A Systematic Review of Laser Treatment for Angiofibromas in Tuberous Sclerosis.

BACKGROUND: While mammalian target of rapamycin inhibitors have revolutionized the management of angiofibroma in tuberous sclerosis complex (TS), physical modalities such as laser are still indicated for recalcitrant lesions. OBJECTIVE: The authors performed a systematic review of the efficacy and safety of laser treatment for TS-related facial angiofibroma. METHODS: The electronic databases such as MEDLINE, Embase, PubMed, Cochrane Central Register of Controlled Trials, and Web of Science were searched from inception to October 10, 2023, for eligible records. RESULTS: Forty-seven articles met the inclusion criteria, representing a total of 217 patients with TS-related facial angiofibroma who received laser treatment. Several lasers have been trialed in patients including carbon dioxide ( n = 95, 43.7%), pulsed dye ( n = 21, 9.7%), argon ( n = 16, 7.4%), neodymium-doped: yttrium aluminum garnet ( n = 12, 5.5%), copper vapor ( n = 9, 4.1%), potassium titanyl phosphate ( n = 7, 3.2%), erbium: yttrium aluminum garnet ( n = 2, 0.9%), lasers and various combination therapies ( n = 55, 25.3%). CONCLUSION: Potassium titanyl phosphate, pulsed dye, and neodymium-dopsed:yttrium aluminum garnet lasers are better suited to manage the vascular components of angiofibroma while ablative lasers such as erbium: yttrium aluminum garnet and carbon dioxide lasers may present better options for lesions with a prominent fibrous component. While several lasers have been trialed with broadly favorable results, the low level of evidence precludes definitive conclusions, and no single laser appears superior.

Radiotherapy for recurrent juvenile nasopharyngeal angiofibroma.

Juvenile nasopharyngeal angiofibroma (JNA) is a rare paediatric tumour known for its local destructiveness and high recurrence rate. Surgery is the primary treatment modality for JNA, though other options, such as hormonal therapy, embolisation and radiotherapy, exist for inoperable cases. The location of the tumour makes surgical intervention challenging. A 14-year-old male presented with epistaxis and headaches as the chief complaints and was diagnosed with nasopharynx angiofibroma by computed tomography (CT) scan in 2018. Pre-operative embolisation was performed and followed by surgical removal of a 4 cm tumour in January 2019. Pathological examination revealed CD34 positivity, S100 negativity and Ki-67 positivity (5 to 10%), confirming angiofibroma. In October 2019, a 3.6 cm recurrent tumour was treated with embolisation and a second surgery. Pathological findings again confirmed JNA. The patient underwent four surgeries in total, but epistaxis persisted. In 2021, local radiotherapy was administered using intensity-modulated radiation therapy (IMRT) at a dose of 60 Gy in 25 fractions. Serial magnetic resonance imaging (MRI) post-radiotherapy showed a decreasing tumour size, with no further epistaxis and no observed radiation side effects 2 years post-treatment. Radiation therapy remains a strong alternative for managing recurrent JNA.

Preoperative Transarterial Embolization of Advanced Juvenile Nasopharyngeal Angiofibroma Using n-Butyl Cyanoacrylate: Case-Control Comparison with Microspheres.

PURPOSE: To evaluate the efficacy and safety of transarterial embolization (TAE) with n-butyl cyanoacrylate (nBCA) for juvenile nasopharyngeal angiofibroma (JNA). MATERIALS AND METHODS: A retrospective review was performed on patients with JNA who underwent TAE and endoscopic resection between 2020 and 2022. Patients embolized with nBCA were identified, and those embolized with microspheres were set as the control group. Data on demographics, symptoms, tumor characteristics, blood loss, adverse events, residual disease, and recurrence were collected, and case-control analysis was performed for the 2 groups. Differences in characteristics between the groups were tested using the Fisher exact and Wilcoxon tests. A generalized linear model (GLM) was used to analyze the univariate and multivariate influences on blood loss. RESULTS: Twenty patients were included in this study: 13 in the microsphere group and 7 in the nBCA group. The median blood loss was 400 mL (interquartile range [IQR], 200-520 mL) in the nBCA group and 1,000 mL (IQR, 500-1,000 mL) in the microsphere group (P = .028). The GLM confirmed lower blood loss in the nBCA group (relative risk, 0.58 [0.41-0.83]; P = .01). A residual tumor was found in 1 patient in each group (7.7% vs 14.3%; P = 1.000). Recurrence was not observed in any patient. None of the patients experienced adverse events during embolization. CONCLUSIONS: TAE of advanced JNA with nBCA glue is safe and effective and can significantly reduce intraoperative blood loss compared with microspheres.

[Experience of transnazal endoscopic surgery of juvenile angiofibroma of the skull base and nasopharynx without performing preoperative embolization]. / Opyt transnazal'noi endoskopicheskoi khirurgii yuvenil'noi angiofibromy osnovaniya cherepa i nosoglotki bez vypolneniya predoperatsionnoi embolizatsii.

Juvenile nasopharyngeal angiofibroma (JNA) is a hypervascular, blood-supplied, benign tumour affecting the sinuses, nasal cavity, nasopharynx and the base of the skull. For intraoperative hemostasis, pre-operative embolism is a common and recommended procedure, but it has serious disadvantages, such as additional radiation exposure, anesthesia, and the risk of iatrogenic complications associated with the occlusion of the central artery of the retina, orbital and middle cerebral arteries. This article presents a report on successful radical removal of the widespread SAS without resorting to preoperative embolization, but with intraoperative transnazal endoscopic clipping of the internal maxillary artery (IMA).

Efficacy and safety of preoperative internal maxillary arterial embolization with gelfoam for nasopharyngeal angiofibroma.

PURPOSE: To investigate the efficacy and safety of preoperative internal maxillary arterial embolization with gelfoam particles in patients with nasopharyngeal angiofibroma. MATERIALS AND METHODS: We retrospectively reviewed a total of 27 consecutive patients with pathologically confirmed nasopharyngeal angiofibroma from August 2006 to September 2018. Of the 27 enrolled patients, 10 patients received surgical excision alone; 17 patients received preoperative internal maxillary arterial embolization followed by surgical excision. Embolic agents were gelfoam particles. RESULTS: The mean volume of intro-operative blood loss was 385.3 ml in patients with preoperative arterial embolization, which was significantly lower than 1215.0 ml in the patients without preoperative arterial embolization (P < 0.001). The mean surgical time was shorter in patients with preoperative arterial embolization than in the patient without preoperative arterial embolization, but the difference had no statistical significance (205.0 vs 264.5 min, P = 0.064). Neurological complications such as facial palsy or vision loss or hemiplegia were not observed in patients with preoperative arterial embolization. CONCLUSION: Internal maxillary artery embolization with gelfoam particles suffices to provide an effective and safe adjuvant procedure for surgical excision of nasopharyngeal angiofibroma.

[The current trends and the tactical-technical approaches to the management of the neoplastic processes in the base of the skull]. / Sovremennye tendentsii i taktiko-tekhnicheskie podkhody pri opukholevykh protsessakh osnovaniia cherepa.

The neoplastic processes localized in the base of the skull and the surrounding region have until recently been considered as the inoperable condition. Juvenile angiofibroma of the base of the skull or angiofibroma of the base of the skull (ABS) is most frequently encountered in the otorhinolaryngological practice. In terms of the histological picture, ABS is a benign tumour having a very complicated structure. The multi-disciplinary approach and the application of up-to-date medical technologies made it possible to obtain an insight into the deepest-lying areas of the base of the skull. A total of 69 patients presenting with juvenile angiofibroma of the base of the skull were available for the examination in the present study. In all this cases, the presence of the tumour was confirmed by the histological studies. Apart from the routine medical examination, all the patients underwent X-ray investigations including computed tomography with 3D-reconstruction, angiography subtracted VRT-reconstruction, and magnetic resonance tomography with contrast enhancement. Moreover, the trans-maxillary-buccal approach was applied with the use of the endovideoscopic equipment. All the patients were discharged from the clinic in the satisfactory condition. Recurrent tumours were documented only in three patients, the lethal cases were altogether absent. The length of the period of follow-up observations amounted to five and more years. It is concluded that the application of the modern medical technologies in the combination with the 'teamwork' interdisciplinary approach greatly contributes to the successful surgical treatment of the pathological processes localized at the base of the skull and the surrounding region.

State-of-the-art lasers and light treatments for vascular lesions: from red faces to vascular malformations.

Notable milestones in the treatment of vascular lesions have been achieved over the past century. Many cutaneous vascular lesions can be successfully treated with lightbased devices. In this review, we will discuss the treatment of port-wine birthmarks, lymphatic malformations, infantile hemangiomas, rosacea, venous lakes, pyogenic granulomas, cherry angiomas, and angiofibromas using lasers, total reflection amplification of spontaneous emission of radiation, intense pulsed light, and photodynamic therapy. In addition, for several of these diagnoses, we will review medical therapies that can be combined with light-based devices to provide enhanced results.

Adjunctive Treatment in Juvenile Nasopharyngeal Angiofibroma: How Should We Approach Recurrence?

A recent case of advanced, recurrent juvenile nasopharyngeal angiofibroma (JNA) at our institution has highlighted the limited evidence regarding adjunctive treatment. We present the case of a 10-year-old boy who is the first to undergo multiple-staged surgical resections alongside vincristine treatment. We performed a review of the literature analyzing the roles of radiation therapy, cytotoxic drugs, and novel targeted agents in JNA relapse. Small cohort studies suggest radiotherapy and flutamide are the most rational treatment options for residual and recurrent JNA. Our review highlights the need for further research into the management of primary and recurrent JNA.

Time trends in recurrence of juvenile nasopharyngeal angiofibroma: Experience of the past 4 decades.

BACKGROUND: An analysis of time distribution of juvenile nasopharyngeal angiofibroma (JNA) from the last 4 decades is presented. METHODS: Sixty recurrences were analyzed as per actuarial survival. SPSS software was used to generate Kaplan-Meier (KM) curves and time distributions were compared by Log-rank, Breslow and Tarone-Ware test. RESULTS: The overall recurrence rate was 17.59%. Majority underwent open transpalatal approach(es) without embolization. The probability of detecting a recurrence was 95% in first 24months and comparison of KM curves of 4 different time periods was not significant. CONCLUSION: This is the first and largest series to address the time-distribution. The required follow up period is 2years. Our recurrence is just half of the largest series (reported so far) suggesting the superiority of transpalatal techniques. The similarity of curves suggests less likelihood for recent technical advances to influence the recurrence that as per our hypothesis is more likely to reflect tumor biology per se.

Two types of lateral extension in juvenile nasopharyngeal angiofibroma: diagnostic and therapeutic management.

Juvenile nasopharyngeal angiofibroma is a benign, locally aggressive nasopharyngeal tumor. Apart from anterior lateral extension to the pterygopalatine fossa, it may spread laterally posterior to the pterygoid process, showing posterior lateral growth pattern, which is less common and more difficult to identify during surgery. We analyzed the routes of lateral spread, modalities useful in its diagnosis, the incidence of lateral extension and its influence on outcomes of surgical treatment. The records of 37 patients with laterally extending JNA treated at our institution between 1987 and 2011 were retrospectively evaluated. Computed tomography was performed in all patients and magnetic resonance imaging in 17 (46 %) patients. CT and MRI were evaluated to determine routes and extension of JNA lateral spread. Anterior lateral extension to the pterygopalatine fossa occurred in 36 (97 %) patients and further to the infratemporal fossa in 20 (54 %) patients. In 16 (43 %) cases posterior lateral spread was observed: posterior to the pterygoid process and/or between its plates. The recurrence rate was 29.7 % (11/37). The majority of residual lesions was located behind the pterygoid process (7/11). Recurrent disease occurred in 3/21 patients with anterior lateral extension, in 7/15 patients with both types of lateral extensions and in 1 patient with posterior lateral extension. JNA posterior lateral extension may spread behind the pterygoid process or between its plates. The recurrence rate in patients with anterior and/or posterior lateral extension is significantly higher than in patients with anterior lateral extension only. Both CT and MRI allow identification of the anterior and posterior lateral extensions.

Vision Loss due to Central Retinal Artery Occlusion Following Embolization in a Case of a Giant Juvenile Nasopharyngeal Angiofibroma.

Juvenile nasopharyngeal angiofibroma (JNA) is a benign, vascular, and locally aggressive tumor that arises in the nasal cavity, extending into the nasopharynx and often in to the orbit. It may rarely present to the ophthalmologist with proptosis and optic neuropathy. Preoperative embolization of JNA is done before surgical resection. In this communication, the authors report a rare occurrence of ipsilateral central retinal artery occlusion (CRAO) following embolization with polyvinyl alcohol in a 13-year-old boy with right-sided JNA. Retrospective review of the angiograms pointed out to a suspicious communication between the external carotid artery and the ophthalmic vessels. Pre-embolization detailed study of the angiograms is necessary to avoid such devastating complications. Although rare, vision loss is a possible complication arising from embolization of nasopharyngeal and intracranial tumors, and all patients undergoing these procedures should be informed of the risk of visual loss because it has a lasting impact on the quality of life.

Treatment of angiofibromas in tuberous sclerosis complex: the effect of topical rapamycin and concomitant laser therapy.

Facial angiofibromas are the most troublesome cutaneous manifestations of the tuberous sclerosis complex and are difficult to treat. Lasers are most commonly used to treat these skin lesions, but results are disappointing with frequent recurrences. Recently, treatment of facial angiofibromas with topical rapamycin has been reported to yield promising results. We observed the need of laser ablation in addition to topical rapamycin to get best results for the treatment of angiofibromas in 4 cases. The result showed that topical rapamycin ointment was enough when the papules were yet small in size, i.e. less than a few millimeters, but additional laser ablation was needed for large papules approximately larger than 4 mm. Considering the natural course of facial angiofibromas, we believe that topical rapamycin can be best used in childhood patients. In adults, topical rapamycin was useful for treating the still present small papules and for preventing recurrences after laser treatment.

Current options for the treatment of facial angiofibromas.

Facial angiofibromas are hamartomatous growths that are closely associated with tuberous sclerosis complex and, in fact, they constitute one of the main diagnostic criteria for that disease. These lesions composed of blood vessels and fibrous tissue appear on the face at an early age. Since they have important physical and psychological repercussions for patients, several treatment options have been used to remove them or improve their appearance. However, the lack of treatment guidelines prevents us from developing a common protocol for patients with this condition. The present article aims to review the treatments for facial angiofibromas used to date and to propose a new evidence-based treatment protocol.

Challenges and innovations in managing a complex giant bilateral juvenile nasopharyngeal angiofibroma with intracranial extension.

Juvenile nasopharyngeal angiofibroma (JNA) is a highly vascular, benign and locally aggressive tumour that predominantly affects adolescent males. Recognised for its persistence and propensity to recur, patients usually present with gradual development of symptoms such as epistaxis and nasal obstruction over several months to years. Diagnosis typically combines clinical assessments and radiographic studies, often involving preoperative angiography to identify feeder vessels and facilitate embolisation, reducing intraoperative bleeding during surgical interventions. A comprehensive approach to treatment, considering both tumour characteristics and patient well-being, is crucial, particularly when dealing with cases involving intracranial extension. Surgical excision remains the primary treatment for angiofibroma, though radiotherapy is considered for cases with intracranial extension. This case report outlines a case involving a young man in his 20s with a large bilateral JNA extending into the intracranial area. The patient underwent preoperative embolisation followed by surgical resection using a nasofrontomaxillary swing approach with a bifrontal craniotomy window. This alternative approach provided enhanced exposure to address the involvement of the infratemporal fossa, anterior and middle skull base. Postoperatively, residual intracranial tumour was managed with radiation therapy. Over a 2-year follow-up, the patient remains asymptomatic, with a minor postradiation reduction in the intracranial component's size.

A comparison of particulate and onyx embolization in preoperative devascularization of juvenile nasopharyngeal angiofibromas.

INTRODUCTION: Juvenile nasopharyngeal angiofibromas (JNAs) are hypervascular tumors that may benefit from preoperative devascularization to reduce intraoperative blood loss (IBL). The purpose of this study was to compare transarterial particulate embolization (TAPE) with the direct percutaneous embolization (DPE) technique using ethylene vinyl alcohol (Onyx, ev3, Irvine, CA) for the preoperative devascularization of a JNA. METHODS: We retrospectively reviewed 50 consecutive JNA resections since 1995 for which preoperative embolization was either transarterial with particulate material (n = 39) or DPE (n = 11) using only Onyx. The IBL, transfusion requirements, operative time, and length of hospital admission were compared between the two groups. RESULTS: The mean IBL was 1,348.7 ± 932.2 mL particulate group, 569.1 ± 700.7 mL Onyx group (one-tailed Student's t test p = 0.003). The mean unit of packed red blood cells was 1.56 ± 2.01 units particulate group, 0.45 ± 1.04 units Onyx group (p = 0.009). The relationship between embolization type and IBL remained significant or strongly correlated when accounting for the Fisch stage of the tumor (p = 0.010 and p = 0.056, respectively, by a multivariate least squares fit; alternately p = 0.0003 and p = 0.023, respectively, in the subset of patients with Fisch stage III tumors only). We also found that the proportion of resections for which an endoscopic approach could be used was significantly higher in the Onyx group than the particulate group (81.8 and 18.2 %; Pearson p = 0.0002), and this was also significant both in our multivariate nominal logistic fit (p < 0.001) and in the subset of patients with Fisch stage III tumors (p = 0.018). CONCLUSION: Pre-operative DPE with Onyx of a JNA when compared to TAPE significantly decreased IBL and RBC transfusion requirement during surgical resection. The proportion of surgical resections performed from an endoscopic approach was higher in the DPE Onyx group, which may have affected the results.

Targeted topical and combination laser surgery for the treatment of angiofibromas.

BACKGROUND AND OBJECTIVE: The angiofibromas of Tuberous sclerosis (TS) is well described manifestation. Due to the progressive nature of the skin lesion, a safe and effective technique for treating these disfiguring skin lesions is needed. STUDY DESIGN/PATIENTS AND METHODS: We report a targeted topical and combination laser technique for treating the angiofibromas of TS in one patient. This includes treatment with topical sirolimus, pinpoint electrosurgery, pulsed-dye laser treatment, and ablative fractional resurfacing (AFR). RESULTS: Improvement in the number and appearance of facial angiofibromas and erythema is noted, without scarring or adverse events. CONCLUSION: The technique of targeted therapy with sirolimus with electrosurgery, pulsed dye laser treatment, and AFR represents an innovative, safe therapeutic option for treating facial angiofibromas associated with TS.

Nasopharyngeal angiofibroma: a concise classification system and appropriate treatment options.

OBJECTIVES: To describe a clear and simplified classification system for juvenile nasopharyngeal angiofibroma (JNA), and to describe suitable management options. STUDY DESIGN: Retrospective medical record review. METHODS: The 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. RESULTS: Among 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. CONCLUSIONS: 1) 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.

[Embolization of skull-base hypervascular tumors: description of a series of cases and proposal of a therapeutic algorithm]. / Embolizacion de tumores hipervasculares de la base del craneo: descripción de una serie de casos y propuesta de un algoritmo terapéutico.

UNLABELLED: Preoperative embolization allows reducing intraoperative blood loss caused by hypervascular intracranial tumors and its undesirable consequences. AIM: To describe the experience with preoperative endovascular embolization of hypervascular skull base tumors, and to develop a therapeutic algorithm. MATERIALS AND METHODS: A retrospective examination of preoperative neuroimaging and results of preoperative embolization was carried out. RESULTS: Fifteen cases were identified, with a median age of 36 years old, most of them harboring meningiomas, nasopharyngeal angiofibromas or paragangliomas. The external carotid artery was involved in 93% of cases and was the only afferent to 60%. In 27%, there were branches from the internal and external carotid arteries simultaneously. An extensive or complete occlusion grade was achieved in 95% of the branches of the external carotid artery. No branch of the internal carotid artery was treated, because poor contribution to tumor irrigation or was not possible to catheterize the pedicle. There were not recorded procedure-related complications. CONCLUSION: Based on the unification of the experiences described in this study and using data from published series, we present an algorithm for rational selection of skull base tumors that can benefit from preoperative embolization.

Juvenile angiofibroma: major and minor complications of preoperative embolization.

INTRODUCTION: Juvenile angiofibromas (JA) are highly vascular, benign tumours for which surgery is the treatment of choice. In most services, embolisation is performed prior to resection. Nevertheless, there are few data on the complications of preoperative embolisation for JA. AIM: To describe major and minor complications of preoperative embolisation in a 32-year experience of patients undergoing surgical resection of JA at a tertiary hospital. METHODS: Retrospective chart review study of 170 patients who underwent surgical resection of JA at a tertiary hospital between September 1976 and July 2008. RESULTS: All patients were male. Age ranged from 9 to 26 years. Ninety-one patients had no complications after embolisation. Overall, 105 complication events occurred of which four major and 101 minor. CONCLUSION: In our series, preoperative embolisation for JA produced no irreversible complications and no aesthetic or functional sequelae. The vast majority of complications were transient and amenable to clinical management.

Pre-Operative Embolisation of an Aberrant Pharyngeal Arterial Branch Arising From the Internal Carotid Artery and Contributing Significantly to the Supply of a Large Juvenile Nasopharyngeal Angiofibroma.

Preoperative embolisation of juvenile nasopharyngeal angiofibromas (JNAs) is a well-established treatment that reduces intraoperative blood loss and improves surgical outcomes. While the bulk of arterial supply to the tumour is derived from the external carotid system, some degree of contribution from the internal carotid artery (ICA) is common. ICA branch embolisation in this setting has previously been avoided due to concerns over ischaemic neurological complications, possibly contributing to the increased intraoperative blood loss observed in patients with tumours with ICA supply. There is a marked paucity of reports of embolisation of ICA branches supplying JNA in the medical literature. We present a case of successful embolisation of an aberrant pharyngeal branch of the ascending pharyngeal artery arising from the proximal cervical ICA, which was making a significant contribution to tumour blood supply in a male adolescent with a very large JNA.