Cases Of Juvenile Nasopharyngeal Angiofibroma Research Articles

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.

Interventional Radiology-Case Report: Pre-operative Embolisation of Juvenile Nasopharyngeal Angiofibroma

We report a case of juvenile nasopharyngeal angiofibroma in a 15-year old male patient. CT PNS was suggestive of juvenile nasopharyngeal angiofibroma. Subsequently preoperative embolisation of the feeding vessel was done to make the surgery blood less.

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.

Biological distinctions between juvenile nasopharyngeal angiofibroma and vascular malformation: An immunohistochemical study

The exact nature of juvenile nasopharyngeal angiofibroma (JNA) is still in dispute. In recent years, the main controversy of its nature has focused on hemangioma and vascular malformation. In this study, the immunolocalization of vascular endothelial growth factor (VEGF), VEGF receptor-1/fms-like tyrosine kinase-1 (VEGFR-1/Flt-1), VEGF receptor-2/fetal liver kinase-1 (VEGFR-2/Flk-1), proliferating cell nuclear antigen (PCNA), and CD34 was investigated in 28 cases of JNA and 20 cases of orbital cavernous hemangiomas (OCH). The immunostaining levels of VEGF, Flt-1, and Flk-1 were higher and more frequent in vascular endothelial cells of JNA than those of OCH ( p < 0.05). The average microvessel density (MVD) marked by CD34 in JNA was (49.3 ± 9.1)/HPF (high power field), which was higher than OCH (29.1 ± 6.7)/HPF ( p < 0.05). Immunoreactivity of PCNA was localized in both endothelial and stromal cell components of JNA, but was predominantly seen in the stromal cells. However, no PCNA immunoreactivity was identified in any of the stromal and endothelial cells in cases of OCH. The immunostaining levels of CD34, VEGF, Flt-1, Flk-1, and PCNA in JNA were higher than those in OCH. These data support the view that JNA has biological characteristics of an angiogenic histogenetic tumor. In the future, anti-angiogenic therapy may represent a novel treatment strategy for JNA.

Endoscopic approach for excision of juvenile nasopharyngeal angiofibroma: complications and outcomes

The purposes of this study are to report the efficacy of the endoscopic approach for juvenile nasopharyngeal angiofibroma (JNA) and to compare its related intra- and postoperative complications with findings from traditional approaches in the literatures. This study is a retrospective report of 47 cases of JNA that were treated with nasal endoscopic surgery between 1998 and 2005. According to the staging system by Radkowski et al ( Arch Otolaryngol Head Neck Surg. 1996;122:122–129), the staging of the included patients were the following: 21 in stages IA to IIB, 22 in IIC, 3 in IIIA, and 1 in IIIB. Five patients were embolized before surgery. The mean blood hemorrhage in embolized patients was 770 mL, whereas in nonembolized patients, it was 1403.6 mL. In the follow-up period (mean, 2.5 years), the recurrence was found in 9 patients (19.1%), and mean time of recurrence was 17 months after surgery. The rupture of cavernous sinus occurred in 2 cases with no mortality. The mean hospital stay was 3.1 days in all cases and 1.8 days in embolized patients. The findings of this study demonstrate that endoscopic resection of JNA is a safe and effective technique because of decrease in blood loss, hospitalization, and recurrence rate, especially in tumors that are not extended through intracranial space. It is therefore strongly recommended that this modality is implemented as the first surgical step for tumors with stages I to IIIA of the Radkowski's staging system.

Juvenile Nasopharyngeal Angiofibroma: The Expanded Endonasal Approach

Juvenile nasopharyngeal angiofibroma (JNA) is a benign but locally aggressively vascular tumor that may involve the skull base and extend intracranially. Endoscopic excision has become the new standard for smaller tumors but remains a challenge for large tumors, leading some to advocate radiation therapy. We reviewed our experience managing JNA, specifically with respect to utility of the expanded endonasal approach (EEA) for lesions with skull base and intracranial extension. All cases of JNA at the University of Pittsburgh Medical Center from 1995 to 2006 were reviewed with respect to tumor size and location, vascular supply and results of embolization, skull base involvement and intracranial extension, surgical approach, blood loss, intraoperative and postoperative complications, and recurrence. Thirty-one cases of JNA were identified. The majority of tumors were completely excised using the EEA, regardless of size or extension into adjacent compartments. Surgical excision of some tumors with intracranial blood supply was staged. Recurrence rates were not associated with extent of tumor or surgical technique. Long-term morbidity was minimal. Most JNA, regardless of tumor extent, may be completely excised using EEA alone or in combination with minor sublabial incisions avoiding the morbidity associated with larger open approaches or postoperative radiation therapy.

Efficacy of Flutamide in Nasopharyngeal Angiofibroma

Objective To assess the efficacy and safety of Flutamide (androgen receptor blocker) as an adjuvant therapy for presurgical tumor volume reduction in cases of Juvenile Nasopharyngeal Angiofibroma (JNA). Methods Therapeutic trial, Janurary-December 2007. 10 previously untreated cases of JNA (Radkowski stage IIC-IIIB) were administered Flutamide (10 mg/kg in 3 divided doses) for 6 weeks prior to surgical excision. Pre- and post-therapy tumor volume assessment undertaken by MR Imaging. All cases had periodic liver function tests and assessment of primary and secondary sexual characteristics continuing till 6 months post-therapy. Results Pre-pubertal cases had inconsistent and minimal responses to Flutamide. All 7 post-pubertal cases (as indicated by age and serum testosterone levels) however, demonstrated consistent tumor volume reduction ranging from 6% to 35% (mean 16.6 %). Two cases with optic nerve compression had significant improvements in visual acuity and visual fields. No adverse effect was noted excepting for mild and transient breast tenderness. Conclusions Preoperative use of Flutamide is safe and leads to partial tumor regression in post-pubertal patients. The regression of the tumor away from adjacent vital structures may facilitate surgical excision and limit complications.

Lacrimal hyposecretion: a surgical complication of juvenile nasopharyngeal angiofibroma

Purpose The aim of this study was to evaluate lacrimal hyposecretion in the surgical management of juvenile nasopharyngeal angiofibroma (JNA) and discuss how to prevent and treat this expected surgical complication. Materials and methods Six cases of JNA were extirpated surgically in the last 3 years in our department. The clinical signs, results of the total tear secretion test (Schirmer I test), imaging studies, surgical findings, and pathologic changes in these cases are reviewed retrospectively. Results Three of the 6 cases developed an irritated dry eye on the affected side postoperatively as a result of lacrimal hyposecretion, caused by damage to or partial removal of the pterygopalatine ganglion and vidian nerve. On follow-up for 1 to at least 2 years, the clinical signs disappeared in 2 cases, whereas there was no improvement in 1 case. Conclusions If a JNA originates near the pterygopalatine foramen adjacent to the pterygopalatine ganglion and vidian nerve, these may be damaged or partially removed intraoperatively. The consequent lacrimal hyposecretion can be a temporary or permanent complication. Significance A few reports have described lacrimal hyposecretion as a surgical complication of JNA; there have been no articles reporting the prognosis of this complication or the related pathologic findings of the involved pterygopalatine ganglions. Prevention of this complication remains a clinical challenge.

Huge nasopharyngeal angiofibroma with intracranial extension: change in the dura mater and choice of surgical management

We aimed to review (1) the imaging changes in the dura mater in cases of huge, lobulated juvenile nasopharyngeal angiofibroma, and (2) the choice of surgical management. Imaging from four cases of juvenile nasopharyngeal angiofibroma showed extrapharyngeal extension of the tumour. The sphenoid sinus, sella turcica and clivus were extensively eroded, and the tumour had spread deep into the cranial fossa. In three cases, intracranial exploration was performed to treat the intracranial tumour lobule. Subsequently, the tumours were removed using extracranial approaches. No perforation of the dura mater was found in these three cases, although the dura mater in the superior orbital fissure was congested, haemorrhagic and solid. Pre-operative imaging for two cases (i.e. the first operation for one and the second operation for the other) revealed no dura mater perforation. A transantral approach via a midfacial degloving incision was used to remove these tumours completely. We conclude that change in the dura mater is a crucial indication for the choice of management. If the dura mater is intact, a transantral approach via a midfacial degloving incision may remove the tumour successfully.

Juvenile nasopharyngeal angiofibromas in Denmark 1981–2003: diagnosis, incidence, and treatment

Conclusions. Juvenile nasopharyngeal angiofibroma (JNA) is a rare tumor in young males, with a non-negligible potential for recurrence. Preoperative embolization is a safe procedure that diminishes the peroperative blood loss and the need for blood transfusion. The endoscopic approach was used with good results in JNA stage I and II (Chandler). Objectives. To estimate the incidence rate of JNA in the Danish population and to describe symptoms and treatment. Patients and methods. This was a national retrospective cohort study. All cases of JNA diagnosed in Denmark from 1981 to 2003 were identified. Data were extracted from medical records. Results. Forty-five male (no female) JNA cases were identified. In 43 cases, clinical data were recovered. Median age was 15 years. The incidence rate in Denmark was 0.4 cases per million inhabitants per year and 3.7 cases per million males (aged 10–24) per year. All patients underwent surgery, and the endoscopic approach was increasingly being used. The embolization procedure proved to be safe and decreased the intraoperative blood loss statistically to 650 ml in the embolized group from an average of 1200 ml in the non-embolized group (p<0.05). Similarly, the need for peroperative blood transfusion was reduced (p<0.005). The primary recurrence rate was 23% and no patients died.

若年性鼻咽腔血管線維腫における病期分類別手術法の検討

Juvenile nasopharyngeal angiofibromas (JNAs) are benign tumors that occasionally invade the pterygopalatine fossa, infratemporal fossa, or middle cranial fossa. Several surgical approaches have been used based on the location of the tumor, including transpalatal, transmaxillary, and lateral rhinotomy, midfacial degloving, and Le Fort type I osteotomy. We reviewed 4 cases of JNA that had been treated by resection in the Department of Otolaryngology of Kagoshima City Hospital and 31 cases of JNA reported in the Japanese literature between 1990 and 2003. We analyzed the outcome of the surgical treatment of JNA in these cases to identify the surgical approaches that were most effective in removing tumors at several different stages. Radkowski staging showed that 17 (48.6%), 2 (5.7%), 4 (11.4%), 9 (25.7%) and 3 (8.6%) patients had stage IA, IB, IIA, IIC, and IIIA tumors, respectively. A transpalatal approach was employed in 11 cases (31.4%), a transmaxillary approach in 9 cases (25.7%), a transnasal approach in 6 cases (17.1%), and other approaches, (25.7%). The recurrence rate was, 5% in the stage I cases, 38% in the stage II cases, and 33% in the stage III cases. The transpalatal approach was followed by a high recurrence rate in the cases of stage II and higher stage. Several factors are critical when choosing the surgical approach to JNA: adequate exposure of the tumor, ability to control bleeding, prevention of postoperative facial deformity, and avoidance of interference with growth of the face.

若年性鼻咽腔血管線維腫７例の検討

We report seven cases of juvenile nasopharyngeal angiofibromas (6 males and a female, averaged 17.3 years old) treated at Mie University Hospital for 20 years during 1983 and 2003. Tumors ranged from 20 to 90mm, but these size was not related to tumor stage. In patients, the surgery was performed by a trans-oral (4 cases) or trans-maxillary (3 cases) approach. In two cases, palatal fistula was observed, and later closed surgically. Two recurrent tumors were observed at about one year postoperatively and those cases were successfully underwent surgery again by the trans-maxillary approach. Intraoperative blood loss volume was large in advanced staged cases, but the volume was not related to tumor size. Pre-operative thrombosis via a maxillary artery under angiographic guidance, was performed in each case. This treatment was effective, when performed the day before surgery.

Endoscopic excision of Juvenile nasopharyngeal angiofibroma — A comprehensive approach

Endoscopie excision of Juvenile Nasopharyngeal Angiofibroma (JNA) was carried out with (he objective of minimizing blood loss and attempting a complete excision of the tumor under direct vision with the help of Hopkins telescopes. A prospective 4 year study of 23 cases of JNA treated by endoscopie excision is presented. Of these, 18 were treated by endoscopie excision alone. The remaining 5 were treated with a two staged approach either by mid-facial degloving followed by endoscopy or by 2 endoscopie procedures. The tumor was excised completely in 17 out of the total 18 cases that were treated exclusively by endoscopy. One case has shown a recurrence. The 5 cases treated by the staged approach represented very large tumours or tumours with intra-cranial extensions. In I of these cases, inoperable tumor remnant engulfing the internal carotid artery was treated by radiotherapy post-operatively. With successful excision of JNA in all but one case, we could reasonably conclude, that endoscopie excision of JNA could become a safer and a more precise alternative to open surgery provided it is practiced judiciously by surgeons who have considerable experience in endoscopie surgery and the necessary backup to convert to open surgery should the need arise.

Endoscopic resection of nasopharyngeal angiofibromas by combined transnasal and transoral routes

Purpose: To determine the role of endoscopic surgery in decreasing intraoperative bleeding, morbidity, and hospitalization period of juvenile nasopharyngeal angiofibroma resection and to describe combined endoscopic transnasal and transoral approaches. Patients and Methods: Twelve cases of juvenile nasopharyngeal angiofibroma diagnosed by endoscopic examination, computed tomography, and angiography were selected for endoscopic resection. Tumor staging ranged from stage I A to II B. Ten patients underwent preoperative selective arterial embolization, and in 1 case selective arterial ligation was used. In general, the tumors were approached endoscopically through nasal and oral cavities with 0° and 30° 4-mm telescopes without any incision and no packing at their termination. Results: The patients were followed by endoscopy and computed tomography. There was a dramatic decrease in intraoperative bleeding and postoperative morbidity. No early postoperative complications were seen. Two recurrences were observed in 12 patients up to a mean follow-up of 15 months. Conclusions: Minimal bleeding, decreased morbidity, and shorter hospitalization period were the main reasons that prompted us to use endoscopic technique for the removal of juvenile nasopharyngeal angiofibroma. Adding transoral endoscopic approach to the transnasal endoscopic approach provides 2-sided exposure and appreciate access to angiofibroma.

Relaxation of imprinting of IGFII gene in juvenile nasopharyngeal angiofibromas.

IGFII and H19 genes are expressed only from one allele due to genomic imprinting, biallelic expression (loss of imprinting) being associated with the tumorigenic process of different types of tumors. The mechanism responsible for genomic imprinting is not yet determined, although DNA methylation has been considered the main genetic event for an imprinted mark. In the current study, the authors analyzed the imprinting status and expression levels of the IGFII and H19 genes in 27 cases of Juvenile Nasopharyngeal Angiofibroma (JNA) using RFLPs, RT-PCR, and Southern and Northern Blots. The authors found that four out of eight informative cases (50%) for ApaI/IFGII polymorphism showed biallelic expression of IFGII whereas none of the nine informative cases for the polymorphism showed biallelic expression of the H19 gene. Overexpression of IFGII was observed in 8 out of 22 cases (36.4%), and 7 out of 19 cases (36.8%) showed H19 overexpression. Hypomethylation was found only in the H19 gene in six out of eight cases analyzed. Therefore, our results demonstrate that alterations in the IFGII/H19 imprinted region occur in JNA.

Juvenile nasopharyngeal angiofibromas: A study of recurrence pattern and role of pre-Operative embolization - ‘a decade’S experience’

This study is a retrospective analysis of 30 consecutive cases of Juvenile Nasopharyngeal Angiofibroma (JXA) operated at. Department of Head and Neck Surgery, Kidwai Memorial Institute of Oncology Bangalore, India: la tertiary referral centre) after prior emohilization by an interventional neuro-radiologisl (1996-2002). This study discusses critically the planning of surgical approach, based on anatomico-radiological factors and highlights the efficacy of preoperalive embolization in expediting total re moral of the tumor in 25 out of JO cases with advanced stage JNA. To analyze the utility of pre-operatire embolisation in surgical extirpation of large JNAs; planning of the surgical approaches based on CT topography of the tumor; to study the various complications of embolisation and surgery associated with JXA & lastly to evaluate the puttern and location of recurrent tumor thus correlating with the original topography. Tertiary care cancer referral centre. PATIENTS ranged in age from ')- 24 years. all being males. Majority of them were accessed by transfacial surgical approach(26). and in the recent past via midfacial degloving(4) within 4H hours of angioembolisation. Complete removal of the tumor was achieved in 25 out of 30 cases with advanced stage JNA.Post surgical CT scans revealed tumor residua in 5 individuals, where the tumor was documented in - the temporal fossa 12), para-cavernous sinus region (I), cavernous sinus! I) and pterygo palatine fossa (I). Only the lesion in pterygopalaline fossa was successfully re-i>xcised & this alongwith the recurrence at para-cavernous & cavernous sinus & another were treated with radiotherapy; the 2 cases in the temporal fossa are under observation. The average blood loss during the procedure was 546.60 ml. Today, advances in radiologie imaging-complemented by interventional neuro-radiological expertise in angio-embolisation have expedited complète excision with minimal morbidity and acceptable recurrence rate. This study has justified pre-operative embolisation and M currently the standard of care for advanced JXA.

Endoscopic and KTP laser-assisted surgery for juvenile nasopharyngeal angiofibroma

Juvenile nasopharyngeal angiofibroma is a highly vascular tumor arising from the area around the sphenopalatine foramen. Various radical and extended radical surgeries have been advocated to surgically excise both extranasopharyngeal and nasopharyngeal juvenile angiofibromas. However angiofibromas involving the nasopharynx, nose, and sphenoid with minimal lateral extension via the sphenopalatine foramen can also be adequately managed endoscopically either alone or with 1 of the traditional approaches. Nine cases of juvenile nasopharyngeal angiofibroma were successfully managed between January, 1999, and March, 2001, by preoperative selective embolization of the internal maxillary artery with or without external carotid artery clamping, followed by endoscopic excision. Two of the 9 cases underwent KTP/532 laser-assisted endoscopic excision, whereas the transpalatal approach was used along with the endoscope in another 2 cases. The patients remained free of disease after a median follow-up period of 17 months. We report our preliminary experience in endoscopic and KTP laser–assisted excision of juvenile nasopharyngeal angiofibroma. (Am J Otolaryngol 2002;23:282-286. Copyright 2002, Elsevier Science (USA). All rights reserved.)

경비 내시경 수술로 제거한 유년성 비인강혈관섬유종 3례

경비 내시경 수술로 제거한 유년성 비인강혈관섬유종 3례

Endoscopic excision of Angiofibroma

We present a case of Juvenile Nasopharyngeal Angiofibroma (JNA) managed by endoscopic excision alone. The selection of the case, technical difficulties and the advantages offered by this technique are detailed.

Anterior approaches in juvenile nasopharyngeal angiofibromas with intracranial extension

Although surgery is regarded as the mainstay of treatment for juvenile nasopharyngeal angiofibromas (JNAs), ancillary treatment modalities such as radiotherapy and on rare occasions chemotherapy are still recommended by many for intracranial extension with apparent radiologic involvement of the cavernous sinus and internal carotid artery. Further, most authors undertaking surgical excision of this subgroup of patients would recommend a lateral or combined frontal and lateral approach for its removal. In a series of 49 cases of JNA, 14 were found during surgery to have intracranial extradural extension; the anterior approach was used for their removal. Although in these cases, on radiography the cavernous sinus often looked to be invaded and the internal carotid artery was displaced superolaterally, there was no difficulty in establishing a plane of dissection. Total removal was achieved in 11 of the 14 cases with a single-stage procedure. Of the 3 cases with residual tumor, only 1 occurred intracranially. Removal was achieved by a subtemporal approach in this case. For the extracranial residual tumors 1 required a midface degloving and the other, with a 1-cm residual tumor in the nasopharynx, has been treated conservatively for 6 years with no evidence of growth. No deaths or significant complications have occurred, and radiotherapy has not been required. We conclude that JNAs are tumors with a predilection for spread but that rarely invade dura, acting instead to displace it. We believe that surgery is the method of choice for treating these lesions and that an anterior surgical approach with microsurgical techniques should be used in the first instance. In the last 2 cases we preferred a midface degloving technique to avoid facial scarring and because this approach allows a widening of the surgical field if needed by the performance of bilateral maxillary free bone flaps. On the rare occasion that a lateral approach, with its attendant permanent conductive hearing loss, is found to be necessary for total tumor removal, this can be done as a staged procedure. This may be necessary when the tumor has spread lateral to the horizontal internal carotid artery. (Otolaryngol Head Neck Surg 2000;122:277-83.)