Juvenile Nasopharyngeal Angiofibroma

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 Dacrocystitis 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

Radkowski Nasopharyngeal Angiofibroma Staging System

Radkowski et al. Arch. Otolaryngology, 1996.

Treatment Options

Surgery

Gold standard
Reserved for unresectable, life-threatening tumors Recurrent tumors with previous surgery and radiation

Radiation therapy

Chemotherapy

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 Stage I patients reduced from 840cc to 275cc blood loss Brain and ophthalmic artery embolization Facial nerve palsy Skin and soft tissue necrosis

Efficacy

Complications

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

Lateral rhinotomy Midfacial degloving

Larger tumors (IIIA, IIIB)

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

Endoscopic
225 ml 1

Open
1250 ml 30

Length of Stay
Recurrence Rate

2 days
0%

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.

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

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.