to the mid-face and anterior skull base. One of the greatest advantages is that it provides broad exposure and direct access often to the tumor in question. As I stated, the advantages of the lateral rhinotomy is that it provides improved lateral exposure. The drawbacks of this include lacrimal duct injury with requirement for dacryocystorhinostomy, lower lid injury or ectropion, hypesthesia with inferior orbital nerve injury and the resultant visible scar. The management of stage 3 and 4 lesions is a little bit trickier. It is important to recognize that approximately 20 to 30% of cases present with intracranial involvement or intracranial extension and adequate access or gaining good vascular control are sort of the big difficulties with regards to these tumors. The surgical management of this is controversial and often associated with increased morbidity. In the absence of extensive intracranial involvement such as into the cavernous sinus or wraping around the intracranial carotid, surgical therapy continues to remain the primary mode for therapy. The intracranial extension usually occurs by way of the middle fossa anterior to the frame and lacerum and lateral to the cavernous sinus and the carotid. There have been multiple combined approaches or different approaches in terms of trying to gain access to these very extensive lesions. In 1979 Jaffeck reported a combined lateral rhinotomy otolaryngologic approach with neurosurgical craniotomy in terms of gaining access here. In 1983 Fisch described the infratemporal fossa approach with respect to gaining access to these nasopharyngeal tumors. In 1988 Gates went through the lateral facial approach. In 1999 Janico described the facial translocation approach. The infratemporal fossa approach described by Fisch has the advantage in that it provides control of the internal carotid artery, good vascular control; however, the drawback does require a subtotal petrosectomy leaving a patient with a maximal conductive hearing loss when he may not have otherwise had any otolaryngologic complaints. In doing this approach this requires a postauricular incision with retraction of the temporalis muscle and temporary dislocation of the zygoma to gain access into the infratemporal fossa. And here you can see by this approach you would have good access and control of the internal carotid artery. The results that he reported from this series: He reported three cases of subtotal resection where further extensive exploration would have resulted in unwarranted morbidity and cranial neuropathies. It is interesting to note, however in his series, patients with subtotal resection had no further evidence of growth at three and five years of followup and that he reported regression of the residual disease. It is also important to note that patients who have residual disease are not necessarily considered recurrences or treatment failures in the absence of symptoms and that recurrences or failures have often in the literature reserved for patients who either have regrowth of the tumor or new onset of symptoms with epistaxis or other complications. The laterofacial approach is similar to Fisch’s infratemporal fossa approach and this was reported by Gates in 1988. This also provides excellent exposure. The advantage of this approach is that it avoids the postauricular incision and avoids the subtotal petrosectomy and begins with a preauricular incision, but also involves the displacement of the temporalis muscle and the temporally dislocation of the zygoma with inferior retraction to gain access into the infratemporal fossa. The drawbacks of both of these procedures include sacrifice of the second branch of the fifth cranial nerve and the potential for trismus. In 1997 Janika described a facial translocation approach which provides direct access with good surgical control. This requires multiple osteotomies through the midface and sacrifices the forehead branch of cranial nerve VII. The advantages of this procedure is that with removal of the midface bones it provides direct wide exposure and access to the tumor at large. However, it is also important to recognize that this again would be osteotomies that would be performed in an adolescent growing population and the resultant affects on facial growth are to be questioned. It is difficult to compare and sort of summarize the overall surgical reports of all of these different series because the tumors are rare enough that most of these patients present with different stages and the approaches that are used have been different by all different authors so there is no good, large series to sort of summarize all of these things but from what I could gather the recurrence rates are obviously much higher for more extensive stage 3 and stage 4 lesions; however, for stage 1 and 2 lesions, or lesions that are small combined to the nasopharynx the success rates have been upwards to 90% with recurrence rates ranging anywhere from 20 to 30%. In Radkowski’s series he reported complete surgical excision being successful in 21 out of 23 patients. However, for this one, this is a report of all the extensive intracranial lesions. Demise’s series there were 21% recurrences, three out of 14 patients using his transfacial approach. Depending on the approach the recurrence rates vary but they range anywhere from 17 to even 86%. Obviously patients with really extensive intracranial involvement are more likely to have recurrences. Other than surgical therapy radiotherapy has been proposed as a treatment option for these patients, particularly in patients with large, extensive, unresectable disease or patients with extensive intracranial involvement. Cummings et al reported the largest series out of the Princess Margaret Hospital in Toronto of 55 patients and he reported in this series 80% success rate for primary radiation therapy when doses of 3000 to 36000 centigrade were used. However, it is important to note if you read his series carefully that the symptomatic, while that may have been immediate, the regression of the tumor is very slow. At one year of followup only 50% of the patient’s treated with primary radiation therapy had regression of the tumor; 50% still had visible tumor in the nasopharynx or nasal cavity at one year and 50% of those patients still had visible tumor at two years. And there were reports of patients still with regression into the third year following radiation therapy. So the regression or response to the radiation therapy is very slow. The drawbacks of radiation therapy or the considerations that you need to take into account when considering this is the possibility for malignant transformation and again it is important to remember that these are adolescents who would be undergoing radiation therapy and the long-term potential for osteoradionecrosis, damage with pituitary dysfunction and damage to the optic nerve with potential for blindness. More recently Dr. Kuppersmith and Dr. Donovan from Baylor advocated the use of intensity modulated radiation therapy for recurrent or extensive JNA with intracranial involvement. They reported a case series of two patients who had recurrent disease and one patient with extensive unresectable disease and these patients were treated with IMRT with radiographic response to therapy. Two of the cases had no evidence of disease endoscopically at 15 and 40 months of followup. The advantage of this is dose limiting to normal tissues and permits higher doses to be delivered to the tumor with supposedly less toxicity to the surrounding tissues. There were no acute toxicities reported from this series. Dr. Goepfert in 1985 reported a series of 5 patients of angiofibroma who were treated successfully using two different chemotherapeutic regimens at M.D. Anderson. These results, while he reported complete successful response to therapy in these five patients, I have not been able to find any other reports of successful treatment or response to chemotherapy. His recommendations are that chemotherapy might be an option or reserved for patients who have recurrent disease and whom further surgery or radiation therapy is no longer an option. There have been other anecdotal reports of treatment options for JNA. These include sclerosing agents, cryotherapy and even estrogen hormonal therapy. None of these have had any significant proven benefit. Additionally there have been also a few anecdotal reports regarding JNA and spontaneous regression in adulthood, or spontaneous regression of tumors following subtotal resection. However, it is important to recognize that this is a vascular and aggressive tumor that often has a propensity to recur if it is not completely resected. So basically in summary, JNA is a benign, locally aggressive tumor which occurs primarily in adolescent males. It is important to recognize that surgery continues to be the mainstay or primary modality for therapy. Radiotherapy, however, may be an option for patients with extensive intracranial or recurrent disease in whom the morbidities of surgery are unwanted. The overall success rate, as I stated, following initial therapy is between 70 and 90%, particularly for smaller tumors. Case Presentation: Case 1 : TM is a 14-year-old male with no significant past medical history who presented to an outside hospital with spontaneous brisk epistaxis, which was refractory to intranasal packing. He subsequently required emergent arteriography with embolization of the left internal maxillary artery to control the bleeding. On questioning, he did report a several week history of progressive nasal congestion prior to this episode. By physical examination, he was noted to have a pink-blue left intranasal mass. He was transferred to Texas Children’s Hospital for definitive care. A CT scan revealed a large left nasopharyngeal and intranasal mass consistent with a juvenile nasopharyngeal angiofibroma. He was scheduled for a repeat angiogram with embolization of the left internal maxillary artery one day prior to surgery. He underwent successful excision of the juvenile nasopharyngeal angiofibroma using a midface degloving approach without complication. He is currently doing well without evidence of disease. Case 2 : SG is a 14-year-old male with no significant past medical history who initially presented with a several week history of nasal congestion and purulent drainage. He was empirically managed with oral antibiotics for presumptive sinusitis; however his symptoms persisted. He was referred to the Otolaryngology service at Texas Children’s Hospital for further care. On examination he was noted to have a large left intranasal mass. A CT scan revealed a large left nasopharyngeal and intranasal mass consistent with a juvenile nasopharyngeal angiofibroma. He underwent arteriogram with embolization of the left internal maxillary artery prior to surgery. He then underwent successful excision of the juvenile nasopharyngeal angiofibroma using a lateral rhinotomy, medial maxillectomy approach without complication. He is currently recovering without complication. Bibliograhpy : Andrews JC, Fisch U, Valavanis A, Aeppli U, Makek MS. 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Tewfik TL, Tan AK, al Noury K, Chowdhury K, Tampieri D, Raymond J, et al. Juvenile nasopharyngeal angiofibroma. J Otolaryngol 1999;28:145-151. Ungkanont K, Byers RM, Weber RS, Callender DL, Wolf PF, Goepfert H. Juvenile nasopharyngeal angiofibroma: an update of therapeutic management. Head Neck 1996;18:60-66. Grand Rounds Archive | Department Home page BCM Public | BCM Intranet | Privacy Notices | Contact BCM | BCM Site Map | ©2001-2005 Baylor College of Medicine
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