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Surgical management and outcome of juvenile nasopharyngeal angiofibroma in a single centre: A fifteen year experience
Egyptian Journal of Ear, Nose, Throat and Allied Sciences xxx (2016) xxx–xxx
Contents lists available at ScienceDirect
Egyptian Journal of Ear, Nose, Throat and Allied Sciences journal homepage: www.ejentas.com
Original article
Surgical management and outcome of juvenile nasopharyngeal angiofibroma in a single centre: A fifteen year experience Jeyasakthy Saniasiaya ⇑, Baharudin Abdullah, Ramiza Ramza Ramli Department of Otorhinolaryngology-Head & Neck Surgery, School of Medical Sciences, Universiti Sains Malaysia Health Campus, 16150 Kota Bharu, Kelantan, Malaysia
a r t i c l e
i n f o
Article history: Received 31 July 2016 Accepted 15 September 2016 Available online xxxx Keywords: Nasopharyngeal neoplasms Angiofibroma
a b s t r a c t Introduction: Juvenile nasopharyngeal angiofibroma (JNA) is a locally aggressive benign vascular tumour exclusively amongst adolescence males. General characteristics, management and outcomes of 11 cases of JNA presented to our centre between 2000 till 2015 were studied and evaluated. Objective: This is a retrospective study to determine general characteristics, management and outcomes amongst the local population of Kelantan, Malaysia. Methods: 11 patients from the local population of Kelantan who presented between 2000 till 2015 were evaluated respectively. Demographical data, clinical presentation, duration of symptoms, stage of disease, surgical approach and outcomes of these 11 patients were reviewed and collected from the medical record office at our centre. Results: All 11 patients were Malay male with the average age at diagnosis being 15 years (range 11–21) years. Among the local population, predominant clinical presentation includes nasal obstruction followed by spontaneous painless epistaxis. All 11 patients were subjected to embolization prior to surgery. Surgery was the first line treatment for all our patients. Our patients were mostly subjected to endoscopic approach (37%) and combined approach (36%). Recurrence were seen in 5 patients (64%). 2 patients underwent radiotherapy one of which was combined with chemotherapy due to intracranial involvement. None of our patients sustained major intra- or post-operative complications. Conclusions: Surgery combined with preoperative embolization is the main modality of treatment at our centre. Based on our observation, patients delay and refusal of surgery, ineffective embolization have led to recurrence. Timely diagnosis and management together with patients co-operation is critical for successful outcome. Ó 2016 Egyptian Society of Ear, Nose, Throat and Allied Sciences. Production and hosting by Elsevier B.V. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-ncnd/4.0/).
1. Introduction Juvenile nasopharyngeal angiofibroma (JNA) is a locally aggressive benign vascular tumour exclusively amongst adolescence males. It comprises of 0.5% of all head and neck tumours1 and is the most common benign tumour of nasopharynx. This tumour is said to originate from the superior aspect of the sphenopalatine foramen which is formed by the sphenoidal process of palatine bone and pterygoid process of the sphenoid bone. Age distribution of this entity is between 14 and 25 years2 although several case has been reported involving men over 25 years and even amongst female.3
Peer review under responsibility of Egyptian Society of Ear, Nose, Throat and Allied Sciences. ⇑ Corresponding author. E-mail address: [email protected] (J. Saniasiaya).
Common clinical presentation includes: nasal obstruction, epistaxis and nasopharyngeal mass.4 JNA originates from the superior margin of the sphenopalatine foramen and has a tendency to grow and extend along the natural foramina and fissures associated with its site of origin. Genetic studies have demonstrated a close relation between these angiomas and androgen receptor expression, suggesting that this tumour is androgen-dependent. This could explain why the prevalence is higher in males.5,6 Diagnosis is made based on a careful clinical history and a detailed physical examination to describe the signs and symptoms, the morphology and epidemiology, and the radiological findings. Advent of imaging techniques have led to major advances in the diagnosis and treatment of angiofibromas. Nasal endoscopy, computed tomography, and magnetic resonance imaging help establish the site and extension of this tumour and the relations with blood vessels and nerves, all of which make it possible to precisely stage the tumour. Biopsies
http://dx.doi.org/10.1016/j.ejenta.2016.09.003 2090-0740/Ó 2016 Egyptian Society of Ear, Nose, Throat and Allied Sciences. Production and hosting by Elsevier B.V. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Please cite this article in press as: Saniasiaya J., et al. Surgical management and outcome of juvenile nasopharyngeal angiofibroma in a single centre: A fifteen year experience. Egypt J Ear Nose Throat Allied Sci (2016), http://dx.doi.org/10.1016/j.ejenta.2016.09.003
2
J. Saniasiaya et al. / Egyptian Journal of Ear, Nose, Throat and Allied Sciences xxx (2016) xxx–xxx
are not recommended because of the vascular and hemorrhagic nature of this tumour. Arteriography is done to assess the vascular supply in larger tumours and to make it possible to embolize these vascular lesions to reduce intraoperative bleeding. Endoscopy approach is the current cornerstone of management. Albeit histologically benign, tumour is locally aggressive, high persistence and recurrence.5 Recurrence has been reported to be 20–42% although it may be higher with intracranial extension. Surgical resection is said to be the first line treatment in many centres although other modalities of treatment including radiotherapy and chemotherapy have been reported. As for the choice of surgical approach, mostly it depends on the location of JNA, the extension and surgeons experience. Types of surgical approach includes: midfacial degloving, lateral rhinotomy, transpalatine and recently endoscopic approach are widely used. 2. Methodology This is a 15 years retrospective study fully conducted in Hospital Universiti Sains Malaysia amongst 11 patients. Patient’s case notes from the 1st January 2000 till 31st December 2015 were collected from the medical record office and were meticulously reviewed and analysed. Demographical data, clinical presentation, duration of symptoms, stage of disease, surgical approach and outcomes of these 11 patients were reviewed and collected from the medical record office at our centre. The Fisch (Fisch, 1983)7 (Table 1) criteria were applied to classify the tumours based on computer tomography (CT) findings. 3. Results A total of 11 patients were managed in our centre from the 1st January 2000 till the 31st December 2015 who were referred from various private and neighbouring government hospitals. All 11 patients were Malay male with the average age upon diagnosis being 15 years (range 11–21 years). Among the local population, predominant clinical presentation includes unilateral spontaneous painless epistaxis (35%) followed by unilateral nasal obstruction (34%), nasal discharge (19%), voice changes (6%) and ocular symptoms and abnormal facial contour seen amongst 3% each. Duration of symptoms till first presentation were between 1 month to 24 months with a mean of 12 months seen amongst our patients. Nasoendoscopic examination performed for all our patients revealed vascular, lobulated mass in the nasopharynx. Provisional diagnosis for all the 11 patients were JNA hence, no biopsy was made. Contract enhanced CT was performed for all our patients and categorized based on Fisch staging. Our patients mostly resented at Stage II (46%) upon diagnosis followed by Stage IV (27%), Stage I (18%) and 9% seen in Stage III. There is no association seen between duration of symptoms and staging in our centre (p value is 0.588). All our patients were subjected to selective embolization
Table 1 Fisch staging. Stage
Description
I
Tumours limited to nasal cavity, nasopharynx with no bony destruction Tumours invading the pterygomaxillary fossa, paranasal sinuses with bony destruction Tumours invading the infratemporal fossa, orbit and parasellar region, remaining lateral to the cavernous sinus Tumours with invasion to the cavernous sinus, optic chiasmal region and pituitary fossa
II III IV
prior to surgery with a mean interval of 48–36 h with no complications documented. Surgery was the first line treatment for all 11 patients in our centre. As for the approach, our patients were mostly subjected to transnasal endoscopy (37%) followed by combined approach which includes a combination of two open approaches (36%), lateral rhinotomy seen in 18% of our patients and midfacial degloving seen in 9% of our patients. No association were noted between the staging and type of surgical approach (p value is 0.094). As for the outcome, 64% of recurrence were noted amongst our patients. No association were seen between staging of the disease and recurrence (p value is 0.662) and type of surgery and recurrence (p value is 0.821). Among our patients, one patient underwent radiotherapy and one patient underwent combined radiotherapy and chemotherapy as there was intracranial involvement. Radiotherapy regime for our patients were 30–50 centigray. No major intraoperative or postoperative complications were noted amongst our patients. Minor complication seen was oroantral fistula which healed itself. One our patient were noted to have single-eye blindness as a result of tumour invasion into the optic chiasm.
4. Discussion Hippocrates in the 5th century BCE mentioned on juvenile nasopharyngeal angiofibroma in relation to nasal polyp.8 However, its association with puberty was identified by Chelius in 1847 and only in 1940 the term ‘angiofibroma’ was coined by Friedberg.2 Juvenile nasopharyngeal angiofibroma is an entity amongst adolescence male.9 In our study, all patients were male with a mean age upon presentation being 15 years which is similar to other published study.10 Our patients mostly presented within 1–24 months with a mean of 12 months upon presentation. Common presenting symptoms include spontaneous painless epistaxis, unilateral progressive nasal obstruction and nasal discharge.11 Albeit all our patients are Malay male, this data is not significant as the local community around the University is resided by Malay population.4 Radhowski mentioned that close to 70% of patients are at Stage II upon presentation.12 This is similar to our patients as mostly presented at Stage II upon diagnosis (46%). Diagnosis of this entity was made based on history, physical examination, nasoendoscopy and imaging mainly contrastenhanced computer tomography similar to most published journals.13 Computer tomography aids in staging the tumour upon diagnosis, presence and extension of tumour upon follow-up.14,15 Apart from that, computer tomography enables surgeon to decide on surgical approach for the patient. Biopsy was not done for any of our patient as biopsy to obtain histological confirmation is not practised in any centre. Most of our patients were at stage 2 (Fisch)7 upon diagnosis 46% with no association seen with the duration of presentation. Preoperative embolization is advocated prior to surgery in many centres as it is postulated that occluding the responsible artery from which the angiofibroma originates will reduce intraoperative blood loss16 and reduce the size of tumour which will facilitate resection. Naturally, an angiography is performed prior to embolization to identify the source vessel. In our centre, all our patients were subjected to preoperative embolization prior to surgery. All embolizations were performed in a single session with a pre-embolization angiogram performed to analyse and determine extent of tumour blush, feeding arteries, draining veins and extracranial to intracranial anastomosis. Preoperative embolization is done 24–72 h prior to surgical resection. In our centre, materials used for embolization includes gelfoam, polyvinyl alcohol foam and most recently, acrylic glue. Materials
Please cite this article in press as: Saniasiaya J., et al. Surgical management and outcome of juvenile nasopharyngeal angiofibroma in a single centre: A fifteen year experience. Egypt J Ear Nose Throat Allied Sci (2016), http://dx.doi.org/10.1016/j.ejenta.2016.09.003
J. Saniasiaya et al. / Egyptian Journal of Ear, Nose, Throat and Allied Sciences xxx (2016) xxx–xxx
used for embolization plays an important role in surgery as PVA foam and acrylic glue is more permanent. However, there are authors who claims that preoperative embolization leads to incomplete tumour removal as it may obscure tumour borders17 leading to residual which may further subject the patient to additional surgery. Pre-operative embolization has been reported to have complications including risk of cerebrovascular accident, blindness following ophthalmic artery embolization and also necrosis of skin and soft tissue based on the compromised vascular supply. No complications post-embolization was noted in our centre. Surgery remains the first line treatment and is widely accepted in most centres. Albeit the myriad surgical armamentarium that exists till date, there is yet to be any consensus on the best choice of surgery. Having said that, transmaxillary route is favoured by many surgeons as it is said to have better exposure, lower morbidity and no facial scar18 Similarly, all our patients were subjected to surgery. Our patients were subjected to transnasal endoscopic approach and a combined approach which comprises of two different open approach to facilitate resection. It is also said that a more extensive surgical resection has a better outcome and less recurrence. Recent advances however, have seen the rise in avantgarde approach: transnasal endoscopy. It is now considered to be a more plausible approach due to the avoidance of incisions, better outcome and shorter duration of hospital stay. As for the association between staging and type of surgery, no significant association were demonstrated amongst our patients. However, study by Bleir et al. reported a significant correlation whereby endoscopic resection was opted for Andrew stage 1, 2, 3a.19 Several authors have stated that tumours at Stage IV ought to undergo a combined intra- and extracranial approach.20 Recurrence is estimated to occur up to 46%21 within six months postoperatively22 However, tumour extension into the sphenoid sinus, pterygoid base and clivus is said to have a higher recurrence rate. In our centre, the recurrence rate id 64% and it is postulated that the possible cause among others includes patients delay in surgical decision and inadequate embolization causing extensive bleeding intraoperatively which leads to inadequate surgical resection. Staging at presentation has no association with recurrence in our centre. Yet, study by Liu et al. showed a significant association between staging and recurrence whereby higher recurrence were observed with stage III and IV.16 Radiotherapy is an alternative treatment modality which was initially considered only for unresectable tumours. Therapeutic dose range is said to be between 30 and 46 Gy.23 Study by Reddy et al. demonstrated that radiotherapy is just as effective as surgery as a primary treatment modality with a 15% recurrence rate.24 In our centre, only 2 patients were subjected to radiotherapy, both patients with recurrence, one with intracranial extension and the other refused surgery. Among notable complications following radiotherapy includes arrest of craniofacial growth, temporal lobe necrosis, cataracts, induction of future malignancies, hypopituitarism and osteoradionecrosis. None of our patients demonstrated serious complications following radiotherapy. Chemotherapy has been said to be used as an alternative or an adjunct when recurrence is seen following surgical treatment. In our centre, only one patient with recurrence underwent combined radiotherapy and chemotherapy. Another treatment modality is hormonal therapy which is said to reduce the size and vascularity of angiofibroma via antiandrogen effect for instance flutamide, but is poorly tolerated due to its physical and psychological side-effects notably feminization effect.25 Apart from that, coblation, cryotherapy, electrocoagulation, gamma-knife, harmonic scalpel, interstitial brachytherapy, KTPlaser embolization and sclerotherapy are amongst other treatment modalities which are used in other centres.
3
In conclusion, based on our observation, surgery combined with pre-operative embolization is the main modality of treatment in our centres. However, inadequate embolization, patients delay and refusal of surgery has led to a significant recurrent rate in our centre. Hence, apart from timely diagnosis and management, patient’s co-operative is prudent as to achieve a successful outcome.
References 1. Paris J, Guelfucci B, Moulin G, Zanaret M, Triglia M. Diagnosis and treatment of juvenile nasopharyngeal angiofibroma. Eur Arch Otorhinolaryngol. 2001;258:120–124. 2. Gullane PJ, Davidson J, ODwyer T, Forte V. Juvenile angiofibroma: a review of the literature and a case series report. Laryngoscope. 1992;102:928–933. 3. Patrocinio JA, Patrocinio LG, Borba BHC, Bonatti BS, Guimaraes AHB. Nasopharyngeal angiofibroma in an elderly women. Am J Otolaryngol. 2005;26:198–200. 4. Tang IP, Sashinder S, Gopala KG, Narayanan P. Juvenile nasopharyngeal angiofibroma in a tertiary centre: ten-year experience. Singapore Med J. 2009;50:261–264. 5. Schick B, Rippel C, Brunner C, Jung V, Plinkert PK, Urbschat S. Numerical sex chromosome aberrations in juvenile angiofibromas: genetic evidence for an androgen-dependant tumour? Oncol Rep. 2003;10:1251–1255. 6. Coutinho-Camillo CM, Brentani MM, Nagai MA. Genetic alterations in juvenile nasopharyngeal angiofibromas. Head Neck. 2008;30:390–400. 7. Fisch U. The infratemporal fossa approach for nasopharyngeal tumours. Laryngoscope. 1983;93:36–44. 8. Babyn PS. Case 18: juvenile nasopharyngeal angiofibroma. In: Babyn PS, ed. Teaching atlas of pediatric imaging. New York: Thieme Medical Publishers; 2005:89. 9. Zito J, Fitzpatrick P, Amedee R. Juvenile nasopharyngeal angiofibroma. J La State Med Soc. 2001;153:395–398. 10. Batsakis G. Vasomotor tumours. In: Batsakis JG, ed. Tumours of the head and neck: clinical pathological considerations. 2nd ed. Williams and Wilkins; 1979:296–300. 11. Glad H, Vainer B, Buchwald C, et al.. Juvenile nasopharyngeal angio fibromas in Denmark 1981–2003: diagnosis, incidence, and treatment. Acta Otolaryngol. 2007;127:292-29. 12. Radkowski D, McGill T, Healy GB, et al.. Angiofibroma. Arch Otolaryngol Head Neck Surg. 1996;122(2):122–129. 13. Gaillard AL, Anastacio VM, Piatto VB, Maniglia JV, Molina FD. A seven-year experience with patients with juvenile nasopharyngeal angiofibroma. Braz J Otorhinolaryngol. 2010;76(2):245–250. 14. Szymanska A, Golabek W, Siwiec H, Pietura R, Szczerbo TM. Juvenile angiofibroma: the value of CT and MRI for treatment planning and follow-up. Otolaryngol Pol. 2005;59:85–90. 15. Alves FRA, Granato L, Maia MS, Lambert E. Surgical approaches to juvenile nasopharyngeal angiofibroma – case report and literature review. Int Arch Otorhinolaryngol. 2006;10:162–166. 16. Liu L, Wang R, Huang D, et al.. Analysis of intraoperative bleeding and recurrence of nasopharyngeal angiofibromas. Clin Otolaryngol Allied Sci. 2002;27(6):536–540. 17. Andrade NA, Pinto JA, Nobrega Mde O, et al.. Exclusively endoscopic surgery for juvenile nasopharyngeal angiofibroma. Otolaryngol Head Neck Surg. 2007;137 (3):492–496. 18. Danesi G, Panizza B, Mazzoni A, Calabrese V. Anterior approaches in juvenile nasopharyngeal angiofibromas with intracranial extension. Otolaryngol Head Neck Surg. 2000;122:227–283. 19. Bleir BS, Kennedy DW, Plamer JN, et al.. Current management of juvenile nasopharyngeal angiofibroma: a tertiary centre experience 1999–2007. Am J Rhinol Allergy. 2009;23(3):328–330. 20. Sinha A, Gupta S. Nasopharyngeal angiofibroma: Staging and management – A review of the case series report. Indian J Otolaryngol Head Neck Surg. 2000;52 (4):366–369. 21. Fagan JJ, Snyderman CH, Carrau RL, Janecka IP. Nasopharyngeal angiofibromas: selecting a surgical approach. Head Neck. 1997;19(5):391–399. 22. Tyagi I, Syal R, Goyal A. Staging and surgical approaches in large juvenile nasopharyngeal angiofibroma-study of 95 cases. Int J Pediatr Otorhinolaryngol. 2006;70(9):1619–1627. 23. Chakraborty S, Ghoshal S, Patil VM, et al.. Conformal radiotherapy in the treatment of advanced juvenile nasopharyngeal angiofibroma with intracranial extension: an institutional experience. Int J Radiat Oncol Biol Phys. 2011;80 (5):1398–1404. 24. Reddy KA, Mendenhall WM, Amdur RJ, et al.. Long-term results of radiation therapy for juvenile nasopharyngeal angiofibroma. Am J Otolaryngol. 2001;22 (3):172–175. 25. Labra A, Chavolla-Magana R, Lopez-Ugalde A, et al.. Flutamide as a preoperative treatment in juvenile angiofibroma (JA) with intracranial invasion: report of 7 cases. Otolaryngol Head Neck Surg. 2004;130(4):466–469.
Please cite this article in press as: Saniasiaya J., et al. Surgical management and outcome of juvenile nasopharyngeal angiofibroma in a single centre: A fifteen year experience. Egypt J Ear Nose Throat Allied Sci (2016), http://dx.doi.org/10.1016/j.ejenta.2016.09.003
Contents lists available at ScienceDirect
Egyptian Journal of Ear, Nose, Throat and Allied Sciences journal homepage: www.ejentas.com
Original article
Surgical management and outcome of juvenile nasopharyngeal angiofibroma in a single centre: A fifteen year experience Jeyasakthy Saniasiaya ⇑, Baharudin Abdullah, Ramiza Ramza Ramli Department of Otorhinolaryngology-Head & Neck Surgery, School of Medical Sciences, Universiti Sains Malaysia Health Campus, 16150 Kota Bharu, Kelantan, Malaysia
a r t i c l e
i n f o
Article history: Received 31 July 2016 Accepted 15 September 2016 Available online xxxx Keywords: Nasopharyngeal neoplasms Angiofibroma
a b s t r a c t Introduction: Juvenile nasopharyngeal angiofibroma (JNA) is a locally aggressive benign vascular tumour exclusively amongst adolescence males. General characteristics, management and outcomes of 11 cases of JNA presented to our centre between 2000 till 2015 were studied and evaluated. Objective: This is a retrospective study to determine general characteristics, management and outcomes amongst the local population of Kelantan, Malaysia. Methods: 11 patients from the local population of Kelantan who presented between 2000 till 2015 were evaluated respectively. Demographical data, clinical presentation, duration of symptoms, stage of disease, surgical approach and outcomes of these 11 patients were reviewed and collected from the medical record office at our centre. Results: All 11 patients were Malay male with the average age at diagnosis being 15 years (range 11–21) years. Among the local population, predominant clinical presentation includes nasal obstruction followed by spontaneous painless epistaxis. All 11 patients were subjected to embolization prior to surgery. Surgery was the first line treatment for all our patients. Our patients were mostly subjected to endoscopic approach (37%) and combined approach (36%). Recurrence were seen in 5 patients (64%). 2 patients underwent radiotherapy one of which was combined with chemotherapy due to intracranial involvement. None of our patients sustained major intra- or post-operative complications. Conclusions: Surgery combined with preoperative embolization is the main modality of treatment at our centre. Based on our observation, patients delay and refusal of surgery, ineffective embolization have led to recurrence. Timely diagnosis and management together with patients co-operation is critical for successful outcome. Ó 2016 Egyptian Society of Ear, Nose, Throat and Allied Sciences. Production and hosting by Elsevier B.V. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-ncnd/4.0/).
1. Introduction Juvenile nasopharyngeal angiofibroma (JNA) is a locally aggressive benign vascular tumour exclusively amongst adolescence males. It comprises of 0.5% of all head and neck tumours1 and is the most common benign tumour of nasopharynx. This tumour is said to originate from the superior aspect of the sphenopalatine foramen which is formed by the sphenoidal process of palatine bone and pterygoid process of the sphenoid bone. Age distribution of this entity is between 14 and 25 years2 although several case has been reported involving men over 25 years and even amongst female.3
Peer review under responsibility of Egyptian Society of Ear, Nose, Throat and Allied Sciences. ⇑ Corresponding author. E-mail address: [email protected] (J. Saniasiaya).
Common clinical presentation includes: nasal obstruction, epistaxis and nasopharyngeal mass.4 JNA originates from the superior margin of the sphenopalatine foramen and has a tendency to grow and extend along the natural foramina and fissures associated with its site of origin. Genetic studies have demonstrated a close relation between these angiomas and androgen receptor expression, suggesting that this tumour is androgen-dependent. This could explain why the prevalence is higher in males.5,6 Diagnosis is made based on a careful clinical history and a detailed physical examination to describe the signs and symptoms, the morphology and epidemiology, and the radiological findings. Advent of imaging techniques have led to major advances in the diagnosis and treatment of angiofibromas. Nasal endoscopy, computed tomography, and magnetic resonance imaging help establish the site and extension of this tumour and the relations with blood vessels and nerves, all of which make it possible to precisely stage the tumour. Biopsies
http://dx.doi.org/10.1016/j.ejenta.2016.09.003 2090-0740/Ó 2016 Egyptian Society of Ear, Nose, Throat and Allied Sciences. Production and hosting by Elsevier B.V. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Please cite this article in press as: Saniasiaya J., et al. Surgical management and outcome of juvenile nasopharyngeal angiofibroma in a single centre: A fifteen year experience. Egypt J Ear Nose Throat Allied Sci (2016), http://dx.doi.org/10.1016/j.ejenta.2016.09.003
2
J. Saniasiaya et al. / Egyptian Journal of Ear, Nose, Throat and Allied Sciences xxx (2016) xxx–xxx
are not recommended because of the vascular and hemorrhagic nature of this tumour. Arteriography is done to assess the vascular supply in larger tumours and to make it possible to embolize these vascular lesions to reduce intraoperative bleeding. Endoscopy approach is the current cornerstone of management. Albeit histologically benign, tumour is locally aggressive, high persistence and recurrence.5 Recurrence has been reported to be 20–42% although it may be higher with intracranial extension. Surgical resection is said to be the first line treatment in many centres although other modalities of treatment including radiotherapy and chemotherapy have been reported. As for the choice of surgical approach, mostly it depends on the location of JNA, the extension and surgeons experience. Types of surgical approach includes: midfacial degloving, lateral rhinotomy, transpalatine and recently endoscopic approach are widely used. 2. Methodology This is a 15 years retrospective study fully conducted in Hospital Universiti Sains Malaysia amongst 11 patients. Patient’s case notes from the 1st January 2000 till 31st December 2015 were collected from the medical record office and were meticulously reviewed and analysed. Demographical data, clinical presentation, duration of symptoms, stage of disease, surgical approach and outcomes of these 11 patients were reviewed and collected from the medical record office at our centre. The Fisch (Fisch, 1983)7 (Table 1) criteria were applied to classify the tumours based on computer tomography (CT) findings. 3. Results A total of 11 patients were managed in our centre from the 1st January 2000 till the 31st December 2015 who were referred from various private and neighbouring government hospitals. All 11 patients were Malay male with the average age upon diagnosis being 15 years (range 11–21 years). Among the local population, predominant clinical presentation includes unilateral spontaneous painless epistaxis (35%) followed by unilateral nasal obstruction (34%), nasal discharge (19%), voice changes (6%) and ocular symptoms and abnormal facial contour seen amongst 3% each. Duration of symptoms till first presentation were between 1 month to 24 months with a mean of 12 months seen amongst our patients. Nasoendoscopic examination performed for all our patients revealed vascular, lobulated mass in the nasopharynx. Provisional diagnosis for all the 11 patients were JNA hence, no biopsy was made. Contract enhanced CT was performed for all our patients and categorized based on Fisch staging. Our patients mostly resented at Stage II (46%) upon diagnosis followed by Stage IV (27%), Stage I (18%) and 9% seen in Stage III. There is no association seen between duration of symptoms and staging in our centre (p value is 0.588). All our patients were subjected to selective embolization
Table 1 Fisch staging. Stage
Description
I
Tumours limited to nasal cavity, nasopharynx with no bony destruction Tumours invading the pterygomaxillary fossa, paranasal sinuses with bony destruction Tumours invading the infratemporal fossa, orbit and parasellar region, remaining lateral to the cavernous sinus Tumours with invasion to the cavernous sinus, optic chiasmal region and pituitary fossa
II III IV
prior to surgery with a mean interval of 48–36 h with no complications documented. Surgery was the first line treatment for all 11 patients in our centre. As for the approach, our patients were mostly subjected to transnasal endoscopy (37%) followed by combined approach which includes a combination of two open approaches (36%), lateral rhinotomy seen in 18% of our patients and midfacial degloving seen in 9% of our patients. No association were noted between the staging and type of surgical approach (p value is 0.094). As for the outcome, 64% of recurrence were noted amongst our patients. No association were seen between staging of the disease and recurrence (p value is 0.662) and type of surgery and recurrence (p value is 0.821). Among our patients, one patient underwent radiotherapy and one patient underwent combined radiotherapy and chemotherapy as there was intracranial involvement. Radiotherapy regime for our patients were 30–50 centigray. No major intraoperative or postoperative complications were noted amongst our patients. Minor complication seen was oroantral fistula which healed itself. One our patient were noted to have single-eye blindness as a result of tumour invasion into the optic chiasm.
4. Discussion Hippocrates in the 5th century BCE mentioned on juvenile nasopharyngeal angiofibroma in relation to nasal polyp.8 However, its association with puberty was identified by Chelius in 1847 and only in 1940 the term ‘angiofibroma’ was coined by Friedberg.2 Juvenile nasopharyngeal angiofibroma is an entity amongst adolescence male.9 In our study, all patients were male with a mean age upon presentation being 15 years which is similar to other published study.10 Our patients mostly presented within 1–24 months with a mean of 12 months upon presentation. Common presenting symptoms include spontaneous painless epistaxis, unilateral progressive nasal obstruction and nasal discharge.11 Albeit all our patients are Malay male, this data is not significant as the local community around the University is resided by Malay population.4 Radhowski mentioned that close to 70% of patients are at Stage II upon presentation.12 This is similar to our patients as mostly presented at Stage II upon diagnosis (46%). Diagnosis of this entity was made based on history, physical examination, nasoendoscopy and imaging mainly contrastenhanced computer tomography similar to most published journals.13 Computer tomography aids in staging the tumour upon diagnosis, presence and extension of tumour upon follow-up.14,15 Apart from that, computer tomography enables surgeon to decide on surgical approach for the patient. Biopsy was not done for any of our patient as biopsy to obtain histological confirmation is not practised in any centre. Most of our patients were at stage 2 (Fisch)7 upon diagnosis 46% with no association seen with the duration of presentation. Preoperative embolization is advocated prior to surgery in many centres as it is postulated that occluding the responsible artery from which the angiofibroma originates will reduce intraoperative blood loss16 and reduce the size of tumour which will facilitate resection. Naturally, an angiography is performed prior to embolization to identify the source vessel. In our centre, all our patients were subjected to preoperative embolization prior to surgery. All embolizations were performed in a single session with a pre-embolization angiogram performed to analyse and determine extent of tumour blush, feeding arteries, draining veins and extracranial to intracranial anastomosis. Preoperative embolization is done 24–72 h prior to surgical resection. In our centre, materials used for embolization includes gelfoam, polyvinyl alcohol foam and most recently, acrylic glue. Materials
Please cite this article in press as: Saniasiaya J., et al. Surgical management and outcome of juvenile nasopharyngeal angiofibroma in a single centre: A fifteen year experience. Egypt J Ear Nose Throat Allied Sci (2016), http://dx.doi.org/10.1016/j.ejenta.2016.09.003
J. Saniasiaya et al. / Egyptian Journal of Ear, Nose, Throat and Allied Sciences xxx (2016) xxx–xxx
used for embolization plays an important role in surgery as PVA foam and acrylic glue is more permanent. However, there are authors who claims that preoperative embolization leads to incomplete tumour removal as it may obscure tumour borders17 leading to residual which may further subject the patient to additional surgery. Pre-operative embolization has been reported to have complications including risk of cerebrovascular accident, blindness following ophthalmic artery embolization and also necrosis of skin and soft tissue based on the compromised vascular supply. No complications post-embolization was noted in our centre. Surgery remains the first line treatment and is widely accepted in most centres. Albeit the myriad surgical armamentarium that exists till date, there is yet to be any consensus on the best choice of surgery. Having said that, transmaxillary route is favoured by many surgeons as it is said to have better exposure, lower morbidity and no facial scar18 Similarly, all our patients were subjected to surgery. Our patients were subjected to transnasal endoscopic approach and a combined approach which comprises of two different open approach to facilitate resection. It is also said that a more extensive surgical resection has a better outcome and less recurrence. Recent advances however, have seen the rise in avantgarde approach: transnasal endoscopy. It is now considered to be a more plausible approach due to the avoidance of incisions, better outcome and shorter duration of hospital stay. As for the association between staging and type of surgery, no significant association were demonstrated amongst our patients. However, study by Bleir et al. reported a significant correlation whereby endoscopic resection was opted for Andrew stage 1, 2, 3a.19 Several authors have stated that tumours at Stage IV ought to undergo a combined intra- and extracranial approach.20 Recurrence is estimated to occur up to 46%21 within six months postoperatively22 However, tumour extension into the sphenoid sinus, pterygoid base and clivus is said to have a higher recurrence rate. In our centre, the recurrence rate id 64% and it is postulated that the possible cause among others includes patients delay in surgical decision and inadequate embolization causing extensive bleeding intraoperatively which leads to inadequate surgical resection. Staging at presentation has no association with recurrence in our centre. Yet, study by Liu et al. showed a significant association between staging and recurrence whereby higher recurrence were observed with stage III and IV.16 Radiotherapy is an alternative treatment modality which was initially considered only for unresectable tumours. Therapeutic dose range is said to be between 30 and 46 Gy.23 Study by Reddy et al. demonstrated that radiotherapy is just as effective as surgery as a primary treatment modality with a 15% recurrence rate.24 In our centre, only 2 patients were subjected to radiotherapy, both patients with recurrence, one with intracranial extension and the other refused surgery. Among notable complications following radiotherapy includes arrest of craniofacial growth, temporal lobe necrosis, cataracts, induction of future malignancies, hypopituitarism and osteoradionecrosis. None of our patients demonstrated serious complications following radiotherapy. Chemotherapy has been said to be used as an alternative or an adjunct when recurrence is seen following surgical treatment. In our centre, only one patient with recurrence underwent combined radiotherapy and chemotherapy. Another treatment modality is hormonal therapy which is said to reduce the size and vascularity of angiofibroma via antiandrogen effect for instance flutamide, but is poorly tolerated due to its physical and psychological side-effects notably feminization effect.25 Apart from that, coblation, cryotherapy, electrocoagulation, gamma-knife, harmonic scalpel, interstitial brachytherapy, KTPlaser embolization and sclerotherapy are amongst other treatment modalities which are used in other centres.
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In conclusion, based on our observation, surgery combined with pre-operative embolization is the main modality of treatment in our centres. However, inadequate embolization, patients delay and refusal of surgery has led to a significant recurrent rate in our centre. Hence, apart from timely diagnosis and management, patient’s co-operative is prudent as to achieve a successful outcome.
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Please cite this article in press as: Saniasiaya J., et al. Surgical management and outcome of juvenile nasopharyngeal angiofibroma in a single centre: A fifteen year experience. Egypt J Ear Nose Throat Allied Sci (2016), http://dx.doi.org/10.1016/j.ejenta.2016.09.003