Presenting signs and symptoms include epistaxis, nasal obstruction and nasal drainage.

This rare tumour-like lesion is characterized by architecturally irregular vessels set in a fibrous stroma.

These lesions are benign histologically but they may become life-threatening with excessive bleeding or intracranial extension.

Angiogenic factors are discussed to participate in growth and promotion of juvenile nasopharyngeal angiofibroma (JNA). However, only few data are available and mechanisms remain unclear. In the presented study we analysed the expression and subcellular distribution of several angiogenic growth factors and receptors potentially involved in JNA-growth and -vascularisation. In a retrospective, descriptive, multicenter-study, we analysed 13 formalin-fixed, paraffin-embedded or cryopreserved JNA-tumors (eleven primary tumors and two recurrent ones) after immunohistochemical staining. We used monoclonal antibodies specific for transforming growth factor beta 1 (TGF-beta(1)), basic fibroblast growth factor (bFGF), the VEGF-receptors 1 and -2 (FLT-1 and FLK-1), and the hypoxia inducible factor (Hif-1alpha). Data were compared to the vessel density. Quantitative analysis of staining intensities was performed by a computer assisted quantification technique. Endothelial and stromal compartments of the samples were analysed separately. Data were compared to vessel densities and patients data. The VEGF-Receptor-2 (FLK) was frequently unregulated in the stroma and endothelium of those samples with high vessel densities. Similarly, we observed high bFGF- and TGF-beta(1) levels in the stroma of strong vascularised samples. No correlations of expression levels to patients' data were found. The reported data support the concept of JNA-growth and -vascularisation driven by factors released from stromal fibroblasts. Therefore, inhibition of these factors might be beneficial for the therapy of inoperable JNA.

Juvenile nasopharyngeal angiofibroma: a revised staging system. Rhinology.2006 Mar;44(1):39-45.

OBJECTIVES: To discuss the shortcomings of current staging systems and to suggest modifications according to new surgical methods and data. STUDY DESIGN: A retrospective chart review. METHODS: The medical records of 36 patients, all of whom underwent resection of juvenile nasopharyngeal angiofibroma by external or endonasal approach between 1983 and 2002, were reviewed retrospectively. Follow-up period of patients ranged from 3 to 7 years (mean, 4.5 years). Tumour extent, sites and rate of persistent disease were analyzed and compared with the literature. RESULTS: Persistent or recurrent disease was found in 12 of the 36 patients (33%). The primary tumour of these 12 cases invaded one or more anatomic region beside nasopharynx: the base of the pterygoid process in 9 cases (75%), the infratemporal fossa in 4 (33%), the pterygomaxillar fossa in 4 (33%), and the sphenoid sinus in 2 cases (17%). Involvement of the pterygoid process base was observed in only 3 of the 24 patients without persistent disease, whereas it was found 10 out of 12 patients with persistent disease. CONCLUSIONS: Advances in radiographic imaging, embolization, and surgical methods of treating angiofibromas have changed the sites associated with a high risk for persistent disease or morbidity. These changes have made it necessary for the authors to devise more appropriate classifications and, subsequently, several new staging systems were gradually introduced. Recent technological advances, particularly angled endoscopes, have resulted in improved exposure. In the light of all these recent advances, data from our series, and the literature, we suggested a new classification for determining the risk of persistent disease, choosing the appropriate surgical method, and for maintaining uniformity.

Juvenile nasopharyngeal angiofibroma: stage and surgical approach.Nippon Jibiinkoka Gakkai Kaiho. 2005 May;108(5):513-21.

Juvenile nasopharyngeal angiofibromas (JNAs) are benign tumors that occasionally invade the pterygopalatine fossa, infratemporal fossa, or middle cranial fossa. Several surgical approaches have been used based on the location of the tumor, including transpalatal, transmaxillary, and lateral rhinotomy, midfacial degloving, and Le Fort type I osteotomy. We reviewed 4 cases of JNA that had been treated by resection in the Department of Otolaryngology of Kagoshima City Hospital and 31 cases of JNA reported in the Japanese literature between 1990 and 2003. We analyzed the outcome of the surgical treatment of JNA in these cases to identify the surgical approaches that were most effective in removing tumors at several different stages. Radkowski staging showed that 17 (48.6%), 2 (5.7%), 4 (11.4%), 9 (25.7%) and 3 (8.6%) patients had stage IA, IB, IIA, IIC, and IIIA tumors, respectively. A transpalatal approach was employed in 11 cases (31.4%), a transmaxillary approach in 9 cases (25.7%), a transnasal approach in 6 cases (17.1%), and other approaches, (25.7%). The recurrence rate was, 5% in the stage I cases, 38% in the stage II cases, and 33% in the stage III cases. The transpalatal approach was followed by a high recurrence rate in the cases of stage II and higher stage. Several factors are critical when choosing the surgical approach to JNA: adequate exposure of the tumor, ability to control bleeding, prevention of postoperative facial deformity, and avoidance of interference with growth of the face.

Juvenile angiofibroma. Ugeskr Laeger. 2005 Aug 22;167(34):3163-6.

Juvenile angiofibroma is a rare, benign, rich vascular tumor, and approximately one new case is diagnosed in Denmark each year. It sits in the foramen sphenopalatinum and occurs in boys from 14 to 25 years of age. The most frequent initial symptoms are nasal obstruction and epistaxis. Through the years, the treatment of juvenile angiofibroma has included many methods, including surgical excision, electrocoagulation, interstitial or external radiation therapy, cryosurgery, hormone administration and chemotherapy. Radiation, chemotherapy and surgery have proven to be the most effective treatments. The most serious complication has been preoperative bleeding, but since the introduction of preoperative particle embolization the blood loss has been greatly reduced. Today, surgery preceded by embolization is the primary standard treatment. It is important to diagnose the tumor early, when radical surgery is easier and the frequency of recurrence is lower.

Juvenile nasopharyngeal angiofibroma.An Otorrinolaringol Ibero Am. 2005;32(4):361-71.

Juvenile Juvenile nasopharyngeal angiofibroma (JNA) is a benign and highly vascular tumor. In this article we present a case treated in our institution. We present form of presentation, exploration and intra-arterial pre-operative embolization by neuroradiology, and the treatment with the Le Fort technique. JNA has a complex anatomy, high recurrence and special histopathology, therefore the surgery is very complex and as an with high morbidity. The pre-operative embolization has decrease the hemorragic complications. We review the literature the of the JNA nowadays.

Vessel density, proliferation, and immunolocalization of vascular endothelial growth factor in juvenile nasopharyngeal angiofibromas.Arch Otolaryngol Head Neck Surg. 2004 Jun;130(6):727-31.

BACKGROUND: Juvenile nasopharyngeal angiofibroma (JNA) is a rare, highly vascularized neoplasm of the nasopharynx that affects boys and young men. The underlying dysregulated molecular mechanisms remain unclear. The participation of angiogenic growth factors has been suggested, but few studies have been published. OBJECTIVES: To evaluate the expression and localization of vascular endothelial growth factor (VEGF), proliferating cells, and vessel density in JNA. STUDY DESIGN: Immunohistochemical examination of 10 consecutive JNAs (8 primary tumors and 2 recurrent tumors). METHODS: Paraffin-embedded and cryopreserved JNA samples were included. VEGF-, CD31-, and Ki67-specific antibodies were applied and visualized using light microscopy. Vascularization was determined by counting CD31-positive vessels. Proliferating and VEGF-expressing vessels as well as stromal cells were quantified by the same method. Patients' age at the time of surgery and tumor stage were correlated with the immunohistochemical data. RESULTS: All tumors were heavily vascularized, but major differences were noted between the samples. About half of the vessels were proliferating (Ki67 positive) and half of the Ki67-positive cells were also VEGF positive. The tumor stroma was VEGF positive in 8 of 10 samples and proliferating in 5 of these 8. The 5 samples with both VEGF- and Ki67-positive stroma showed high vessel densities. No correlation was observed between age or tumor stage and vessel density, VEGF expression, or Ki67 expression. CONCLUSIONS: In JNA, VEGF is frequently expressed by stromal cells and vessels and is associated with proliferation and increased vessel density. We suggest the promotion of vascularization by VEGF, but the involvement of androgens in JNA angiogenesis still needs to be analyzed.

Juvenile nasopharyngeal angiofibroma presenting as Foster Kennedy Syndrome. Ethiop Med J.2001 Jul;39(3):251-60.

Juvenile nasopharyngral angiofibroma (JNA) is a rare benign tumor of the nasopharynx that occurs in adolescent boys with epistaxis and nasal obstruction. It may grow into the cranium causing elevated intra-cranial pressure and compression on the optic nerve. A histological-proven case of JNA in an 18 year-old Ethiopian boys is presented. He became blind due to optic atrophy in the right eye, but salvaged a useful vision in the left eye radiotherapy. The possibility of Foster-Kennedy Syndrome, a presentation of one atrophic and one papilloedematous optic nerve head of bilateral asymmetric optic atrophy, is discussed. Controversies about its histological appearance, natural history, diagnostic methods and management modalities are reviewed. JNA should be considered in adolescent boys who present with optic atrophy and/or nasal mass. Early detection and initial surgical treatment with adjunct radiotherapy could have prevented visual loss on this boy.

Nasopharyngeal angiofibroma: true neoplasm or vascular malformation?
Adv Anat Pathol. 2000 Jan;7(1):36-46.

Nasopharyngeal angiofibromas (NA) are rare tumor-like lesions characterized by architecturally irregular vessels set in a fibrous stroma. The unique morphology, the strong predilection for male adolescents, and the uncertainty about its etiology contributes to significant confusion regarding the classification of NA, which still has not been solved today. Based on immunohistochemical and electron microscopic examinations, we demonstrate in detail the various unusual vascular architectural features of NA. They represent discontinuous vascular basal laminae, focal lack of pericytes, and pronounced irregularity of the smooth muscle layers. In thick smooth muscle layers and pads, the orientation of muscle cells is frequently disturbed, and the individual cells differ in size and shape. Occasionally, the muscle layers disperse peripherally into individual cells, creating the impression of vessel-independent smooth muscle cells within the stroma. The summation of all morphological irregularities demonstrated in this paper allows the conclusion that NA represent vascular malformations.

Expression of CD34-antigen in nasopharyngeal angiofibromas.Int J Pediatr Otorhinolaryngol. 1998 Aug 1;44(3):245-50.

Formalin-fixed, paraffin-embedded and frozen tissues of 24 patients with primary nasopharyngeal angiofibroma, of whom seven had recurrences, were studied immunohistochemically for the expression of CD34 antigen using two different antibodies (HPCA-1 and QBEND 10). In all cases, there was an exclusive staining of endothelial cells, while pericytes, smooth muscle cells and stromal fibroblasts were not reactive. The staining intensity, however, was more pronounced in small tumor vessels of capillary- and sinusoidal-type than in larger vessels, which were usually characterized by an irregular smooth muscle coat. This differential staining indicates an increased proliferative potential of the endothelium of the small vessel component of nasopharyngeal angiofibroma (neoangiogenesis) and an inhibitory influence of vascular smooth muscle cells on endothelial cell growth. Moreover, the positive immunoreaction of all endothelial cells for CD34 is indicative of the absence of lymphatic vessels, which confirms previous ultrastructural observations. No differences in the staining pattern were observed between primary versus recurrent tumors, formalin fixed, paraffin embedded versus snap-frozen acetone fixed material, or between both CD34 antibodies. Our findings indicate that nasopharyngeal angiofibroma is a vasoproliferative malformation.

Juvenile nasopharyngeal angiofibroma: diagnosis and treatment.Otolaryngol Head Neck Surg. 1987 Dec;97(6):534-40.

Juvenile nasopharyngeal angiofibroma (JNA) is an uncommon, histologically benign vascular tumor that occurs almost exclusively in adolescent males. While occasional mention is made of the occurrence of JNA in females and in older males, only four cases have withstood rigorous clinicopathological review. This neoplasm accounts for less than 0.05% of all benign lesions that originate in the nasopharynx. Its clinically malignant behavior is a result of its propensity for locally destructive growth and fatal hemorrhage. The evolution in the management of these tumors has been the subject of much interest and much confusion. The advents of computed tomographic (CT) scanning, selective angiography with embolization, and refinements in surgical approaches have revolutionized operative management of these lesions by allowing more realistic selection of surgical candidates, better preoperative planning, and more flexible (yet aggressive) primary surgical treatment. The attendant reduction in morbidity and mortality of contemporary surgical management of this disease has largely obviated the argument of those who advocate use of radiotherapy as the primary treatment modality for this benign lesion. This latter view is based on the reports of massive hemorrhage, significant incidence of incomplete removal, and mortality reported in the older surgical literature. This article details our experience with a series of 31 patients who had clinicopathological diagnoses of JNA and were treated between 1954 and 1984. It furthermore represents an update of our previously reported series.

Juvenile nasopharyngeal angiofibroma. A 30 year clinical review.Am J Surg. 1983 Oct;146(4):521-5.

Thirty-one patients with juvenile nasopharyngeal angiofibroma treated at Memorial Sloan-Kettering Cancer Center from 1949 to 1979 were reviewed. Eighteen of the patients were previously untreated and in the other 13, previous treatment elsewhere had failed. Median follow-up was 54 months. All the patients were male adolescents whose presentations were characterized by epistaxis (73 percent) and nasal obstruction (60 percent). The tumors invariably arose within the nasal cavity or nasopharynx and involved neighboring structures in 58 percent of the patients. Treatment included surgery (30 patients), radiotherapy (13 patients), the administration of androgens (11 patients), sclerotherapy (2 patients), and cryotherapy (1 patient). Of the 18 primary patients, 14 were managed surgically with irradication of disease in 12 (86 percent). Of the four primary patients initially treated nonsurgically, disease recurred in three, all of whom were rendered free of disease by surgical excision. Of the 13 secondary patients, 8 were free of disease after surgery only, and 2 were free of disease after multimodal therapy with surgery being the last treatment employed. There were no deaths. Maxillary radionecrosis (one patient) and facial cellulitis (three patients) constituted the only significant morbidity. This study has demonstrated the clinical characteristics of juvenile nasopharyngeal angiofibroma and supports the primary role of surgical excision in its management.
 

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