Juvenile Nasal Angiofibroma Research Articles

Our First Experience of Using Blue Light Laser for Endoscopic Endonasal Removal of Juvenile Angiofibromas

Background. Juvenile nasal angiofibroma (JNA) is a very rare benign lesion originating from the pterygopalatine fossa with distinctive epidemiologic features and growth patterns. The typical patient is an adolescent male with a clinical history of recurrent severe nasal bleedings and blockage the nose. Currently, even though surgery often caused severe operative bleeding, it is considered the ideal treatment for JNA. Refinement in preoperative embolization, which provides significant reduction of intraoperative bleeding. In spite of breakthrough of Endoscopic techniques which minimize the risk of residual disease, the search of new devises which can help to management of JNA is still continue in order to minimize the surgical complications. Laser methods as surgical treatment have been actively used in rhinology during the past decades. We purposed to summarize information about current lasers and their use in rhinology. Aim. The purpose of the study is the literature review and to describe our experience of using new blue laser during JNA removal. Evaluation of advantages and disadvantages, determination of indications and limits of its use in rhinosurgery Methods- A literature review from 2000 to 2022 using the PubMed database was employed. Keywords used included “laser surgery”, “blue light laser”, “photoangiolytic laser”, “laser in rhinosurgery”. The most up to date studies published for each rhinology condition that was treated with laser surgery was reviewed. Then endoscopic removal through the nose was performed. During the surgery TrueBlue laser was used to cut the tissues and coagulate injured vessels. Results. Rhinological conditions appropriate for laser applications are discussed. There are related papers to a number of applications including hereditary hemorrhagic telangiectasia, rhinitis, turbinate surgery, dacryocystorhinostomy, septoplasty, choanal atresia, and sphenopalatine artery ligation, paying attention to the outcomes of the studies. It is the first experience of using blue laser in rhinosurgery and particulary ib removing JNA. Conclusion. Intranasal laser surgery, despite the fact that interventions are performed almost bloodlessly, and often do not require nasal tamponade, indications for their performance are limited, due to deep burns of the nasal mucosa, and alteration of mucociliary clearance therefore lasers are not so often used in rhinology. The short-wave blue laser with a wavelength of 445 nm, used in Europe in oto- and laryngosurgery, may have some potential advantages in rhinosurgery, but the practical dataset are limited yet.

Angiectatic Polyp-An Unusual Nasal Mass in a Young Adult.

Angiectatic nasal polyps (ANP) are rare pseudo-neoplastic lesions that might raise intriguing pathophysiological issues and present a significant diagnostic challenge. They are also referred to as inflammatory granuloma telangiectaticum, vascular granuloma, pseudo-angioma, and angiomatous/angiectatic polyp. However, the name angiectatic polyp refers to the fact that the lesion is not a true tumour and is clinically distinguished by ectatic vasculature, haemorrhage, and persistent proliferation. The most confusing aspect of this polyp is its clinical appearance, which resembles a juvenile nasal angiofibroma. Despite its typical imaging characteristics, this lesion is difficult to differentiate radiologically. Hence, histopathology is paramount for establishing diagnosis. We report a rare case of a young adult who presented with complaints of nasal block and epistaxis. Examination revealed a vascular nasal mass and CT scan demonstrated the lesion to be epicentred in right nasal cavity extending to multiple sinuses with adjacent bone erosion. Patient underwent endoscopic excision of the mass with JNA as the predominant differential and only following histopathological examination, the final diagnosis was confirmed.

Single-Center Experiences of Preoperative Juvenile Nasal Angiofibroma Embolization With Gelfoam, Reducing Financial Burden on Patients in Developing Country.

IntroductionJuvenile nasal angiofibroma (JNA) is a highly vascular tumor of the nasopharynx. Endovascular embolization followed by surgery is the treatment of choice. This study aimed to determine that single catheter technique with Gelfoam is an effective and safe technique for embolization to reduce the financial burden on patients in a developing country.Materials and methodsWe retrospectively reviewed the imaging, surgical, and histopathological records of 108 patients who underwent preoperative endovascular tumor embolization followed by surgical resection between March 2017 and March 2021.ResultsAfter embolization no major complication was observed in any patient. Complete devascularization of tumor was done in 87.8%. Intraoperative blood loss resulting in transfusion was almost the same as with other embolization techniques.ConclusionSingle catheter with Gelfoam is a cost-effective and safe technique for JNA embolization.

Pterygoid Base Pneumatization Simulating Residual Disease-A Pitfall of Structural Imaging in Juvenile Nasal Angiofibroma.

As prostate-specific membrane antigen (PSMA) expression is universal in juvenile nasopharyngeal angiofibroma, it is commonly used for specifically differentiating recurrences from surgical site reparative tissues. Postoperative contrast-enhanced MRI revealed a residual disease in the left pterygoid base, but there was no PET/CT uptake with 68Ga-labeled PSMA at the same site. In retrospect, in comparison with preoperative PET/CT, it was found that the pterygoid base pneumatization was showing enhancement in contrast-enhanced MRI and not in functional PSMA. Postoperative 68Ga-PSMA PET/CT is a useful clinical tool in ambivalent diagnostic conditions avoiding morbidities.

Physiological PSMA Uptake in the Tubarial Salivary Glands and Its Implications in the PARIS Protocol-A First Study of Its Kind!

Because prostate-specific membrane antigen (PSMA) expression is universal in juvenile nasal angiofibroma (JNA), PSMA PET/CT is commonly used in the postoperative setting using the postoperative angiofibroma radionuclide imaging study protocol. Our study aims to illustrate physiological PSMA ligand uptake distribution characteristics in the salivary glands, specifically the tubarial glands with tumor, to aid in accurate PSMA PET/CT analysis. Forty-three consecutive patients who underwent a dedicated head and neck spot PSMA PET/CT for JNA were evaluated retrospectively. PSMA SUVmax values in the tumor, tubarial, parotid, submandibular, and sublingual salivary glands were determined visually and quantitatively. All the tumors and the salivary glands showed increased tracer uptake. The median SUVmax ± SD ratios of the tumor, right and left tubarial gland, right and left parotid gland, right and left submandibular gland, and right and left sublingual gland were 2.7 ± 0.8 (range, 0.5-7.5), 3.8 ± 1.9 (range, 0.9-8.1), 4.7 ± 2.1 (range, 0.8-7.4), 9.5 ± 4.8 (range, 3.2-21.9), 9.2 ± 4.7 (range, 2.9-18.9), 10.4 ± 5.7 (range, 3.5-25.4), 10.4 ± 5.5 (range, 3.7-26.4), 6 ± 4.1 (range, 1.3-20.6), and 6.5 ± 4.2 (range, 1.8-19.7), respectively. The uptake in the tubarial glands was comparable with that of the tumor but less compared with other major salivary glands. Ours is the first study demonstrating the quantitative uptake of tubarial salivary glands in detail. Because the tubarial glands uptake and the anatomical location are similar to that of the tumor, these physiological uptakes must be borne in mind to circumvent false-positive interpretations and care must be given during the planning of stereotactic radiotherapy for JNAs.

Juvenile nasal angiofibroma significant locoregional involvement

Juvenile nasal angiofibroma is a benign tumor characterized by abundant vascularization and a tendency to hemorrhage. Despite its benign histology, it can present locally expensive growth that usually presents bone remodeling and the mass effect of adjacent stuctures. We present the case of a 15-year-old patient who came to the emergency department of our center for recurrent epistaxis. After physical examination, it was decided to carry out imaging tests (CT an MRI) that reveal the presence of a large hypervascular mass in the right nostril and maxillary sinus. The patient undergoes surgery after embolization of the tumor.

Clinical Characteristics and Prognostic Factors in Pediatric Vascular Tumors.

BackgroundThe most common type of vascular tumors reported among children is hemangioma. The determinant factors to predict clinical outcomes among those patients were not well studied.ObjectiveThe study aimed to explore clinical characteristics and treatment approaches as well as associated prognostic factors of vascular tumors specifically in a pediatric population.MethodsPediatric patients with a confirmed diagnosis of vascular tumors between January 1, 2005 and December 31, 2017 were enrolled in this study. Clinical data includes initial clinical manifestations with associated complications, and diagnostic studies were used. To establish a diagnosis, the treatment modalities provided and final outcomes were retrospectively reviewed and analyzed.ResultsIn all, 50 patients with a confirmed diagnosis of vascular tumors were enrolled. The median age at diagnosis was 11.5 years with equal gender distribution. The most common type of vascular tumor was hemangioma (n=41, 82%), followed by pyogenic granuloma (n=4, 8%), kapasiform hemangioendothelioma with Kasabach-Merritt phenomenon (n=2, 4%), infantile hepatic hemangioma (n=2, 4%), and juvenile nasal angiofibroma (n=1, 2%). The median age at diagnosis among patients with cutaneous vascular tumors (12.4 years) was significantly older than the age of those with visceral vascular tumors (1.3 years) witha P-value of 0.009. The mean size among patients with visceral tumors (7.46±4.84 cm) was significantly greater than the size among patients with cutaneous tumors (3.21±3.7 cm) with a P-value of 0.023. The size of the tumor was the only independent risk factor associated with clinical outcomes.ConclusionAge at diagnosis in cutaneous vascular tumors was significantly older than in visceral vascular tumors. Clinical outcomes are favorable among most patients and the size of the tumors is an independent risk factor associated with outcomes.

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.

Juvenile nasal angiofibroma (JNA) - review of management with a special focus on surgical approach and resection

Juvenile Nasal Angiofibroma is a condition which almost exclusively occurs in young males who present with features of nasal obstruction and epistaxis. Over the past few decades there has been a paradigm shift in how we manage this condition with changes in diagnostic criteria, imaging modalities, staging as well as surgical approaches. Endoscopic and Endoscopic Assisted resection techniques have been increasingly popular over the past two decades and have shown to decrease surgical time, post-operative stay as well as intraoperative blood loss. It is our recommendation that future surgeons be adequately exposed and trained in endoscopic resection techniques of angiofibroma, as the authors believe, given the data at hand that this is the way forward.

Juvenile Nasal Angiofibroma on 68Ga-DOTANOC PET/CT: Exploring Theranostic Avenues.

Somatostatin (SST) cell surface receptors (SSTRs) are expressed in many different malignant, benign, and neuroendocrine tumors. SSTRs are also expressed in the endothelium of human vessels during angiogenesis and not in the nonproliferating vessels. We present a case of 19-year-old boy with juvenile nasal angiofibroma (JNA), who underwent 68Ga-DOTANOC PET/CT to explore SSTRs expression and theranostic potential. The scan revealed high uptake in the tumor, and in certain areas, the uptake was similar to that of the pituitary gland. Performance of DOTANOC PET/CT in JNA opens up new frontiers with respect to radiological staging, early recurrence identification, better delineation from postoperative scar tissue, possible preoperative treatment with SST analogs, and perhaps even radiopharmaceutical based-ligand therapy of inoperable/residual/recurrent JNAs in the future.

Clinical Utility of 68Ga-DOTANOC Positron Emission Tomography-Computed Tomography Scan on Postoperative Assessment of Juvenile Nasal Angiofibroma.

Somatostatin cell surface receptors are expressed on the peritumoral vessels of various benign and malignant neoplasms including angiofibromas. Positive initial uptake on positron emission tomography-computed tomography (PET/CT) imaging with 68Ga-labeled DOTANOC is noted in a patient with juvenile nasal angiofibroma (JNA), and the same is noted to be absent following complete surgical excision. Functional 68Ga-DOTANOC PET/CT may be an early useful tool for JNA residual/recurrence identification and precise postoperative surveillance.

Clinical Applications of 68Ga-PSMA PET/CT on Residual Disease Assessment of Juvenile Nasopharyngeal Angiofibroma (JNA).

We present a case of a 16-year-old boy who underwent 68Ga-PSMA PET/CT for residual disease assessment of juvenile nasal angiofibroma. Positive uptake was noted in residual tumor on PET/CT imaging. However, there was no abnormal uptake in surrounding scar tissues as compared with contrast-enhanced magnetic resonance imaging. These findings were confirmed by biopsy from the scar tissue on posterior ethmoids. 68Ga-PSMA PET/CT may be a potentially valuable tool especially in distinguishing recurrences from surgical site reparative tissue and in planning and delivering stereotactic radiotherapy.

Clinical Utility of 68Ga–Prostate-Specific Membrane Antigen PET/CT Scan on Postoperative Assessment of Juvenile Nasal Angiofibroma

Prostate-specific membrane antigen (PSMA) is expressed on the endothelial cells of tumor-associated neovasculature of various nonprostatic benign and malignant neoplasms. Positive uptake on PET/CT imaging with Ga-labeled PSMA is noted in a patient with juvenile nasal angiofibroma, and the same is noted to be absent following complete surgical excision. Ga-PSMA PET/CT may be a useful tool for juvenile nasal angiofibroma recurrence identification and in differentiating recurrence from surgical site reparative tissue.

Prostate-Specific Membrane Antigen Expression in Primary Juvenile Nasal Angiofibroma-A Pilot Study.

Prostate-specific membrane antigen (PSMA) is highly expressed in prostate cancer cells and is exploited for imaging and treatment of patients with prostate cancer. Prostate-specific membrane antigen expression is also demonstrated in the tumor-associated neovasculature endothelium. Juvenile nasal angiofibroma (JNA), being a similar highly vascular tumor, may also demonstrate significant PSMA expression, which may be utilized for its imaging and treatment. In this prospective study, 25 clinicoradiologically diagnosed primary JNA patients underwent PSMA PET/CT scan. The scan was performed after 45 to 60 minutes of intravenous injection of 2 to 3 mCi (74-111 MBq) of Ga-PSMA-HBED-CC on a dedicated PET/CT scanner. Low-dose CT scan was acquired from vertex to sternoclavicular joint (100 mA, 20 kVp, 3-mm slice thickness, 0.8 pitch). Images were reconstructed with iterative reconstruction technique (4 iterations, 24 subsets). The objective was to assess the intensity and pattern of PSMA uptake in primary JNA patients. All cases (n = 25) of primary JNA showed PSMA expression in the tumor (100%). The median PSMA SUVmax ratio of tumor to background was 4.57 (range, 2.08-7.27). Intracranial extension in 14 of 25 patients was prominently visualized because of absence of background uptake in the brain. Advanced stage tumors demonstrated greater uptake than early tumors (P = 0.011). A statistically nonsignificant trend was noted for decreasing uptake with increasing age after normalizing for stage (Spearman correlation coefficient r = -0.08). Assessment of PSMA expression in JNA by PSMA PET/CT opens up a new window of opportunity with respect to its radiological staging, vascularity assessment, and molecular characterization. A potential role in identification of the difficult residual-recurrent disease is anticipated and perhaps also in radioligand therapy for residual/recurrent JNA.Clinical Trials Registry of India (CTRI/2018/08/015479).

Epistaxis in Children: Evaluation and Management

To review treatment options for patients with both ongoing acute epistaxis and chronic recurring epistaxis. While a common occurrence, epistaxis can successfully be treated with adequate first aid, without a medical provider in most cases. Despite its common nature, widespread knowledge of appropriate first aid is lacking. Recurrent episodes may require further testing depending on the severity. Although a minority of patients referred to specialists have significant underlying pathology, there is little guidance in the literature to help inform practitioners regarding which patients warrant referral. While treatment options for idiopathic pediatric recurrent epistaxis is not currently an active area of research, investigators are actively pursuing novel treatments for hereditary hemorrhagic telangiectasia and endoscopic surgery for juvenile nasal angiofibroma (two rare causes of recurrent epistaxis). Epistaxis is a common problem in the pediatric population which can lead to patient and family distress. Knowledge of appropriate treatment pathways is lacking and an area for improvement. Epistaxis that persists despite conventional treatment, or epistaxis in the context of a family history of excessive bleeding or a personal history of other sources of recurrent bleeding, may warrant additional evaluation.

Embryonal rhabdomyosarcoma of the nasopharynx

Embryonal rhabdomyosarcoma has the second highest frequency in the head and neck region. A rare case of embryonal rhabdomyosarcoma in a 9-year-old male child involving nasopharynx with hydrocephalus is reported. The child presented with difficulty in breathing and swallowing with bleeding from nose and mouth. Local examination revealed a granular mass in the left nasal cavity. There was associated proptosis of the left eye. On probing, the mass was friable, nontender and did not bleed on touch. Posterior limit of the mass could not be assessed. Examination of throat showed smooth downward bulge of soft palate on the left side extending into the lateral wall of pharynx behind the posterior pillar of left tonsil. Examination under general anesthesia confirmed the presence and attachment of granular mass to the posterosuperior wall of the nasopharynx and filling the left choana as well. Fibrosarcoma, mucormycosis, nasal polyposis, juvenile nasal angiofibroma, and rhabdomyosarcoma of the nasopharynx were considered in the differential diagnosis. Nasal polyposis was excluded from the differentials after gating computed tomography (CT) scan report was obtained. Histopathology of the excised mass proved the diagnosis of rhabdomyosarcoma and CT scan of the brain showed communicating hydrocephalus. Treatment plan included multi-drug chemotherapy and debulking surgery. The following core competencies are addressed in this article: Medical knowledge, Patient care.

A Comparative Study of Juvenile Nasal Angiofibroma with other Nasal Masses Presenting in a Tertiary Centre of Jharkhand

A Comparative Study of Juvenile Nasal Angiofibroma with other Nasal Masses Presenting in a Tertiary Centre of Jharkhand

Juvenile Nasal Angiofibromas: A Comparison of Modern Staging Systems in an Endoscopic Era

Objectives To compare the clinical utility of four juvenile nasal angiofibroma (JNA) staging systems in a large cohort of patients. Design Retrospective case series. Setting Tertiary referral academic center. Participants Pediatric patients undergoing surgical resection of JNAs between January 2008 and June 2015. Main Outcome Measures Intraoperative blood loss and transfusions, number of staged operations, postoperative residual disease, and recurrent disease. Results In total, 34 patients were identified; all underwent preoperative embolization followed by surgery. Of the 34 patients, 33 (97%) underwent an exclusively endoscopic surgical approach, with 6 (18%) requiring planned staged operations. Ten (29%) patients had residual disease and three (9%) required further surgical resection. Using the area under the curve (AUC) of receiver operating characteristic curves, the University of Pittsburgh Medical Center (UPMC) staging system was most predictive of patients who required staged procedures, received intraoperative transfusions, and had residual postoperative disease (AUC: 0.89, 0.88, 0.86, respectively). Conclusions The UPMC JNA staging system accounts for both route of skull base extension and tumor vascularity, which are two important tumor attributes in the age of preoperative embolization and endoscopic endonasal surgery. The UPMC staging system is a reliable modern staging system that closely reflects prognostic information and aids in surgical planning.

The Role of Radiosurgery in the Management of Benign Head and Neck Tumors

Head and neck tumors are a heterogeneous group and often invade the skull base. Various radiation techniques can be used for these tumors when surgery is unavailable. This study investigated the indications for gamma knife radiosurgery (GKRS) in benign head and neck tumors. Thirty-seven lesions in 35 patients were treated with GKRS for schwannoma, juvenile nasal angiofibroma, choroidal hemangioma, and pleomorphic adenoma. The median follow-up period was 43.0 months (range, 12.2-174.1 months). Tumor control was achieved in 35 of 37 lesions (94.6%) at last follow-up after GKRS. Thirty-four lesions (91.9%) decreased, 1 lesion (2.7%) remained stable, and 2 lesions (5.4%) increased in size. Clinically, 15 cases (40.5%) showed improvement, 17 cases (45.9%) were stable, 1 case (2.7%) experienced deterioration, and 4 cases (10.8%) developed new symptoms. Four of the 5 cases that exhibited deterioration or new symptoms improved. GKRS is a reasonable alternative to surgery that can effectively control tumor growth and preserve functions of the head and neck in primary, residual, or recurrent benign head and neck tumors.

Direct Endoscopic Intratumoral Injection of Onyx for the Preoperative Embolization of a Recurrent Juvenile Nasal Angiofibroma

Percutaneous injection of embolization material within head and neck tumors is being described as an alternative or adjunct to transarterial embolization. Access in these reports is by computed tomography (CT) guidance, which is cumbersome given the need to transport the patient from the CT scanner to angiography suite. We describe a case of direct percutaneous onyx embolization of juvenile nasal angiofibroma following endoscopic access in the angiography suite including self-sustained onyx combustion during surgical electrocautery.