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

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%

JNA Facts and Statistics

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 endothelium-

lined 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.

Pryor et al. Laryngoscope. 2005.

Surgical Technique

Approach (65 pts) Endoscopic Open

EBL 225 ml 1250 ml

Complications 1 30

Length of Stay 2 days 5 days

Recurrence Rate 0 % 24 %

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

1 case report used as primary treatment modality successfully

Dare et al. Neurosurgery. 2003.

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%

Tumor stage – extracranial vs. intracranial tumor

Extracranial = 5%

Intracranial = 50%

Herman F et al. Laryngoscope 1999.

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: 140-147.

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.

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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.