Juvenile Nasopharyngeal Angiofibroma A Case Report
JUVENILE NASOPHARYNGEAL ANGIOFIBROMA A CASE REPORT
Ashri Yudhistira, Farhat, Rizalina A Asnir, Syamsul
Otorhinolaryngology Head and Neck Department Medical Faculty, University of Sumatera Utara
INTRODUCTION
Juvenile nasopharyngeal angiofibroma is a rare hypervascular, locally aggressive benign tumour which is exclusively found in the nose and paranasal sinuses of male adolescents. 1
Juvenile Nasopharyngeal Angiofibroma usually originates from the region of the sphenopalatine foramen just posterior to the middle turbinates.2
Unilateral or bilateral nasal obstruction, recurrent epistaxis, proptosis and facial asymmetry are its common signs and symptoms. Typically in boys or young men complaining from recent nasal obstruction and recurrent epistaxis, this diagnosis should be considered. On nasal endoscopy a soft vascular and submucosal mass in seen beyond the middle turbinate.3
The imaging characteristic of Juvenile Nasopharyngeal Angiofibroma are typical and diagnostic, and recourse to a biopsy or angiography for confirming the diagnosis is not required in current times. Computed Tomography and MRI offer complimentary information.4
Surgery is considered to be the gold standard Juvenile Nasopharyngeal Angiofibroma treatment. Other treatement options include radiation therapy (external beam), chemotherapy and hormone therapy antiandrogen (flutamide).5
We report a case of nasopharyngeal angiofibroma in a boy aged 17 years following surgery in degloving approach.
Case Reports ( MR: 56 74 12 ) YS, 17 years old boy, came to Adam Malik General Hospital Medan on July 16, 2013
with a main complaint of nasal obstruction. Right nasal obstruction since about 8 months ago and he also complained masif nosebleeds and stopped after the treatment to the hospital the next 1 months back.
1
1. 2. Figure 1 & 2.
Anterior rhinoscopy showed tumor with smooth surface and hypervascularitation in dextra nasal cavity and posterior rhinoscopy showed tumor in nasopharynx.
Patient had hormonal therapy (microgynon) for 1 month. There was bleeding where the bleeding can not be controlled,and had therapy propanolol within 3 month.
The patient had angiografi on July 31,2013, and the result: mass hipervaskuler at nasopharynx area is very possibly a angiofibroma.
The first CT-Scan (18 th July 2013) that taken before surgery showed a mass soft tissue in blood density nasopharynx fills all size nasopharynx cavity of 4-5 cm. Surface of lobulated. Mass extends to choana and nasal cavity especially right side and sinus sphenoidalis.
Figure 3 & 4. CT Scan Sinus Paranasal We repeat the CT Scan evaluation on the November 12th 2013 after had therapy propanolol within 4 months. CT Scan showing a soft tissue mass wide of assertive boundary with necrotic solid component is very post nasopharynx projection contrast with picture of obliteration of torus tubarius and fossa bilateral russen. Superior the mass seen extend to sphenoidalis sinus with suspicion of destruksi base os sphenoidalis. Anterior the mass seen extend to dextra nasal cavity.
2
Figure 4. CT Scan Nasopharynx Laboratory tests Hb : 12,20 g%, protrombin time 12,50 second, APTT 27,1 second. We planned for surgery under general anasthesia on November 26th 2013. Thorax x-ray and ECG was normal. We diagnose the patient is Juvenile Nasopharyngeal Angiofibroma and we performed surgical technique used for removal of the mass with Degloving approach in general anasthesia to removed the mass. Desinfection and pehacain infiltration to the incission site, the incission made in the sublabial, bilaterally start from maxilla tuberosity from the right to the left to the periosteum. The soft tissue then detached, the septum cartilage was cut from the nasal spina up to the nasofrontal suture.
5. 6.
3
7. 8.
9. 10. Figure 5-10. Procedure of degloving to remove the mass from nasopharynx Evaluate nasopharynx, with finger and respactorium mass was detached and extracted
from its surrounding tissue. Nasopharynx was evaluated endoscopically to find any residual tumor. We used posterior tamponade (belloque) and followed by anterior tamponade to prevent bleeding.
Figure 11. Mass of the nasopharynx The mass consisted of several lobules with approximate size of the lobules were about 11x8x6 cm. Total blood loss during operation was 700 ml.
4
Post operatively, patient was given antibiotic, analgetic, antifibrinolytic and steroid. On follow-up the overall result was satisfying and patient was discharged on the 4th day after operation. The histopathology examination showed nasopharingeal angiofibroma.
Discussion Juvenile nasopharyngeal angiofibroma is uncommon tumor originating primarily in
the nasopharynx with extension to surrounding structures such as nasal cavity, sphenoid sinus, sella, pterygomaxillary fossa, infratemporal space, inferior orbital fissure, and intracranial region.6 In this case report the tumor extend to dextra nasal cavity and sphenoidalis sinus with suspicion of destruksi base os sphenoidalis.
Extranasopharyngealangiofibroma are vascular fibrous nodules occuring outside the naspharynx and are rare, benign neoplasms characterized by a different biological history and clinical features with respect to nasopharyngeal tumours, and for these reasons, should be regarded as a separate clinical entity.7
Surgical excision is the treatment of choice. Various treatment modalities like surgery, radiotherapy, embolization, chemotherapy and hormonal therapy are used and each has its own limitations.8 A hormonal influence in sinonasal angiofibromas has long been suggested by the manifestation of this tumor in adolescent males.9 In this case report, patient had treatment hormonal therapy, propanolol therapy, angiografi and surgey with degloving approached and successfully remove the mass completely.
.Various systems of classification exist for angiofibroma. The Radkowski’s classification is currently popular and increasing stages have been correlated with incremental rise in tumor reccurences. In this case report by this staging this patient was as stage II A.
Table 1. Radkowski staging system for juvenile nasopharyngeal angiofibroma.9
5
References
Sinha NK. (2011). Juvenile Nasopharyngeal Angiofibroma Excision Through Lateral Rhinotomy and Sublabial Approach. J Dhaka Med Coll. 2011; 20(1) : 78-81 Tyagi,I. (2007). Recurrent and residual juvenille angiofibromas. Sarjay Gandhi Post Graduate Institute of Medical Sciences :pp. 460-461 Ardehali, MM. (2011). Juvenile Nasopharyngeal Angiofibroma, New Aspects in Management. Iranian Journal of Otorhinolaryngology No.3, Vol.23, Serial No.64, Summer2011 Thakar, A. (2013). Nasopharyngeal Angiofibroma. Department of Otolaryngology and Oral and Head-Neck Surgery,All India Institute of Medical Sciences 2013;6 (1) :pp :25-34 Singh, AC. (2013). Anaesthetic management of endoscopic resection of juvenile nasopharyngeal angiofibroma: our experience and a review of the literature. Department of Otolaryngology and Oral and Head-Neck Surgery, All India Institute of Medical Sciences 2013;19 (6) : pp 314-316 Park CK et all. (2006). Reccurent Juvenile Nasopharyngeal angiofibroma treated with Gamma Knife Surgery. J Korean Med Sci 2006; 21: 773-7 Karthikeyan P. (2014). Extra Nasopharyngeal Angiofibroma of the Sphenoid Sinus: A rare Case Report. Int J Cur Res Rev, June 2014/Vol 06 (11) p: 8 Junuki, MG. (2007). Nasopharyngeal angiofibroma treated with radiotherapy. Department of ENT and Head-Neck Surgery, India vol.2; pp 100-101 Montag, AG (2006). Steroid Hormone Receptor Expression in Nasopharyngeal Angiofibromas. Am J Clin Pathol 2006;125:832-837 Quin, MS. (2012). Juvenile nasopharyngeal angiofibroma : Evaluation and Treatement. Department of Otolaryngology the University of Texas Medical branch
6
Ashri Yudhistira, Farhat, Rizalina A Asnir, Syamsul
Otorhinolaryngology Head and Neck Department Medical Faculty, University of Sumatera Utara
INTRODUCTION
Juvenile nasopharyngeal angiofibroma is a rare hypervascular, locally aggressive benign tumour which is exclusively found in the nose and paranasal sinuses of male adolescents. 1
Juvenile Nasopharyngeal Angiofibroma usually originates from the region of the sphenopalatine foramen just posterior to the middle turbinates.2
Unilateral or bilateral nasal obstruction, recurrent epistaxis, proptosis and facial asymmetry are its common signs and symptoms. Typically in boys or young men complaining from recent nasal obstruction and recurrent epistaxis, this diagnosis should be considered. On nasal endoscopy a soft vascular and submucosal mass in seen beyond the middle turbinate.3
The imaging characteristic of Juvenile Nasopharyngeal Angiofibroma are typical and diagnostic, and recourse to a biopsy or angiography for confirming the diagnosis is not required in current times. Computed Tomography and MRI offer complimentary information.4
Surgery is considered to be the gold standard Juvenile Nasopharyngeal Angiofibroma treatment. Other treatement options include radiation therapy (external beam), chemotherapy and hormone therapy antiandrogen (flutamide).5
We report a case of nasopharyngeal angiofibroma in a boy aged 17 years following surgery in degloving approach.
Case Reports ( MR: 56 74 12 ) YS, 17 years old boy, came to Adam Malik General Hospital Medan on July 16, 2013
with a main complaint of nasal obstruction. Right nasal obstruction since about 8 months ago and he also complained masif nosebleeds and stopped after the treatment to the hospital the next 1 months back.
1
1. 2. Figure 1 & 2.
Anterior rhinoscopy showed tumor with smooth surface and hypervascularitation in dextra nasal cavity and posterior rhinoscopy showed tumor in nasopharynx.
Patient had hormonal therapy (microgynon) for 1 month. There was bleeding where the bleeding can not be controlled,and had therapy propanolol within 3 month.
The patient had angiografi on July 31,2013, and the result: mass hipervaskuler at nasopharynx area is very possibly a angiofibroma.
The first CT-Scan (18 th July 2013) that taken before surgery showed a mass soft tissue in blood density nasopharynx fills all size nasopharynx cavity of 4-5 cm. Surface of lobulated. Mass extends to choana and nasal cavity especially right side and sinus sphenoidalis.
Figure 3 & 4. CT Scan Sinus Paranasal We repeat the CT Scan evaluation on the November 12th 2013 after had therapy propanolol within 4 months. CT Scan showing a soft tissue mass wide of assertive boundary with necrotic solid component is very post nasopharynx projection contrast with picture of obliteration of torus tubarius and fossa bilateral russen. Superior the mass seen extend to sphenoidalis sinus with suspicion of destruksi base os sphenoidalis. Anterior the mass seen extend to dextra nasal cavity.
2
Figure 4. CT Scan Nasopharynx Laboratory tests Hb : 12,20 g%, protrombin time 12,50 second, APTT 27,1 second. We planned for surgery under general anasthesia on November 26th 2013. Thorax x-ray and ECG was normal. We diagnose the patient is Juvenile Nasopharyngeal Angiofibroma and we performed surgical technique used for removal of the mass with Degloving approach in general anasthesia to removed the mass. Desinfection and pehacain infiltration to the incission site, the incission made in the sublabial, bilaterally start from maxilla tuberosity from the right to the left to the periosteum. The soft tissue then detached, the septum cartilage was cut from the nasal spina up to the nasofrontal suture.
5. 6.
3
7. 8.
9. 10. Figure 5-10. Procedure of degloving to remove the mass from nasopharynx Evaluate nasopharynx, with finger and respactorium mass was detached and extracted
from its surrounding tissue. Nasopharynx was evaluated endoscopically to find any residual tumor. We used posterior tamponade (belloque) and followed by anterior tamponade to prevent bleeding.
Figure 11. Mass of the nasopharynx The mass consisted of several lobules with approximate size of the lobules were about 11x8x6 cm. Total blood loss during operation was 700 ml.
4
Post operatively, patient was given antibiotic, analgetic, antifibrinolytic and steroid. On follow-up the overall result was satisfying and patient was discharged on the 4th day after operation. The histopathology examination showed nasopharingeal angiofibroma.
Discussion Juvenile nasopharyngeal angiofibroma is uncommon tumor originating primarily in
the nasopharynx with extension to surrounding structures such as nasal cavity, sphenoid sinus, sella, pterygomaxillary fossa, infratemporal space, inferior orbital fissure, and intracranial region.6 In this case report the tumor extend to dextra nasal cavity and sphenoidalis sinus with suspicion of destruksi base os sphenoidalis.
Extranasopharyngealangiofibroma are vascular fibrous nodules occuring outside the naspharynx and are rare, benign neoplasms characterized by a different biological history and clinical features with respect to nasopharyngeal tumours, and for these reasons, should be regarded as a separate clinical entity.7
Surgical excision is the treatment of choice. Various treatment modalities like surgery, radiotherapy, embolization, chemotherapy and hormonal therapy are used and each has its own limitations.8 A hormonal influence in sinonasal angiofibromas has long been suggested by the manifestation of this tumor in adolescent males.9 In this case report, patient had treatment hormonal therapy, propanolol therapy, angiografi and surgey with degloving approached and successfully remove the mass completely.
.Various systems of classification exist for angiofibroma. The Radkowski’s classification is currently popular and increasing stages have been correlated with incremental rise in tumor reccurences. In this case report by this staging this patient was as stage II A.
Table 1. Radkowski staging system for juvenile nasopharyngeal angiofibroma.9
5
References
Sinha NK. (2011). Juvenile Nasopharyngeal Angiofibroma Excision Through Lateral Rhinotomy and Sublabial Approach. J Dhaka Med Coll. 2011; 20(1) : 78-81 Tyagi,I. (2007). Recurrent and residual juvenille angiofibromas. Sarjay Gandhi Post Graduate Institute of Medical Sciences :pp. 460-461 Ardehali, MM. (2011). Juvenile Nasopharyngeal Angiofibroma, New Aspects in Management. Iranian Journal of Otorhinolaryngology No.3, Vol.23, Serial No.64, Summer2011 Thakar, A. (2013). Nasopharyngeal Angiofibroma. Department of Otolaryngology and Oral and Head-Neck Surgery,All India Institute of Medical Sciences 2013;6 (1) :pp :25-34 Singh, AC. (2013). Anaesthetic management of endoscopic resection of juvenile nasopharyngeal angiofibroma: our experience and a review of the literature. Department of Otolaryngology and Oral and Head-Neck Surgery, All India Institute of Medical Sciences 2013;19 (6) : pp 314-316 Park CK et all. (2006). Reccurent Juvenile Nasopharyngeal angiofibroma treated with Gamma Knife Surgery. J Korean Med Sci 2006; 21: 773-7 Karthikeyan P. (2014). Extra Nasopharyngeal Angiofibroma of the Sphenoid Sinus: A rare Case Report. Int J Cur Res Rev, June 2014/Vol 06 (11) p: 8 Junuki, MG. (2007). Nasopharyngeal angiofibroma treated with radiotherapy. Department of ENT and Head-Neck Surgery, India vol.2; pp 100-101 Montag, AG (2006). Steroid Hormone Receptor Expression in Nasopharyngeal Angiofibromas. Am J Clin Pathol 2006;125:832-837 Quin, MS. (2012). Juvenile nasopharyngeal angiofibroma : Evaluation and Treatement. Department of Otolaryngology the University of Texas Medical branch
6