Cases Of Juvenile Nasopharyngeal Angiofibroma Research Articles

An Unexpected Cause of Catastrophic Bleeding During Juvenile Nasopahryngeal Angiofibroma Excision.

We describe the first case of juvenile nasopharyngeal angiofibroma receiving blood supply from caroticotympanic artery which resulted in catastrophic bleeding intraoperatively. The patient was an 12-year-old boy with a Radkowski stage 3B JNA. We utilized preoperative embolization of feeders from external carotid artery though the surgeon failed to excise the tumor completely due to uncontrollable bleeding from the tumor. On retrospective analysis of angiography only the culprit feeder found. Tumor was excised in the second surgery conducted a week later. Although selective embolization of ICA branches are described in literature the data are sparse and so is the management of such cases.

Clinicopathological Challenges in Tumors of the Nasal Cavity and Paranasal Sinuses: Our Experience.

Background and objectiveNasal and paranasal lesions are one of the most common otorhinolaryngological presentations encountered in clinical practice. Common presenting symptoms of these lesion range from nasal blockades, facial swellings, pain, nasal discharge, and epistaxis to orbital and ear symptoms. Diagnosis can be tricky as these symptoms are common in inflammatory conditions and tumors. The aim of our study was to observe the epidemiology and clinical pathological findings in patients with nasal and paranasal masses presenting to our institute and discuss the challenges in proper diagnosis and management due to similar presentations, and the role of histopathological examination (HPE) and immunohistochemistry (IHC) in overcoming these challenges.MethodsThe IPD records of 396 patients were taken up for the study. All the specimens were sent in 10% neutral buffered formalin for examination as biopsy for diagnosis or after surgical excision. After adequate fixation, the biopsy specimen was submitted for routine processing, followed by paraffin embedding, and stained with hematoxylin and eosin (H&E). Special stains like periodic acid-Schiff (PAS) and Ziehl-Neelsen (ZN) stains for acid-fast bacilli (AFB) were used as required. IHC was performed in the required samples. IHC markers were performed on representative paraffin-embedded sections according to the streptavidin-biotin immunoperoxidase technique as needed. The findings were noted, and histology was correlated with clinical presentations and investigations, tabulated, and statistically analyzed using SPSS Statistics (IBM, Armonk, NY).ResultsOf note, 67.92%% were non-neoplastic lesions whereas 18.18% came out to be benign neoplasms and 13.88% were malignant lesions on HPE. Nasal obstruction was the most common presenting symptom (73.23% of patients) followed by nasal mass (64.14% of cases). Inflammatory sinonasal polyps were the most prominent cases in our study, accounting for 41.16% of all lesions; 18.68% fungal RS (mucormycosis) were seen in our study and 4.54% were cases of juvenile nasopharyngeal angiofibroma (JNA). The majority of benign neoplasms encountered were Schneiderian papilloma or inverted papilloma (06.81%). Eighteen (4.54%) cases of squamous cell carcinomas (SCC) were seen in our study and 2.77% (n=11) cases were of adenoid cystic carcinoma. Of 18 cases of SCC, moderately differentiated SCC carcinoma accounted for 10 cases followed by poorly differentiated SCC (5/18) and nonkeratinizing SCC (3/18). IHC for p40 was performed in all the cases of nonkeratinizing SCC, which showed strong and diffuse nuclear positivity.ConclusionThe nasal cavity is the site of the most varied presentation of tumors in the upper respiratory tract. Mass in the nose and paranasal sinus (PNS) form a heterogeneous group of lesions with varied histopathological features. The proximity of the area to the eyes and brain warrants early definitive diagnosis so that the lesion is treated before it can involve important and vital centers. Even though malignant nasal tumors have a very low incidence, they cause a lot of morbidity due to their long course and frequent local recurrences. Nasal tumors tend to become polypoidal. Epithelioid papilloma of the nasal cavity often resembles a nasal polyp. Clinical diagnosis can be challenging due to similar presentations and appearances, and hence histological examination is a vital tool for the timely diagnosis of such patients.

Juvenile nasopharyngeal angiofibroma tumor – Diagnosis and management in our tertiary hospital

Juvenile nasopharyngeal angiofibroma (JNA) is a rare benign tumor described by slow progression, vigorous growth, high vascularization, and increased recurrence rate. The aim of this article is to describe a case of JNA from our tertiary hospital and discuss the diagnosis and management in our current practice. A 16-year-old male patient presented with bleeding from the left nasal cavity. On examination, there was a non-pulsatile ovoid like mass occluding the entire left nasal cavity which was able to probe medially, superiorly, and laterally except inferiorly and on probing and it resulted in minimal bleed. Cold spatula test revealed decreased fogging on the left side. Contrast-enhanced computed tomography scan of the paranasal sinuses and nose showed the presence of a large ill-defined heterogeneously enhancing soft-tissue density mass lesion with its epicenter in posterior part of the left nasal cavity and left pterygopalatine fossa and was extending into the left orbit and left cavernous sinus. Complete embolization of the left nasopharyngeal vascular mass was done preoperatively. Two days post-embolization, surgical excision of JNA under general anesthesia was done. The approach was done through sublabial incision. This classic case of JNA was operated endoscopically because of its benefits over the conventional methods.

The role of combined computed tomographic angiography and digital subtraction angiography in the management of cervico-facial vascular lesions

BackgroundCervicofacial vascular lesions are one of the most challenging diseases managed by intervention radiologists and specialized surgeons. These lesions were diagnosed to be vascular in origin by other imaging techniques such as Duplex and/or magnetic resonance imaging (MRI). All patients underwent computed tomographic angiography (CTA) to confirm the diagnosis as well as to decide the most effective choice of management based on multidisciplinary team (MDT). Then, Digital subtraction angiography (DSA) was done as it is offering an accurate vascular map for the vascular lesions which is inevitable for successful super-selective endovascular embolization which can be done at same sitting. Small cases of arteriovenous malformations (AVMs) were sufficiently managed by only embolization, whereas cases of adequately embolized large AVMs as well as juvenile nasopharyngeal angiofibroma (JNA) cases were followed by surgery with the advantage of less blood loss and decreasing operation time as well as complications. This prospective study was done to highlight the role of combined CTA and DSA in cervicofacial vascular lesion management.ResultsTwenty patients were included in our study: 16 patients were males, and 4 were females; 17 of them were under 40 years, and three were above 40 years. Preoperative embolization was done in 12 patients, which were all the cases of JNAs and large AVMs. Four patients were managed by only embolization as a definitive treatment. Complete technical success rate was about 94%, while only 6% (single case) of the partial technical success rate which was due to a very small accessory feeder that could not be catheterized even with repeated trials.ConclusionsCombined CTA and DSA is the cornerstone for managing vascular lesions in the cervicofacial region, which necessitates a MDT to decide the most beneficial and least complicated way for management.

Nasomaxillary swellings: our experience at tertiary care hospital

<p class="abstract"><strong>Background: </strong>A wide spectrum of lesions may present as nasomaxillary swellings ranges from benign to malignant lesions including fungal sinusitis and fibro vascular lesions example: JNA (Juvenile nasopharyngeal angiofibroma) and Inverted papilloma. Many Pathologies ranging from benign to malignant tumors may mimic a simple Nasomaxillary mass. It is diagnostic challenge to determine pathology behind it. A detailed clinical evaluation with nasal endoscopy and relevant pre-operative investigations including radiological imaging and histopathological examination is essential to reach up to final diagnosis. The aim of the study was to do clinicopathological evaluation of patients presenting with nasomaxillary swelling and correlation of clinical, radiological and Histopathological findings.</p><p class="abstract"><strong>Methods: </strong>This was a prospective observational study on 50 nasomaxillary swelling patients who are presented in the Department of ENT and Head and Neck Surgery of N.S.C.B. MCH Jabalpur, a tertiary care Hospital, between March 2018 to August 2019. A detailed history taking and clinical examination with nasal endoscopy and relevant preoperative investigations including CECT Nose and PNS and histopathological examination was done in all patients.</p><p class="abstract"><strong>Results: </strong>50 Nasomaxillary swelling patients were selected for this study. 30 patients were male and 20 were females. Ages of patients were varied from 12 years to 72 years. 23 patients (46%) were malignant and 27 (54%) were benign. Histopathological examination results shows benign lesions like JNA (6 cases), fungal sinusitis (6 cases), dermoid cyst (1 case), inverted papilloma (3 cases), sebaceous cyst (1 case), jaw lesions (10 cases), (odontogenic like radicular/ infected cyst, dentigerous cyst, cystic ameloblastoma and non-odontogenic like fibrous dysplasia) and malignant lesions like SCC (squamous cell carcinoma) (12 cases), spindle cell sarcoma (2 cases), undifferentiated carcinoma (3 cases), adenocarcinoma (3 cases), adenoid cystic carcinoma (1 case), Invasive pleomorphic sarcoma (1 case) and malignant melanoma (1 case). SCC was most common lesion f/b JNA and fungal sinusitis. Well differentiated SCC was most common histological type (10 out of 12 cases of SCC). Most common symptom was nasal obstruction (66% cases) f/b epistaxis (52% cases) but epitasis was most common symptom among malignant and JNA cases. In 6 cases (3 JNA, 1 inverted papilloma, 1 malignancy and 1 radicular cyst) radiological diagnosis were not correlated with histological findings.</p><p class="abstract"><strong>Conclusions: </strong>Most our cases were malignant nasomaxillary lesions followed by fungal sinusitis and JNA. Most patients presented in advanced stage of disease so rapidly evaluation including nasal endoscopy should be done. CECT scan is essential to determine tumors extent and bony lesions. All patients should undergo hisotopatholigical examination. The final diagnosis should be made on the basis of clinical, radiological and histopathological findings.</p>

Clinical utility of Ga68 DOTANOC PET/CT imaging in juvenile nasal angiofibroma and the PARIS protocol: a preliminary report.

Juvenile nasopharyngeal angiofibroma (JNA) expresses different somatostatin cell surface receptors and Ga68 [DOTA, 1-Nal3]-octreotide (DOTANOC)-PET/computed tomography (CT) scan may be used for its imaging. Also, functional imaging with DOTANOC-PET/CT may promise of greater accuracy in the detection or exclusion of recurrent/residual JNA. In this prospective study, five JNA patients who underwent a DOTANOC-PET-CT scan both preoperatively and postoperatively during June 2018-March 2020 were included. Postcontrast enhancement of a definite lesion was considered residual/recurrent tumor in contrast-enhanced MRI (CEMRI). In DOTANOC-PET/CT, any abnormal uptake apart from physiological sites was considered as residual lesions. Radiological results were categorized as negative, suspicious or definite residual/recurrent tumors. Any discrepancy was resolved by endoscopic biopsies. Preoperatively all five cases of JNA showed avid DOTANOC expression in the tumor. The mean (SD) value of DOTANOC standardised uptake value in the tumor was 4.3 (1.4) (range = 2.1-6.2). In postoperative CEMRI evaluation, three of five patients had residual tumors and two had normal scans. On DOTANOC-PET/CT, two of five patients had residual tumors and three had normal scans. In one patient with residual tumor, the discrepancy with regard to tumor extent was noted in two scans, and further biopsy confirmed the findings of DOTANOC-PET/CT as accurate. 68Ga-DOTANOC-PET/CT uptake is universal in JNA and appears to be more specific in the identification of residual/recurrent JNA. In residual tumors, DOTANOC-PET/CT may have an advantage over CEMRI in the diagnosis, decision making and planning stereotactic radiation. However, these findings are to be validated in studies with larger patients.

Endoscopic Modified Denker's Approach for the Treatment of Juvenile Nasopharyngeal Angiofibroma.

Juvenile nasopharyngeal angiofibroma is a benign vascular lesion with propensity of bony erosion leading to skull base involvement and intracranial extension. It involves multiple compartments which are difficult to access surgically. With surgical expertise endoscopic resection of angiofibroma has become the preferred choice of surgical management. This article presents our experience with endoscopic approach for angiofibroma and establishes the modified Denker's procedure as a effective approach for removal of tumor. A prospective case study was done on 25 cases of juvenile nasopharyngeal angiofibroma treated surgically by endonasal endoscopic modified Denker's approach in the department of ENT at BJ Medical College, Ahmedabad over a period of 4years from January 2015 to January 2019. This technique was used up to Fisch stage 3b with extension up to infratemporal fossa. Using this technique no major recurrence or major morbidity was observed and did not produce any late serious complications. The endoscopic technique allows the surgeon to achieve an extensive exposure of the sinuses and control of the sphenopalatine and internal maxillary arteries without the risk of palatal dysfunction, oronasal fistula, or facial scarring. In addition, the modified Denkers approach can be an effective and less mutilating technique in managing recurrences.

Management of Juvenile Nasopharyngeal Angiofibroma in a Referral Hospital in West Java, Indonesia

Background : Juvenile nasopharyngeal angiofibroma (JNA) is a fibrovascular tumor originating from the supero-posterior area of the sphenopalatine foramen. This tumor is histologically benign, but is clinically malignant because JNA has potentially destructive spread extending to the skull base and the cranium. The aim of the study was to evaluate the management of JNA in Dr. Hasan Sadikin General Hospital Bandung, a referral hospital in West Java. Methods : This was a descriptive retrospective study using medical record data of JNA patients during the period of 2011-2018. The stage of JNA was designated, based on the FISCH classification. Results : There were112 JNA cases, consisting of 99% male and 1% female. Most of them (97%) were aged 10-20 years old (median16 years old). The chief complaints were nasal obstruction (46%) and recurrent epistaxis (36%). Most of the JNA stage was stage II (43%). Major feeding vessel of JNA was from an internal maxillary artery (IMAX) and ascending pharyngeal artery (28%). Preoperative management was equal for ligation and embolization. The complication of embolization was local pain (75%). The most surgical technique performed was the transpalatal approach (41.36%). Blood loss in a different stage in JNA surgery with embolization was less than surgery with ligation. There were patients (10%)with residual disease after 6 months follow-up. Conclusions : JNA is essentially a disease of the adolescent males. The management of JNA patients is surgery, mostly using transpalatal technique. Preoperative embolization of JNA is a safe procedure, minimizes intraoperative blood loss, decrease the incidence of residual or recurrence, and shortening the duration of the surgery.

Management of Juvenile Nasopharyngeal Angiofibroma in a Referral Hospital in West Java, Indonesia

Background : Juvenile nasopharyngeal angiofibroma (JNA) is a fibrovascular tumor originating from the supero-posterior area of the sphenopalatine foramen. This tumor is histologically benign, but is clinically malignant because JNA has potentially destructive spread extending to the skull base and the cranium. The aim of the study was to evaluate the management of JNA in Dr. Hasan Sadikin General Hospital Bandung, a referral hospital in West Java. Methods : This was a descriptive retrospective study using medical record data of JNA patients during the period of 2011-2018. The stage of JNA was designated, based on the FISCH classification. Results : There were112 JNA cases, consisting of 99% male and 1% female. Most of them (97%) were aged 10-20 years old (median16 years old). The chief complaints were nasal obstruction (46%) and recurrent epistaxis (36%). Most of the JNA stage was stage II (43%). Major feeding vessel of JNA was from an internal maxillary artery (IMAX) and ascending pharyngeal artery (28%). Preoperative management was equal for ligation and embolization. The complication of embolization was local pain (75%). The most surgical technique performed was the transpalatal approach (41.36%). Blood loss in a different stage in JNA surgery with embolization was less than surgery with ligation. There were patients (10%)with residual disease after 6 months follow-up. Conclusions : JNA is essentially a disease of the adolescent males. The management of JNA patients is surgery, mostly using transpalatal technique. Preoperative embolization of JNA is a safe procedure, minimizes intraoperative blood loss, decrease the incidence of residual or recurrence, and shortening the duration of the surgery.

Juvenile nasopharyngeal angiofibroma with intracranial extension – diagnosis and treatment.

This retrospective study analyzes radiological findings, therapeutic management and outcomes of patients with intracranial extension of JNA. The routes of intracranial spread, incidence of intracranial disease and influence on therapeutic approach are discussed. An evaluation on the records of 62 patients with JNA was performed and 10 patients with intracranial tumors were included in the study. All patients were males aged 10 to 19 years. R esults: According to Andrews' classification 8 patients presented with stage IIIb, 1 patient stage IVa and another patient stage IVb tumor. Intracranial invasion was extradural in 8 cases and intradural in 2 patient. Surgery was performed in 9 cases and the most common was combined approach: infratemporal fossa and sublabial transantral. One patient was referred for radiotherapy. Follow-up ranged from 8 to 26 years. There was extracranial recurrence in 2 (22%) of 9 operated patients. C onclusions: The superior orbital fissure is the most frequent route of intracranial spread in patients with extensive involvement of the infratemporal fossa. Due to high risk of recurrence and potential serious complications advanced cases of JNA should be managed by experienced multidisciplinary team, preferably in tertiary referral centers, with an access to modern diagnostic and therapeutic modalities.

Juvenile Nasopharyngeal Angiofibroma Outcomes and Cost: Analysis of the Kids' Inpatient Database.

To report trends in Juvenile Nasopharyngeal Angiofibroma (JNA) hospitalizations and identify key factors affecting treatment outcomes and cost of care in JNA patients. The Healthcare Cost and Utilization Project (HCUP) Kids' Inpatient Database was queried for all cases of JNA between the years of 1997 and 2016. Key factors extracted were patient demographics, geographic region, hospital size, teaching status, elective admissions, and number of diagnoses and procedures performed during the hospitalization. These elements were correlated to length of stay (LOS) and cost-per-day (CPD) using a multiple linear regression (MLR). Regional variation in JNA diagnosis and changes in LOS and CPD trends over time were also analyzed. A total of 614 JNA patients were hospitalized in this time period, with a majority of patients identifying as male (98%) and Caucasian/White (55%). The average LOS has decreased by 0.14 day per year since 1997 (P = .0034) whereas the CPD has steadily increased by $2 380 per year (P < .001). MLR analysis revealed that while holding all other factors constant, patients who stayed at teaching hospitals had an increased LOS of 1.7 days (P = .026), but paid $11 961 less per day (P = .05). Regional variation in CPD was found in the Northeast region, where hospitalizations were more expensive by $9 801 per day compared to the South (P = .017). These results indicate hospital characteristics, such as teaching status and geographic region, may predict differences in JNA outcomes and cost. Healthcare providers should be cognizant of these variations to ensure optimal patient outcomes and expenditures.

Trend analysis in management of juvenile nasopheryngeal angiofibroma: our institutional experience

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; Juvenile nasopharyngeal angiofibroma is a vascular malformation rather than true neoplasm. It accounts for 0.5% of all head and neck tumors and its general incidence is 1:150,000. It is uncommon, benign, extremely vascular tumor that arises from tissues in the sphenopalatine foramen, the pterygoid base causing Early spread submucosally towards nasopharynx. Surgical excision is mainstay of treatment.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; This is a retrospective observational study carried out from March 2002 to March 2019, 130 patients underwent surgical resection of juvenile nasopharyngeal angiofibroma at Civil Hospital, Ahmedabad. Patients were divided in two groups with Group-A having patients from 2002-2010 and Group B having patients from 2011-2019.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results:&lt;/strong&gt; A total 130 cases of juvenile nasopharyngeal angiofibroma with a mean age of 16.25 years (range 9-33) were treated by surgical excision from March 2002 to March 2019. In Group A patients open approach was used most often with endoscopic approach used only till stage II-A. In Group B patients the preferred surgical approach was trans-nasal endoscopic approach cases up to stage IV-A and open approach including the midfacial degloving approach and infratemporal approach was used cases with extensive intracranial, lateral infratemporal fossa, orbit, optic nerve, cavernous sinus involvement.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; Our study shows that over the years there has been shift in surgical approaches used in treating angiofibroma with the advent of newer endoscopic method and addition of various technology such as coblation, navigation has made the procedure for a highly vascular tumor simpler.&lt;/p&gt;&lt;p class="abstract"&gt; &lt;/p&gt;

Juvenile Nasopharyngeal Angiofibroma: Changing Paradigms in Management

Introduction&#x0D; Juvenile Nasopharyngeal Angiofibroma (JNA) is a tumor of young and adolescent males. It is a benign vascular tumor arising from the spheno-palatine foramen. It is best managed surgically at present by endoscopic methods with or without pre-operative embolization. Tumor attributes like intracranial extent and residual vascularity after embolization need to be assessed pre-operatively before undertaking endoscopic surgery, in order to reduce surgical blood loss and morbidity.&#x0D; Materials and Methods&#x0D; Twenty-three cases of JNA (n=23) were operated endoscopically at a tertiary level military hospital. They were staged with the Snyderman staging system. Demographic variables including stage wise management were brought out with intraoperative time and blood loss recorded for different stages.&#x0D; Results &#x0D; The intraoperative surgical time, intra-operative blood loss and recurrence/residual rates were compared with similar studies in existing literature and correlated well.&#x0D; Conclusion&#x0D; Endoscopic Endonasal approach to JNA is now a well-established technique. With improvements in embolization techniques and better instrumentation like HD camera systems, endo-bipolar cautery, Coblation and endo-liga clips and neuro-navigation, better visualization and better haemostatis can be achieved, resulting in significant reduction in the morbidity and improvement in surgical results.

Patterns of vascularization and surgical morbidity in juvenile nasopharyngeal angiofibroma: A case series, systematic review, and meta-analysis.

Vascular patterns of juvenile nasopharyngeal angiofibroma (JNA) are poorly defined. We performed both institutional and systematic literature reviews to characterize the relationship between arterial supply patterns of JNA with intraoperative blood loss and tumor recurrence. A retrospective review of 26 patients with JNA treated at our institution from 1995 to 2015 with available angiograms, and systematic reviews and meta-analyses of 828 JNA cases undergoing angiographic embolization published between 1995 and 2015 were completed per Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The systematic review (828 cases) found internal carotid artery (ICA) supply in 35.6% of tumors, and 30.8% of tumors received bilateral vascular supply. Our institutional data (n = 26) indicated 69% had bilateral supply. Meta-analysis of data from 5 studies demonstrated ICA/bilateral arterial supply is predictive of increased operative blood loss (P < .01). Complex vascular contributions to JNA are frequent, underreported, and portends increased blood loss. This information can justifiably be included in staging systems to enhance prognostic counseling of patients.

Clinico-radiological evaluation of Juvenile Nasopharyngeal Angiofibromas: two case reports

Background: Juvenile nasopharyngeal angiofibroma (JNA) also called as bleeding fibroma as this is highly vascular tumor. This is a benign tumor of prepubertal and young adults which is locally aggressive and has got tendency to bleed. The extension of the tumor takes place because of potential available nasopharyngeal space. Case presentation: We present two cases of juvenile nasopharyngeal angiofibromas in different stages with highlighting the role of contrast enhanced computerized tomography (CECT). Conclusion: We emphasize over the clinico-radiological evaluation for making the diagnosis. The correct pre-operative radiological staging is mandatory prerequisite for the surgical management.

Endonasal Endoscopic Excision of Juvenile Nasopharyngeal Angiofibroma- The Technical Difficulties &amp; Advantages

Objective: Endoscopic excision of juvenile nasopharyngeal angiofibroma (JNA) was carried out with the objective of minimizing blood loss and attempting to complete excision of tumor under direct vision with the help of Hopkins telescope.Study design: A retrospective 12 year study of 6 cases of JNA treated by endoscopic excision is presented.Result: According to Radkowski’s classification, two patients were stage Ia, two were stage Ib and two patients were stage IIb. The mean duration of the surgery was 2 hours. The mean intra-operative blood loss was 575 ml. The mean follow –up after the primary operation was 23.3 months. All the patients but one were free of disease. One patient had a recurrence in the pterygopalatine fossa requiring a successful revision procedure 3 years after the primary surgery.Conclusion: Endoscopic resection of JNA is a difficult but effective operation in experienced hands. The endoscopic management had less intra operative blood loss, lower occurrence of complications, shorter length of hospital stays and lower rate of recurrence. So it should be considered as a first choice option.Birdem Med J 2014; 4(2): 74-78

Nasopharyngeal angiofibroma: A concise classification system and appropriate treatment options

ObjectivesTo describe a clear and simplified classification system for juvenile nasopharyngeal angiofibroma (JNA), and to describe suitable management options. Study designRetrospective medical record review. MethodsThe clinical and imaging materials of 51 cases of JNA diagnosed at our hospital between 1981 and 2011 were collected and studied. Based on our experiences, we prefer to divide JNAs into three types. Type I includes JNAs fundamentally localized to the nasal cavity, paranasal sinus, nasopharynx, or pterygopalatine fossa. Type II is a JNA extending into the infratemporal fossa, cheek region, or orbital cavity, with anterior and/or minimal middle cranial fossa extension but intact dura mater. Type III is a calabash-like massive tumor lobe in the middle cranial fossa. The management and prognosis for the three types of JNA were compared and evaluated. ResultsAmong cases of type I JNA (n=16), the entire mass was removed by the initial operation in 15 cases and by a repeat operation in 1 case. Among cases of type II JNA (n=29), the entire mass was removed by the first operation in 24 cases and by repeat operation in 5 cases. In cases of type III JNA (n=6), the huge calabash-like lobe in the middle cranial fossa could not be completely excised; 4 cases underwent radiotherapy and 2 cases were lost to follow-up. Conclusions1) The transnasal cavity approach with endoscopic guidance is suitable for type I JNA resection. 2) The transantral–infratemporal fossa–nasal cavity combined approach is reliable for resection of a type II JNA, which extends into the deep anterior cranial fossa and/or minimally into the middle cranial fossa, with intact dura mater. 3) The complete removal of a type III JNA is difficult, even through a combined extracranial and intracranial approach. Radiotherapy is useful for treating the residual intracranial tumor. The successful or failed experiences of 6 typical cases prove that this revised classification system is reasonable and reliable.

Letter to the editor: surgical management of advanced juvenile nasopharyngeal angiofibromas

Dear Editor We read with great interest the paper of Cherekaev et al., Advanced craniofacial juvenile nasopharyngeal angiofibroma. Description of surgical series, case report, and review of literature” published in Acta Neurochir (2011) 153:499–508 [3]. In this report the authors review their experience with 27 cases of patients with juvenile nasopharyngeal angiofibromas (JNAs) Fisch grade III and IV. Although the authors must be congratulated for their high rates of gross total resection (83%), there are some specific points in their paper that deserve special consideration. The authors rightly state that in advanced cases of JNA, “Non-radical excision of tumors may be also due to presence of blind zones and areas of limited access which cannot be visualized by operative microscope." Due to this fact most of the recent surgical literature has focused on the progressive role of either purely endoscopic or endoscopicassisted procedures, instead of the conventional microscopic techniques [1, 4, 5]. In our recently published series focusing exclusively on JNAs with intracranial extension, total resection was achieved in 85% of the cases through endoscopic-assisted and purely endoscopic techniques in up to two surgical interventions [8]. Our series seems to reflect the broad tendency of the surgical literature, especially regarding the transition from combined microscopic-endoscopic techniques (used in our institution before 2008) to purely extended endoscopic approaches alone or in combination with minor sublabial incisions [7]. Therefore, based on this experience, we cannot agree with the authors that the “endoscopic approach has its limitations: tumors Fisch grades III/V with advanced intracranial involvement including cavernous sinus, orbit, growth around optic nerves, lateral growth into infratemporal fossa, and spreading behind the pterygoid process which conflicts with endoscopic exploration. In such cases, endoscopic resection is not indicated.” In our experience of 20 patients with JNAwith intracranial extension, all patients presented involvement of the cavernous sinus, and spread into the orbit was observed in five cases. Such cases were successfully treated with endoscopic techniques with the addition of anterior maxillectomy in some selected cases (Fig. 1). All the aforementioned areas, which the authors suppose to be a contraindication for endoscopic approaches, can be successfully exposed by the addition of the extended modules of endoscopic endonasal approaches reported by the UPMC group (transpterygoid/infratemporal approach, zone 5; the cavernous sinus, zone 4) [9]. In relation to advanced tumors, the authors state that “Currently, three different opinions can be distinguished:

99mTC‐octreotide scintigraphy and somatostatin receptor subtype expression in juvenile nasopharyngeal angiofibromas

The main goal of the study was the analysis of somatostatin receptor (SSTR) expression on juvenile nasopharyngeal angiofibroma (JNA) cells and a subsequent analysis of the utility of SST analog-based scintigraphy in JNA diagnostics. Nine JNA cases were analyzed. All tissue samples were analyzed for the expression of SSTRs. In 2 cases, scintigraphy was performed after the intravenous (IV) administration of an SST analog. MRI of the craniofacial region was subsequently performed. The SST analogues were accumulated in areas matching pathologic tissue in the nasopharynx. Immunohistochemical evaluation of the tissue samples proved the overexpression of SSTRs. SSTRs are overexpressed on JNA cells. The SST analog (99m)TC-octreotide is effectively bound to JNA cells. SST analogues might be used in the diagnostics and treatment of primary, recurrent, or residual JNA.

Bilateral vascular supply in juvenile nasopharyngeal angiofibromas

Juvenile nasopharyngeal angiofibroma (JNA) is a rare, benign tumor of the nasopharynx, exclusively affecting males in their teens and twenties. Historically, it has been thought that JNAs primarily receive their blood supply from the ipsilateral external carotid system. We demonstrate in our case series and literature review that bilateral vascular supply in these tumors is, in fact, very common. Case series and literature review. We present four consecutive cases of JNA in a three-year period from a major tertiary care center. A literature review on the topic of vascular supply in JNA was performed. The first patient's preoperative angiogram demonstrated only right-sided blood supply. However, significant bleeding was encountered, and an intraoperative angiogram showed significant new vascularization from the left ascending pharyngeal artery (APA), which was embolized. The surgery was completed successfully without further significant hemorrhage after converting to an open approach. Preoperative angiograms from the next three patients demonstrated bilateral vascular supply. Embolization and endoscopic resection was successful in these cases. Review of the literature demonstrates that bilateral vascular supply is not commonly documented; however, it may be more frequent than previously thought. Other series demonstrate bilateral vascularity from 0% to 100%. Combining patients from nine studies, including our own, we found that 57 of 157 (36%) patients had tumors with bilateral vascular supply. Bilateral vascular supply may be an underappreciated factor in JNA, and thorough radiographic investigation via angiography of bilateral carotid systems should be routinely done preoperatively.