Juvenile Nasopharyngeal Angiofibroma Garrett Hauptman, MD Faculty Advisor: Seckin Ulualp, MD Grand Rounds Presentation The University of Texas Medical Branch Department of Otolaryngology 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
MRI
Excellent for bone detail Lesion enhances with contrast on CT
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
Radiation therapy
Reserved for unresectable, life-threatening tumors
Chemotherapy
Gold standard
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
Efficacy
Gelfoam: resorbed in approximately 2 weeks Polyvinyl alcohol: more permanent 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)
Endoscopic
Open
225 ml
1250 ml
Complications
1
30
Length of Stay
2 days
5 days
0%
24 %
EBL
Recurrence Rate
Pryor et al. Laryngoscope. 2005.
Surgical Technique
Retrospective study of 24 patients using Radkowski staging scale 10 patients IA through IIA had transpalatal approach
9 patients IA through IIIA had transnasal endoscopic approach
Before 1999
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.
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.
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. Cansiz H, Guvenc MG, Sekecioglu N. Surgical approaches to juvenile nasopharyngeal angiofibroma. J Craniomaxillofac Surg. 2006 Jan;34(1):3-8. Epub 2005 Dec 15. Cummings BJ, Blend R, Keane T, et al. Primary radiation therapy for juvenile nasopharyngeal angiofibroma. Laryngoscope 1984; 94: 1599-1605. Douglas R, Wormald PJ. Endoscopic surgery for juvenile nasopharyngeal angiofibroma: where are the limits? Curr Opin Otolaryngol Head Neck Surg. 2006 Feb;14(1):1-5. Enepekides DJ. Recent advances in the treatment of juvenile angiofibroma. Curr Opin Otolaryngol Head Neck Surg. 2004 Dec;12(6):495-499. Hardillo JA, Vander Velden LA, Knegt PP. Denker operation is an effective surgical approach in managing juvenile nasopharyngeal angiofibroma. Ann Otol Rhinol Laryngol. 2004 Dec;113(12):946-950. Herman F, Lot G, Chapot R, et al. Long term follow up of juvenile nasopharyngeal angiofibromas: Analysis of recurrences. Laryngoscope 1999; 109: 140147. Hosseini SM, Borghei P, Borghei SH, Ashtiani MT, Shirkhoda A. Angiofibroma: an outcome review of conventional surgical approaches. Eur Arch Otorhinolaryngol. 2005 Oct;262(10):807-812. Epub 2005 Mar 1. Labra A, Chavolla-Magana R, Lopez-Ugalde A, Alanis-Calderon J, Huerta-Delgado A. Flutamide as a preoperative treatment in juvenile angiofibroma (JA) with intracranial invasion: report of 7 cases. Otolaryngol Head Neck Surg. 2004 Apr;130(4):466-469. Lee 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 Pt 1):1213-1220. Liu L, Wang R, Huang D, Han D, Ferguson EJ, Shi H, Yang W. Analysis of intra-operative bleeding and recurrence of juvenile nasopharyngeal angiofibromas. Clin Otolaryngol. 2002; 27:536-540. Mann WJ, Jecker P, Amedee RG. Juvenile angiofibromas: changing surgical concept over the last 20 years. Laryngoscope. 2004 Feb;114(2):291-293. Pryor SG, Moore EJ, Kasperbauer JL. Endoscopic versus traditional approaches for excision of juvenile nasopharyngeal angiofibroma. Laryngoscope. 2005 Jul;115(7):1201-1207. Radkowski D, McGill T, Healy GB, et al. Angiofibroma. Archives of Otolaryngology. Volume 122(2), February 1996, pp 122-129 Reddy KA, Mendenhall WM, Amdur RJ, Stringer SP, Cassisi NJ. Long-term results of radiation therapy for juvenile nasopharyngeal angiofibroma. Am J Otolaryngol. 2001 May-Jun;22(3):172-175. Schick B, Kahle G, Hassler R, Draf W. Chemotherapy of juvenile angiofibroma--an alternative? HNO. 1996 Mar;44(3):148-152. German. Tosun F, Ozer C, Gerek M, Yetiser S. Surgical approaches for nasopharyngeal angiofibroma: comparative analysis and current trends. J Craniofac Surg. 2006 Jan;17(1):15-20.
