Angiofibroma-Juv_NP-slides-070103

Angiofibroma-Juv_NP-slides-070103 - Juvenile Nasopharyngeal...

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Unformatted text preview: Juvenile Nasopharyngeal Juvenile Nasopharyngeal Angiofibroma Garrett Hauptman MD Seckin Ulualp MD January 3, 2007 JNA JNA Overview Anatomy Diagnosis Radiology Staging Treatment Overview Overview JNA 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 10­20% Recurrence Rates as high as 50% Anatomy Anatomy Origin Origin Considered to be posterolateral nasal wall at sphenopalatine foramen Blood supply Primarily internal maxillary artery off of external carotid Origin Origin Posterolateral nasal wall near sphenopalatine foramen Routes of Spread 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 Foramen Sphenopalatine vessels Nerves Nasopalatine Posterior superior nasal Histology 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 Diagnosis Midface and Anterior Skull Base Midface and Anterior Skull Base Tumors Juvenile Nasopharyngeal Angiofibroma Osteoma Craniopharyngioma Olfactory Neuroblastoma Chordoma Chondrosarcoma Rhabdomyosarcoma Nasopharyngeal Carcinoma Diagnosis Diagnosis History Physical Exam Radiological study CT Scan MRI Angiogram Characteristic Presentation Characteristic Presentation Teenage or young adult male Recurrent epistaxis Nasal obstruction Additional Findings at Presentation Additional Findings at Presentation Conductive hearing loss Rhinolalia Hyposmia/Anosmia Swelling of cheek Dacrocystits Deformity of hard and/or soft palate Orbital proptosis Appearance Appearance Smooth lobulated mass in the nasopharynx or lateral nasal wall Pale, purplish, red­gray, or beefy red Compressible Radiology Radiology Radiological Studies 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 Holman­Miller Sign Characteristic anterior bowing of posterior maxillary wall Coronal CT: Bone Window Coronal CT: Bone Window Widening of left sphenopalatine foramen Lesion fills left choanae Extends into sphenoid sinus Axial CT: Soft Tissue Window with 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 Axial CT: Soft Tissue Window with Contrast Homogenous enhancement Widening of right sphenopalatine foramen Extension into Nasopharynx Pterygopalatine fossa Axial MRI: T1 Axial MRI: T1 Heterogeneous intermediate signal Flow voids represent enlarged vessels Extension into Nasopharynx Masticator space Coronal MRI: T1 with Contrast Coronal MRI: T1 with Contrast Diffuse intense enhancement Multiple flow voids within hypervascular mass Extension into Nasopharynx Pterygopalatine fossa Axial MRI: T2 Axial MRI: T2 Heterogeneous intermediate to high signal enhancement Multiple flow voids within hypervascular mass Extension into Nasopharynx Pterygopalatine fossa External Carotid Arteriogram External Carotid Arteriogram Feeding vessel = Internal Maxillary Artery Staging Staging Radkowski Nasopharyngeal Radkowski Nasopharyngeal Angiofibroma Staging System Radkowski et al. Arch. Otolaryngology, Treatment Treatment Treatment Options Treatment Options Surgery Radiation therapy Reserved for unresectable, life­threatening 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 Surgical Approaches Endoscopic transnasal Transpalatal Denker approach Facial translocation Medial maxillectomy Infratemporal fossa with or without craniotomy Preoperative Embolization 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 intra­operative bleeding and recurrence of juvenile nasopharyngeal angiofibromas. Clin Otolaryngol. 2002 Embolization Embolization Embolization Embolization Surgical Approaches Surgical Approaches Endoscopic transnasal Transpalatal Denker approach Facial translocation Medial maxillectomy Infratemporal fossa with or without craniotomy Endoscopic Transnasal Endoscopic Transnasal Middle turbinectomy may be performed for improved exposure Endoscopic Transnasal Endoscopic Transnasal Middle meatus antrostomy Resection of posterior maxillary wall Endoscopic Transnasal Endoscopic Transnasal Sphenopalatine artery ligation Tumor resection from pterygopalatine fossa Surgical Approaches Surgical Approaches Endoscopic transnasal Transpalatal Denker approach Facial translocation Medial maxillectomy Infratemporal fossa with or without craniotomy Transpalatal Transpalatal Soft palate is split and retracted Transpalatal Transpalatal Hard palate resection for enhanced exposure Transpalatal Transpalatal Palatine bone and inferior aspect of pterygoid plate resected Surgical Approaches Surgical Approaches Endoscopic transnasal Transpalatal Denker approach Facial translocation Medial maxillectomy Infratemporal fossa with or without craniotomy Denker Approach Denker Approach Wide anterior antrostomy Removal of ascending process of maxilla Removal of inferior half of lateral nasal wall Surgical Approaches Surgical Approaches Endoscopic transnasal Transpalatal Denker approach Facial translocation Medial maxillectomy Infratemporal fossa with or without craniotomy Midface Degloving with Maxillary Midface Degloving with Maxillary Osteotomies Gingivobuccal incision Nasal intercartilaginous incisions with transfixion incision Midface Degloving with Maxillary Midface Degloving with Maxillary Osteotomies Soft tissue elevation Midface Degloving with Maxillary Midface Degloving with Maxillary Osteotomies Le Fort I osteotemies Surgical Approaches Surgical Approaches Endoscopic transnasal Transpalatal Denker approach Facial translocation Medial maxillectomy Infratemporal fossa with or without craniotomy Maxillectomy Maxillectomy Maxillary osteotomies Sagittal osteotomy Maxillectomy Maxillectomy Alternative Approaches to Nasal Alternative Approaches to Nasal Cavities and Paranasal Sinuses Lateral Rhinotomy Weber­Ferguson incision Weber­Ferguson with Lynch extension Weber­Ferguson with lateral subciliary extension Weber­Ferguson with subciliary extension and supraciliary extension Surgical Approaches Surgical Approaches Endoscopic transnasal Transpalatal Denker approach Facial translocation Medial maxillectomy Infratemporal fossa with or without craniotomy Infratemporal Fossa with or without Infratemporal Fossa with or without Craniotomy Choosing the Surgical Approach Choosing the Surgical Approach Retrospective chart review of surgical intervention­ 37 patients Staged using CT scan and/or MRI Follow­up CT scan or MRI: 3 months, 6 months x 3 years, yearly Recurrence rate = 27% Hosseini et al. Eur Arch Otorhinolaryngol. 2005. Surgical Planning Surgical Planning Smaller tumors (IA, IB, IIA, IIB, IIC) Trans­nasal 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 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 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. Pryor et al. Laryngoscope. 2005. Surgical Technique 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 12­56 month follow­up range Transpalatal approach Tosun et al. J Craniofac Surg. 2006. Surgical Technique 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 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. Gamma Knife Surgery 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 External Beam Radiation Retrospective review of efficacy of radiation as primary treatment modality for JNA 15 patients received 3000­3500 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 External Beam Radiation Retrospective review of efficacy of radiation as primary treatment modality for JNA 27 patients received 3000­5500 cGy Recurrence rate of 15% 2­5 years post­ treatment External beam radiation is effective mode of treatment of advanced JNA Lee JT et al. Laryngoscope. 2002. External Beam Radiation External Beam Radiation Long­term 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 Cummings et al. Laryngoscope 1984. receiving 3500cGy initially, then 3000cGy for Chemotherapy 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 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 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 Surveillance Frequent physical examinations CT Scan / MRI Recurrence Rates Recurrence Rates Post­operative 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 Conclusions Rare, benign, vascular tumor found almost exclusively in young males Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow­up after treatment is necessary Questions Questions Bibliography Bibliography Bremer JW, Neel HB III, De Santo LW, et al. Angiofibroma: Treatment trends in 150 patients during 40 years. Laryngoscope 1986; 96: 1321­1329. Cansiz H, Guvenc MG, Sekecioglu N. Surgical approaches to juvenile nasopharyngeal angiofibroma. J Craniomaxillofac Surg. 2006 Jan;34(1):3­8. Epub 2005 Dec 15. Cummings BJ, Blend R, Keane T, et al. Primary radiation therapy for juvenile nasopharyngeal angiofibroma. Laryngoscope 1984; 94: 1599­1605. Douglas R, Wormald PJ. Endoscopic surgery for juvenile nasopharyngeal angiofibroma: where are the limits? Curr Opin Otolaryngol Head Neck Surg. 2006 Feb;14(1):1­5. Enepekides DJ. Recent advances in the treatment of juvenile angiofibroma. Curr Opin Otolaryngol Head Neck Surg. 2004 Dec;12(6):495­499. 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):946­950. 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):807­812. Epub 2005 Mar 1. Labra A, Chavolla­Magana R, Lopez­Ugalde A, Alanis­Calderon J, Huerta­Delgado 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):466­469. 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):1213­1220. Liu L, Wang R, Huang D, Han D, Ferguson EJ, Shi H, Yang W. Analysis of intra­operative bleeding and recurrence of juvenile nasopharyngeal angiofibromas. Clin Otolaryngol. 2002; 27:536­540. 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):1201­1207. Radkowski D, McGill T, Healy GB, et al. Angiofibroma. Archives of Otolaryngology. Volume 122(2), February 1996, pp 122­129 Reddy KA, Mendenhall WM, Amdur RJ, Stringer SP, Cassisi NJ. Long­term results of radiation therapy for juvenile nasopharyngeal angiofibroma. Am J Otolaryngol. 2001 May­Jun;22(3):172­175. Schick B, Kahle G, Hassler R, Draf W. Chemotherapy of juvenile angiofibroma­­an alternative? HNO. 1996 Mar;44(3):148­152. 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):15­20. ...
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