juvenile nasopharyngeal angiofibroma - welcome to · pdf filejna benign highly vascular tumor...
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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.
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