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Juvenile Nasopharyngeal Angiofibroma

Garrett Hauptman MD Seckin Ulualp MD January 3, 2007

JNA
Overview Anatomy Diagnosis Radiology Staging Treatment

Overview

JNA
Benign highly vascular tumor Locally invasive, submucosal spread Vascular supply most commonly from internal maxillary artery
Also: internal carotid, external carotid, common carotid, ascending pharyngeal

JNA Facts and Statistics


Up to 0.5% of head and neck tumors Occurring almost exclusively in males Average age of onset = 15 years old Intracranial Extension between 10-20% Recurrence Rates as high as 50%

Anatomy

Origin
Considered to be posterolateral nasal wall at sphenopalatine foramen Blood supply
Primarily internal maxillary artery off of external carotid

Origin
Posterolateral nasal wall near sphenopalatine foramen

Routes of Spread
Medial growth
Nasal cavity Nasopharynx

Lateral growth
Pterygopalatine fossa
Vertical expansion through inferior orbital fissure to orbit possible

Infratemporal fossa
Superior expansion through pterygoid process may involve middle cranial fossa Lateral and posterior walls of sphenoid sinus can be eroded Cavernous sinus may be involved Pituitary may be involved

Sphenopalatine Foramen
Sphenopalatine vessels Nerves
Nasopalatine Posterior superior nasal

Histology
Myofibroblast is cell of origin Fibrous connective tissue with abundant endotheliumlined vascular spaces Pseudocapsule of fibrous tissue Blood vessels lack a complete muscular layer

Diagnosis

Midface and Anterior Skull Base Tumors


Juvenile Nasopharyngeal Angiofibroma Osteoma Craniopharyngioma Olfactory Neuroblastoma Chordoma Chondrosarcoma Rhabdomyosarcoma Nasopharyngeal Carcinoma

Diagnosis
History Physical Exam Radiological study
CT Scan MRI Angiogram

Characteristic Presentation
Teenage or young adult male Recurrent epistaxis Nasal obstruction

Additional Findings at Presentation


Conductive hearing loss Rhinolalia Hyposmia/Anosmia Swelling of cheek Dacrocystits Deformity of hard and/or soft palate Orbital proptosis

Appearance
Smooth lobulated mass in the nasopharynx or lateral nasal wall Pale, purplish, red-gray, or beefy red Compressible

Radiology

Radiological Studies
CT Scan
Excellent for bone detail Lesion enhances with contrast on CT

MRI
Differentiate tumor from other soft tissue structures

Angiogram
Evaluation of feeding blood vessels

Holman-Miller Sign Characteristic anterior bowing of posterior maxillary wall

Coronal CT: Bone Window


Widening of left sphenopalatine foramen Lesion fills left choanae Extends into sphenoid sinus

Axial CT: Soft Tissue Window with Contrast


Homogenous enhancement Widening of left sphenopalatine foramen Extension into
Nasopharynx Pterygopalatine fossa

Axial CT: Soft Tissue Window with Contrast


Homogenous enhancement Widening of right sphenopalatine foramen Extension into
Nasopharynx Pterygopalatine fossa

Axial MRI: T1
Heterogeneous intermediate signal Flow voids represent enlarged vessels Extension into
Nasopharynx Masticator space

Coronal MRI: T1 with Contrast


Diffuse intense enhancement Multiple flow voids within hypervascular mass Extension into
Nasopharynx Pterygopalatine fossa

Axial MRI: T2
Heterogeneous intermediate to high signal enhancement Multiple flow voids within hypervascular mass Extension into
Nasopharynx Pterygopalatine fossa

External Carotid Arteriogram

Feeding vessel = Internal Maxillary Artery

Staging

Radkowski Nasopharyngeal Angiofibroma Staging System

Radkowski et al. Arch. Otolaryngology, 1996.

Treatment

Treatment Options
Surgery
Gold standard

Radiation therapy
Reserved for unresectable, life-threatening tumors

Chemotherapy
Recurrent tumors with previous surgery and radiation

Hormone therapy
Estrogens and antiandrogens used to decrease tumor size and vascularity

Surgical Approaches
Endoscopic transnasal Transpalatal Denker approach Facial translocation Medial maxillectomy Infratemporal fossa with or without craniotomy

Preoperative Embolization
24 to 72 hours preoperatively Gelfoam or polyvinyl alcohol foam
Gelfoam: resorbed in approximately 2 weeks Polyvinyl alcohol: more permanent

Efficacy
Stage I patients reduced from 840cc to 275cc blood loss

Complications
Brain and ophthalmic artery embolization Facial nerve palsy Skin and soft tissue necrosis
Liu L et al. Analysis of intra-operative bleeding and recurrence of juvenile nasopharyngeal angiofibromas. Clin Otolaryngol. 2002

Embolization

Embolization

Surgical Approaches
Endoscopic transnasal Transpalatal Denker approach Facial translocation Medial maxillectomy Infratemporal fossa with or without craniotomy

Endoscopic Transnasal

Middle turbinectomy may be performed for improved exposure

Endoscopic Transnasal

Middle meatus antrostomy Resection of posterior maxillary wall

Endoscopic Transnasal

Sphenopalatine artery ligation Tumor resection from pterygopalatine fossa

Surgical Approaches
Endoscopic transnasal Transpalatal Denker approach Facial translocation Medial maxillectomy Infratemporal fossa with or without craniotomy

Transpalatal

Soft palate is split and retracted

Transpalatal

Hard palate resection for enhanced exposure

Transpalatal

Palatine bone and inferior aspect of pterygoid plate resected

Surgical Approaches
Endoscopic transnasal Transpalatal Denker approach Facial translocation Medial maxillectomy Infratemporal fossa with or without craniotomy

Denker Approach

Wide anterior antrostomy Removal of ascending process of maxilla Removal of inferior half of lateral nasal wall

Surgical Approaches
Endoscopic transnasal Transpalatal Denker approach Facial translocation Medial maxillectomy Infratemporal fossa with or without craniotomy

Midface Degloving with Maxillary Osteotomies

Gingivobuccal incision Nasal intercartilaginous incisions with transfixion incision

Midface Degloving with Maxillary Osteotomies

Soft tissue elevation

Midface Degloving with Maxillary Osteotomies

Le Fort I osteotemies

Surgical Approaches
Endoscopic transnasal Transpalatal Denker approach Facial translocation Medial maxillectomy Infratemporal fossa with or without craniotomy

Maxillectomy

Maxillary osteotomies Sagittal osteotomy

Maxillectomy

Alternative Approaches to Nasal Cavities and Paranasal Sinuses


Lateral Rhinotomy Weber-Ferguson incision Weber-Ferguson with Lynch extension Weber-Ferguson with lateral subciliary extension Weber-Ferguson with subciliary extension and supraciliary extension

Surgical Approaches
Endoscopic transnasal Transpalatal Denker approach Facial translocation Medial maxillectomy Infratemporal fossa with or without craniotomy

Infratemporal Fossa with or without Craniotomy

Choosing the Surgical Approach


Retrospective chart review of surgical intervention- 37 patients Staged using CT scan and/or MRI Follow-up CT scan or MRI: 3 months, 6 months x 3 years, yearly Recurrence rate = 27%

Hosseini et al. Eur Arch Otorhinolaryngol. 2005.

Surgical Planning
Smaller tumors (IA, IB, IIA, IIB, IIC)
Trans-nasal endoscopic Transpalatal Transantral: lesions extending laterally up to pterygopalatine fossa

Larger tumors (IIIA, IIIB)


Lateral rhinotomy Midfacial degloving Extensive resection with higher morbidity Limited resection with higher recurrence
Hosseini et al. Eur Arch Otorhinolaryngol, 2005.

Changing Technique
Retrospective chart review of surgical intervention- 30 patients Marked shift towards endonasal procedures while tumor stages remained the same Endonasal approach contraindicated in Stage IV and some Stage III cases
May be used in conjunction with other approach in these cases
Mann et al. Laryngoscope. 2004.

Surgical Approach

Mann et al. Laryngoscope. 2004.

Surgical Technique
Approach (65 pts)
EBL Complications Length of Stay Recurrence Rate

Endoscopic
225 ml 1 2 days 0%

Open
1250 ml 30 5 days 24 %

Pryor et al. Laryngoscope. 2005.

Surgical Technique
Retrospective study of 24 patients using Radkowski staging scale 10 patients IA through IIA had transpalatal approach
Before 1999

9 patients IA through IIIA had transnasal endoscopic approach


After 1999

5 patients IIA through IIIA had lateral rhinotomy or degloving approach Recurrence in 1 case with 12-56 month follow-up range
Transpalatal approach
Tosun et al. J Craniofac Surg. 2006. 2006

Surgical Technique
Transnasal endoscopic approach can replace transpalatal approach
Less morbidity

Patients with IIA through IIIA previously treated with lateral rhinotomy may be treated with transnasal endoscopic approach Tumors extending to infratemporal fossa require lateral rhinotomy and degloving for optimal exposure
Greater morbidity

Tosun et al. J Craniofac Surg. 2006. 2006

Surgical Technique
Surgical limitations of endoscopic resection evaluated in literature review Extremely limited IIIA and IIIB may be approached endoscopically Preoperative embolization recommended Unlikely that limits on endoscopic resection of JNA have been reached

Douglas et al. Curr Opin Otolaryngol Head Neck Surg. 2006.

Gamma Knife Surgery


2 case reports used as booster treatment for residual tumor after surgery
No change in tumor size of one patient, regression in other patient
Dare et al. Neurosurgery. 2003.

1 case report used as primary treatment modality successfully


Park et al. J Korean Med Sci. 2006.

External Beam Radiation


Retrospective review of efficacy of radiation as primary treatment modality for JNA 15 patients received 3000-3500 cGy Recurrence rate of 15% External beam radiation is effective mode of treatment of advanced JNA

Reddy et al. Am J Otolaryngol. 2001.

External Beam Radiation


Retrospective review of efficacy of radiation as primary treatment modality for JNA 27 patients received 3000-5500 cGy Recurrence rate of 15% 2-5 years post-treatment External beam radiation is effective mode of treatment of advanced JNA

Lee JT et al. Laryngoscope. 2002.

External Beam Radiation


Long-term sequelae of concern
Growth retardation, panhypopituitarism, temporal lobe necrosis, cataracts, radiation keratopathy

Retrospective review reported 2 cases out of 55 patients developing secondary malignancies


Thyroid carcinoma 13 years after receiving 3500cGy Basal cell carcinoma of skin 14 years after receiving 3500cGy initially, then 3000cGy for recurrence
Cummings et al. Laryngoscope 1984.

Chemotherapy
Chemotherapy alternative therapy 1 unresectable tumor had chemotherapy for palliation
Adriamycin and decarbazine Extensive regression of tumor Possible alternative to radiation?

Shick et al. HNO. 1996.

Hormonal Therapy
Estrogen, progesterone, and androgen receptors have been identified with varying frequencies in JNAs
Some JNAs lack these receptors

Limited utility
Delays surgery Feminizing side effects Cardiovascular complications

Hormonal Therapy
Efficacy of treatment with flutamide evaluated in 7 patients Before and after measurement comparison made using CT scan
No statistically significant difference in size No difference in blood loss

No advantage with treatment


Labra A et al. Otolaryngol Head Neck Surg. 2004.

Surveillance
Frequent physical examinations CT Scan / MRI

Recurrence Rates
Post-operative
Stage I and II = 7% Stage III = 39.5%
Herman F et al. Laryngoscope 1999.

Tumor stage extracranial vs. intracranial tumor


Extracranial = 5% Intracranial = 50%
Bremer JW et al. Laryngoscope 1986.

Conclusions
Rare, benign, vascular tumor found almost exclusively in young males Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary

Questions

Bibliography
Bremer JW, Neel HB III, De Santo LW, et al. Angiofibroma: Treatment trends in 150 patients during 40 years. Laryngoscope 1986; 96: 1321-1329. Angiofibroma: Laryngoscope 1321Cansiz H, Guvenc MG, Sekecioglu N. Surgical approaches to juvenile nasopharyngeal angiofibroma. J Craniomaxillofac Surg. 2006 Jan;34(1):3-8. Epub . . angiofibroma Surg Jan;34(1):32005 Dec 15. Cummings BJ, Blend R, Keane T, et al. Primary radiation therapy for juvenile nasopharyngeal angiofibroma. Laryngoscope 1984; 94: 1599-1605. angiofibroma. 1599Douglas R, Wormald PJ. Endoscopic surgery for juvenile nasopharyngeal angiofibroma: where are the limits? Curr Opin Otolaryngol Head Neck Surg. angiofibroma: Surg. 2006 Feb;14(1):1-5. Feb;14(1):1Enepekides DJ. Recent advances in the treatment of juvenile angiofibroma. angiofibroma. Curr Opin Otolaryngol Head Neck Surg. 2004 Dec;12(6):495-499. Surg. Dec;12(6):495Hardillo JA, Vander Velden LA, Knegt PP. Denker operation is an effective surgical approach in managing juvenile nasopharyngeal angiofibroma. Ann juvenile angiofibroma. Otol Rhinol Laryngol. 2004 Dec;113(12):946-950. Laryngol. Dec;113(12):946Herman F, Lot G, Chapot R, et al. Long term follow up of juvenile nasopharyngeal angiofibromas: Analysis of recurrences. Laryngoscope 1999; 109: 140angiofibromas: 140147. Hosseini SM, Borghei P, Borghei SH, Ashtiani MT, Shirkhoda A. Angiofibroma: an outcome review of conventional surgical approaches. Eur Arch SM, SH, MT, Angiofibroma: Otorhinolaryngol. 2005 Oct;262(10):807-812. Epub 2005 Mar 1. Otorhinolaryngol. Oct;262(10):807Labra A, Chavolla-Magana R, Lopez-Ugalde A, Alanis-Calderon J, Huerta-Delgado A. Flutamide as a preoperative treatment in juvenile angiofibroma (JA) ChavollaLopezAlanisHuertawith intracranial invasion: report of 7 cases. Otolaryngol Head Neck Surg. 2004 Apr;130(4):466-469. Surg. Apr;130(4):466Lee JT, Chen P, Safa A, Juliard G, Calcaterra TC. The role of radiation in the treatment of advanced juvenile angiofibroma. Laryngoscope. 2002 Jul;112(7 juvenile angiofibroma. Pt 1):1213-1220. 1):1213Liu L, Wang R, Huang D, Han D, Ferguson EJ, Shi H, Yang W. Analysis of intra-operative bleeding and recurrence of juvenile nasopharyngeal Analysis intraangiofibromas. Clin Otolaryngol. 2002; 27:536-540. angiofibromas. Otolaryngol. 27:536Mann WJ, Jecker P, Amedee RG. Juvenile angiofibromas: changing surgical concept over the last 20 years. Laryngoscope. 2004 Feb;114(2):291-293. angiofibromas: Laryngoscope. Feb;114(2):291Pryor SG, Moore EJ, Kasperbauer JL. Endoscopic versus traditional approaches for excision of juvenile nasopharyngeal angiofibroma. Laryngoscope. juvenile angiofibroma. 2005 Jul;115(7):1201-1207. Jul;115(7):1201Radkowski D, McGill T, Healy GB, et al. Angiofibroma. Archives of Otolaryngology. Angiofibroma. Volume 122(2), February 1996, pp 122-129 122Reddy KA, Mendenhall WM, Amdur RJ, Stringer SP, Cassisi NJ. Long-term results of radiation therapy for juvenile nasopharyngeal angiofibroma. Am J Longangiofibroma. Otolaryngol. 2001 May-Jun;22(3):172-175. Otolaryngol. May- Jun;22(3):172Schick B, Kahle G, Hassler R, Draf W. Chemotherapy of juvenile angiofibroma--an alternative? HNO. 1996 Mar;44(3):148-152. German. angiofibroma--an Mar;44(3):148Tosun F, Ozer C, Gerek M, Yetiser S. Surgical approaches for nasopharyngeal angiofibroma: comparative analysis and current trends. J Craniofac Surg. angiofibroma: Surg. 2006 Jan;17(1):15-20. Jan;17(1):15-