Introduction: Juvenile nasopharyngeal angiofibroma is a rare, highly .. Luiz A CR, Romualdo S LT, Fava A S. Angiofibroma nasofaríngeo: revisão de literatura. Juvenile nasopharyngeal angiofibroma (JNA) is a rare benign tumor arising predominantly in the nasopharynx of adolescent males. It is an aggressive. Juvenile nasopharyngeal angiofibromas (JNA) are a rare benign but locally aggressive vascular tumor. Epidemiology Juvenile nasopharyngeal angiofibromas.
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Professor in the Department of Otolaryngology. Service chief medical residency in Otorhinolaryngology, Universidade Federal de Sergipe.
Juvenile nasopharyngeal angiofibroma is a rare, highly vascular, and histologically benign tumor, generally observed in male adolescents. It shows very aggressive behavior due to local invasiveness and is associated with various symptoms.
Nasopharyngeal Angiofibroma: Review of the Genetic and Molecular Aspects
Juvenile nasopharyngeal angiofibroma originates in the sphenopalatine forame, causing epistaxes and nasal obstruction. To retrospectively describe our experience in the diagnosis and treatment of patients with nasofaringeoo nasopharyngeal angiofibroma. Retrospective, descriptive study conducted after approval from the Ethics Committee of the Federal University of Sergipe protocol We analyzed findings in 20 patients who underwent surgery between and Factors analyzed include patient age and gender, symptoms, stages, treatment, length of surgery, angiofibrona bleeding, postoperative need for nasal tampons, hospitalization time, complications, and tumor recurrence.
Patients were aged 10—29 years.
Endoscopic Surgery of Nasopharyngeal Angiofibroma
All patients were treated surgically, including 17 who underwent endoscopic surgery. Seventeen patients required clamping of the external carotids and tumor embolization.
Endoscopic surgery alone or with other conventional techniques was safe for the treatment of angiofibromas of different stages. Nasopharyngeal angiofibroma is a histologically and biologically benign tumor with aggressive behavior due to its location and associated symptoms including significant epistaxis and nasal obstruction 1 2 3 4 5.
These tumors occur almost exclusively in male adolescents and account for 0. The tumors are frequently located in the posterolateral wall of the nasal cavity, where the sphenoid process of the palatine bone articulates with the base of the pterygoid process, forming the sphenopalatine foramen. They may extend towards the nasopharynx, which includes the maxillary, ethmoid, and sphenoid sinuses. Laterally, these tumors may extend to and fill the pterygomaxillary fossa, causing the posterior wall of the maxillary sinus to bulge and eroding the pterygoid plate.
Tumors that further extend into the infratemporal fossa may progress into the orbit, through the inferior orbital fissure and to the base of the skull through the base of the pterygoid process. From a triangular region formed by the foramen ovale, a round and lacerated tumor may reach the middle fossa, migrating to the parasellar region and remaining generally extradual and lateral to the cavernous sinus. Tumors may invade the anterior fossa through the ethmoid and sphenoid sinuses, being more frequent in the middle fossa and remaining extradural 8 9 The classic triad of epistaxis, unilateral nasal obstruction, and a mass in the nasopharynx suggests a diagnosis of nasopharyngeal angiofibroma and is supplemented by imaging 11 12 13 14 A biopsy is recommended only in cases of diagnostic uncertainty4.
Examinations such as computed tomography, nuclear magnetic resonance and even nasal endoscopy can clearly establish the extent of the tumor, its pattern of spread, and consequently, surgical planning 10 11 12 16 17 18 These tests have led to the formulations of multiple classification methods, including the FischR adkowskiA ndrewsB remerand A ntonelli methods 1 4 5 7 8.
These classifications are very important in helping surgeons decide the appropriate surgical approach. Non-surgical treatments are available, including the use of hormones estrogen, testosteroneradiation therapy, chemotherapy, and recently, embolization; however, surgery is considered the treatment of choice, with the route depending on the stage of the tumor and the overall condition of the patient 11 12 Vascularization arises most frequently from the maxillary artery i.
Juvenile nasopharyngeal angiofibroma
Surgical approaches for conventional techniques include transpalatal, transmaxillary, lateral rhinotomy, mid-facial degloving, extended sublabial Denker, and Le Fort type I osteotomy 11 13 20 The development of minimally invasive techniques has led to the increased use of endoscopic surgery for the treatment of nasopharyngeal angiofibroma 21making it ideal for tumors confined to the nasopharynx, nasal cavity, and sphenoid sinus with minimal extension into the pterygopalatine fossa 10 12 13 15 18 The first description of an endoscopic resection was published in Since then, several cases have been reported, all showing that endoscopic resection resulted in lower morbidity during the early stages of disease In this study, we have described our experience in treating 20 patients with nasopharyngeal angiofibromas in the Department of Otorhinolaryngology.
We analyzed 20 patients with nasopharyngeal angiofibroma who underwent surgery in the otolaryngology service between and Variables analyzed included patient age, sex, symptoms, tumor stage, treatment, duration of surgery, volume of intraoperative bleeding, need for nasal packing after surgery, length of hospitalization, immediate and late complications, recurrence, and preoperative embolization and clamping of the external carotid during surgery.
All patients were classified radiologically and surgically according to the Fisch system.
All patients were male, and all had symptoms of progressive nasal obstruction and recurrent epistaxis. Surgical methods in all patients were based on Fisch classifications. Of the 20 patients, 3 had surgery by combined approaches conventional and endoscopicincluding 1 patient with a Fisch IIIA tumor who was treated by the Caldwell-Luc expanded technique and endoscopic surgery, 1 patient with a Fisch IIIA tumor who was treated by the degloving mid-facial technique and endoscopic surgery, and 1 with a Fisch IV tumor who was treated by the degloving mid-facial technique associated with craniotomy and endoscopic surgery.
The other 17 patients underwent endoscopic surgery alone. None of these patients required postoperative nasal packing, and all were discharged the day after surgery.
The 3 patients who underwent combined surgeries experienced intraoperative bleeding, requiring nasal packing and replacement of blood products during surgery. All 20 patients were angiodibroma for follow-up maximum, 7 yearswith none showing evidence of tumor recurrence.
The first patient who underwent surgery in our department had a Fisch I tumor, but did not undergo embolization or clamping of the external carotid arteries. The second patient, also with a Fisch I tumor, underwent clamping of the external carotid arteries without embolization.
The third patient with a Fisch I tumor underwent surgery with embolization, but without clamping of the external carotid arteries. The remaining 17 patients, regardless of angioibroma tumor classification, underwent embolization and clamping of the external carotid arteries due to better control of bleeding during surgery. Angioflbroma nasopharyngeal angiofibromas present almost exclusively in men aged 14—25 years 23 24 25 26 27 Similarly, our patients ranged in age from 10 to 29 years, with a mean age of 16 years, and all were male.
Several classification methods have been utilized to stage tumors and assist in nasofaingeo the appropriate treatment. These classification systems are based on examination methods, including CT, MRI, and endoscopy, and have been utilized to establish the extent of the tumor, its pattern of spread, and consequently, surgical planning 10 11 12 16 17 18 Computed tomography of a year-old patient with a Fisch class IIIa juvenile nasopharyngeal angiofibroma. CT of a year-old patient with a Fisch class II juvenile nasopharyngeal angiofibroma.
Several surgical approaches have been utilized for the removal of nasopharyngeal angiofibromas, including transnasal, transpalatal, transzygomatic, and transcervical accesses, in addition to lateral rhinotomy and mid-facial degloving, with or without extension to the upper lip or concomitant craniotomy 9. The combination of different accesses to angiofibeoma tumor can be required 9. Of our 20 patients, 3 required a combination of endoscopic and angiofkbroma surgery, with one, with a Fisch IIIA tumor, having expanded Caldwell-Luc and endoscopic techniques; one, with a Fisch IIIA tumor, undergoing mid-facial degloving and endoscopic surgery; and one, with a Fisch IVA tumor, having mid-facial degloving, craniotomy, and endoscopic surgery Figure 4.
The other 17 patients required only endoscopic surgery. Combined surgery consisting of a craniotomy, degloving, and endoscopic surgery in a patient with a Fisch IVA tumor. Longer duration of surgery is associated with greater changes in body homeostasis due to surgical trauma. Improvements in surgical techniques are designed to shorten surgical time and thereby reduce patient morbidity.
Endoscopic surgery is less invasive than open surgery, causing less damage to the patient. Moreover, the mean blood loss in these patients was mL.
This relatively low blood loss may be due to the use of surgical endoscopy, providing better control of bleeding than open surgery, with or without endoscopy.
Long-term tumor recurrence has been reported due to incomplete initial resection. For example, a retrospective study of 15 patients found tumor recurrence in 1 patient 6. The recurrence rate of endoscopically resected tumors has been shown to be low, and we found that none of our 20 patients experienced tumor recurrence. This may have been due to their relatively early diagnosis, when tumors are found smaller and easier to remove completely, as well as due to the use of endoscopic surgery, which assists in controlling tumor removal, including more accurate examinations of the spaces previously occupied by the tumor.
Many studies highlight the benefit of preoperative arterial embolization to control bleeding intraoperative 16 29 Studies comparing surgical treatment with or without embolization have shown that preoperative embolization reduced intraoperative blood loss and the need for replacement of blood products 31 Blood loss, which was — mL in a non-embolized patient, was reduced to — mL in embolized patients 31 32 Of our 20 patients, only 2 underwent surgery without embolization, with these 2 showing significantly greater intraoperative bleeding than the 18 patients who underwent embolization.
Tumor size was not associated with bleeding. Embolization of a year-old patient with a Fisch IIIa juvenile nasopharyngeal angiofibroma. Some surgeons, however, do not routinely perform embolization preoperatively, finding that embolization is justified only in larger tumors The volume of intraoperative bleeding has been shown to be similar in patients with and without embolization 34whereas tendency to relapse was greater in patients undergoing embolization.
In addition, preoperative embolization may complicate the identification of the full extent of surgical margins by reducing the tumor size and increasing the risk of relapse None of our patients experienced complications due to embolization.
However, as in any invasive procedure, embolization presents risks that should be fully explained to the patients The most significant risk is blindness, for patients with occlusion of the ophthalmic or middle cerebral artery through the collateral circulation. To minimize complications, surgery should be performed at centers with extensive experience.
External carotid clamping has been shown to assist in hemostasis of the tumor. Intraoperative control of bleeding during the resection of nasopharyngeal angiofibromas can be achieved successfully by temporary clamping of the external carotid arteries in the neck Of our 20 patients, only 2 underwent surgery without clamping of the external carotid arteries, with both showing more bleeding than the 18 who underwent surgery with clamping.
Tumor volume was not associated with bleeding in patients with and without clamping. We also found that patients who underwent clamping of the external carotid artery and embolization showed less intraoperative bleeding than patients who underwent embolization alone. Patients undergoing embolization require external carotid clamping due to collateral circulation, which can increase bleeding.
Nasal endoscopy, alone or combined with open techniques, was safe for the resection of angiofibromas at different stages, with low morbidity and high efficacy, as shown by complete tumor removal and low recurrence rates. Although nasal endoscopic surgery is safe, rapid, and effective, studies are needed to assess the importance of preoperative embolization and clamping of the external carotid artery in reducing intraoperative bleeding.
National Center for Biotechnology InformationU. Journal List Int Arch Otorhinolaryngol v. Find articles by Francis Vinicius Fontes de Lima. Find articles by Eduardo Passos Fiel de Jesus. Find articles by Arlete Cristina Granizo Santos. Author information Article notes Copyright and License information Disclaimer. Universidade Federal de Sergipe. Received Aug 21; Accepted Oct 7. This article has been cited by other articles in PMC. Introduction Nasopharyngeal angiofibroma is a histologically and biologically benign tumor with aggressive behavior due to its location and associated symptoms including significant epistaxis and nasal obstruction 1 2 3 4 5.
Classification of Fisch Type I: