Treatment Of Angiofibromas Research Articles

Analysis of current data on the use of topical mTOR inhibitors in the treatment of facial angiofibromas in tuberous sclerosis complex-An update.

Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous syndrome causing hamartomatous growths in multiple organs. Facial angiofibromas occur in up to 80% of patients and can be highly disfiguring. Treatment for these lesions is challenging. Recently, topical rapamycin has been proposed as an effective option to treat angiofibromas but a commercially available compound has not yet been developed in Europe. We conducted a retrospective review with the aim to update the current data on the use of topical rapamycin in the treatment of angiofibromas in TSC, focusing on the optimal concentration and trying to establish which vehicle should be preferred. Thirty-nine reports describing the use of topical rapamycin in the treatment of angiofibromas in TSC were considered, involving a total of 483 patients. An improvement of the lesions has been shown in over 90% of subjects, particularly if the treatment was started at early stages. Several different formulations (ointment, gel, solution and cream) with a wide range of concentrations (0.003%-1%) were proposed, of which a pharmacological analysis has also been performed. Topical rapamycin can be considered an effective and safe option for the treatment and the prevention of facial angiofibromas in younger patients, but the best formulation has yet to be established. Our review demonstrates that ointment and gel should be preferred, but it is not clear which concentration is optimal. However, according to this study, the 0.1% concentration represents the first choice. Long-term and comparative studies between topical rapamycin formulations are required in order to establish which treatment has a better outcome and lower recurrence rate.

Topical sirolimus for the treatment of facial angiofibromas in tuberous sclerosis patient: a case report

<p class="abstract">Tuberous sclerosis complex (TSC) is an autosomal-dominant neurocutaneous disorder seen in 1/6000 births. Facial angiofibroma is the most common cosmetic disfiguring cutaneous manifestation of it. Therapeutic modalities include cryotherapy, podophyllotoxin application, electrocoagulation, surgery and laser therapy associated with their own high risk of complications and sequelae. Sirolimus is a lipophilic lactone isolated from <em>Streptomyces hygroscopicus</em> (soil bacteria), found to have anti-T-cell properties and classifies as an anticancer drug from the mammalian target of rapamycin (mTOR) inhibitors. Sirolimus binds with mTOR and causes inhibition of mTOR activity and VEGF‑stimulated endothelial cell proliferation. Topical preparation produced by crushing tablets of sirolimus 1 mcg and mixing in an aqueous base emollient or the solution form of sirolimus has been used with beneficial effects in the treatment of angiofibroma, especially in younger patients. Here we reported a case of a 34-year-old male, a known case of TSC with facial angiofibroma in which the topical 0.1% sirolimus had been used with beneficial effects and clinical outcomes.</p><p class="abstract"> </p>

Early Sirolimus Gel Treatment May Diminish Angiofibromas and Prevent Angiofibroma Recurrence in Children With Tuberous Sclerosis Complex.

Introduction: Tuberous sclerosis complex (TSC) is a multisystem neurocutaneous disorder. Angiofibromas (AF), fibrous plaques, and hypopigmented macules are the major skin findings in TSC. Topical sirolimus reduces the volume and redness of AF and other skin findings. However, the efficacy of early intervention and long-term treatment remains to be clarified. We investigated the efficacy of sirolimus gel for AF in children with TSC.Methods: We recruited nine children (five boys; four girls) with TSC and AF. We used 0.2% sirolimus gel over 6 months. We reviewed each patient's medical records and photographs for clinical information and data related to improvements in skin lesions. We evaluated the size of AF, fibrous plaques, and color changes in AF and hypopigmented macules.Results: Age at the initiation of treatment ranged from 3.5 to 11.0 years. The follow-up period ranged from 6 to 36 months (≥24 months in 3 children). Patients presented with papular AF (9), miliary AF (8), AF redness (9), fibrous plaques (5), and hypopigmented macules (2). After 6 months of treatment, improvement of AF size and redness was seen in all nine patients. Patients treated for ≥24 months showed significant decrease in AF size that persisted until the final follow-up. Gradual improvement in fibrous plaques was observed, and marked reduction in size was achieved by 4–18 months.Conclusion: Early sirolimus gel intervention is effective for the treatment of AF and fibrous plaques in children with TSC. Early intervention with sirolimus gel may maintain the skin at near-normal levels in patients with TSC.

Facial angiofibromas of tuberous sclerosis treated with topical sirolimus in an Indian patient.

Facial angiofibromas are the most visible and unsightly of all the cutaneous manifestations of tuberous sclerosis (TSC). A 17-year-old female, a known case of TSC, presented for the treatment of cosmetically disfiguring facial angiofibromas. She was started on twice daily application of 0.1% sirolimus ointment prepared from crushed tablets of sirolimus compounded in white soft paraffin. After 3 months of use, there was visible decrease in the erythema and the size of the angiofibromas. In an attempt to accelerate the response, the concentration was further increased to 1% sirolimus which was used for a month, resulting in a decrease not only in the size and redness but also in the number of the angiofibromas. The patient did not experience any cutaneous or systemic complications related to therapy. Sirolimus belongs to a novel class of anticancer drugs known as mTOR (mammalian target of Rapamycin) inhibitors. Sirolimus has been used as a targeted therapy for the renal and neurological manifestations of TSC. Topical preparation of sirolimus is not commercially available till date and hence preparations from crushed tablets or oral solution of sirolimus have been used with beneficial effects in treatment of angiofibromas especially in younger patients with flatter lesions. Randomized controlled trials are necessary to enable us to confirm the efficacy, long-term safety, the optimal dosage and possibility of reappearance once the drug is withdrawn. This is possibly the first case report of the use of topical sirolimus in India.

Gene Mutation in Tuberous Sclerosis Complex (TSC) and Reversal Treatment of the Mal-formation in Collagens Synthesis: Treatment of Angiofibromas via LLysine Therapy

Angiofibromas are malformation of skin caused by genetic disorders and gene mutation. The purpose of this case study is to find a natural and cost effective solution to overcome the formation and proliferation of angiofibroma structures. At the present time there no simple treatment is available for angiofaibromas and the only available techniques involve the use of carbon dioxide and laser. Besides being costly these techniques need repetitive treatments over a long time and no guarantee of permanent removal of these angiofaibromas. They seem to reform in the same location and in some cases may extend to the surrounding area. The manifestation of these, in the facial area, is in addition to the disfiguring of the face cause health problems as a result of bleeding and possible infection. These angiofaibromas with sizes ranging from granules on the micrometer size to larger ones with diameters on the millimeter scale. They are being reddish to brown, depending on the amount of blood vessels and vacuolated blood. Why are angiofibromas developed around the onset of puberty and accompanying hormonal change? What are the physiological changes that occur at this age? Are they associated with skin and protein synthesis and are conducive to the development of angiofibromas? Because of the fact that these are considered to be genetics the only treatments proposed and performed by medical-doctors only symptomatic treatments. In this paper we propose a treatment of the actual protein synthesis and an enhancement of the production of the proper collagen protein to sharply minimize the formation or may be in some cases totally eradicating the angiofaibromas or in the worst scenario just decrease of blood vacuoles supplement and hence shrinking the fibromas. Results presented here are based on case studies and are very encouraging to perform clinical testing on a larger scale, i.e., a larger group of patients younger than 20 years of age. The limitation of such study is defined under testing patients who do not have any angiofibromas but who are likely to develop angiofibromas. We did not have this kind of sample in the group, which would strengthen the results by providing a baseline on the effectiveness of L-Lysin in inhibiting the initial development of the facial fibromas. In future study this could be compensated for by conducting some tissue culture and monitoring the effect of various amino acids on normal cells vis-a-vis mutated cell lines. This type of research is very crucial for both researchers and consumers as it emphasizes the concept of simplicity in prevention and treatment. Angiofibromas are also caused as side effects of some seizure medications such as phenytoin and the results are devastating for the patients. We hope that maybe some will benefit from this research by following healthy diets with regard to the inclusion of essential amino acids in a healthy diet especially that of L-Lysine.

TREATMENT OF FACIAL ANGIOFIBROMAS OF TUBEROUS SCLEROSIS WİTH RADIOFREQUENCY DESSICATION AND COAGULATION

Tuberous sclerosis is an autosomal dominant neurocutaneous disorder with an incidence of approximately 7/100 000. Inheritance is autosomal dominant, but up to 70% of cases are new mutations. Tuberous sclerosis is mainly characterized by a triad of epilepsy, learning difficulties and skin lesions including angiofibromas, hypopigmented macules, connective tissue naevi and periungual fibromas. Angiofibromas are a common presentation of tuberous sclerosis. They cause considerable cosmetic and hygienic morbidity for patients. Treatments of angiofibromas have included curettage, cryosurgery, chemical peeling, dermabrasion, shave excision, lasers and 13-cis retinoic acid. Traditional methods of treating angiofibromas are not entirely successful as they frequently lead to scarring and pigmentary changes. We report the successful use of radiofrequency dessication and coagulation in treating multiple angiofibromas of four patients. The technique is easy to use and very cost effective. Key words: tuberous sclerosis, angiofibroma, ambulatory surgical procedures. Cite this article : Ulas Guvenc, Anil Gunsel Bahali, Umit Tursen, Tamer Irfan Kaya, Guliz Ikizoglu, Erdinc Terzi. Treatment of facial angiofibromas of tuberous sclerosis with radiofrequency dessication and coagulation. RoJCED 2017;3(4):182-185 https://doi.org/10.26574/rojced.2017.4.3.182

Analysis of current data on the use of topical rapamycin in the treatment of facial angiofibromas in tuberous sclerosis complex.

Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous syndrome causing hamartomatous growths in multiple organs. Facial angiofibromas occur in up to 80% of patients and can be highly disfiguring. Treatment for these lesions has historically been challenging. Recently, topical rapamycin has been proposed as an effective option to treat angiofibromas but a commercially available compound has not yet been developed. The aim of this review is to analyse the current data on the use of topical rapamycin in the treatment of angiofibromas in TSC, focusing on the risk-benefit profile. A retrospective review of the English-language literature was conducted. Sixteen reports describing the use of topical rapamycin in the treatment of angiofibromas in TSC were considered, involving a total of 84 patients. An improvement of the lesions has been shown in 94% of subjects, particularly if the treatment was started at early stages. Several different formulations (ointment, gel, solution and cream) with a wide range of concentrations (0.003%-1%) were proposed. Only 4 local adverse side-effects were reported after the use of rapamycin solution. Topical rapamycin can be considered a safe option for the treatment and the prevention of facial angiofibromas in younger patients, but the best formulation has not been established. Our review demonstrates that ointment and gel should be preferred, but it is not clear which concentration is optimal. Long-term and comparative studies between topical rapamycin and ablative techniques are required to establish which treatment has a better outcome and lower recurrence rate.

Nd:YAG laser for “fractional” treatment of angiofibromas

Tuberous sclerosis is an autosomal dominant disorder with hamartomas in multiple organ systems. Facial angiofibromas (AF) are a common cutaneous manifestation of tuberous sclerosis. They may lead to significant cosmetic and psychological problems. Many treatment modalities have been used with variable satisfaction. Fractional photothermolysis (FP) is a novel concept of laser therapy, which induces non-contiguous microscopic thermal injury. This study was designed to evaluate the efficacy and side effects of Nd:YAG laser treatment for facial AF by using spot-like irradiation technique similar to FP. Twelve patients (skin types III-IV, aged 17-45 years) with facial AF received monthly treatment with Nd:YAG laser in a pinpoint coagulation fashion. Clinical improvements were evaluated in five categories. Side effects were monitored at each follow-up visit. Excellent improvement was seen in 10 patients and good improvement in two at one month after the final treatment session. After 12 months of follow-up, no patient had significant recurrence of AF. Five patients were followed up for three years with few pinpoint-sized regrowth occurring; none of these patients received additional treatments. Side effects included transient erythema and edema in each patient but no bleeding, infection, dyspigmentation, or scarring. The present study indicates that Nd:YAG laser is an effective and safe management for the fractional treatment of facial AF.

Co2/Erbium:YAG/Dye Laser Combination: An Effective and Successful Treatment for Angiofibromas in Tuberous Sclerosis

Tuberous sclerosis, an autosomal dominant neurocutaneous syndrome seen in approximately 1 in 6,000 people worldwide, is characterized by the appearance of hamartomas in multiple organs. The classic clinical triad consists of angiofibromas, epilepsy, and developmental delay. Dermatologic disorder is one of the main characteristics. Angiofibromas, a common form of presentation, causes significant cosmetic and medical problems. The current treatment for skin lesions is laser therapy. The carbon dioxide (CO2) laser has been used satisfactorily in treating these lesions, but several studies have demonstrated a high percentage of recurrences. Erbium:yttrium-aluminum-garnet (YAG) laser treatment has been used to resurface skin abnormalities in patients with dermatologic conditions. The dye laser as an alternative uses the principles of selective photothermolysis and is very effective in treating the vascular component of tuberous sclerosis. The use of all these lasers to treat skin lesions in patients affected by tuberous sclerosis has never been described in the literature. A retrospective study, conducted from 2007 to May 2013, investigated 13 patients who had tuberous sclerosis treated with an erbium:YAG/CO2/dye laser combination. All the patients showed great improvement of their skin lesions. The results were evident immediately after the first treatment. No patient experienced complications or recurrence. The combined use of the erbium:YAG/Dye/CO2 laser is a safe and effective treatment for skin lesions in patients affected by tuberous sclerosis. This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Treatment of Angiofibromas of Tuberous Sclerosis with 5-Aminolevulinic Acid Blue Light Photodynamic Therapy Followed by Immediate Pulsed Dye Laser

Treatment of Angiofibromas of Tuberous Sclerosis with 5-Aminolevulinic Acid Blue Light Photodynamic Therapy Followed by Immediate Pulsed Dye Laser

Endonasal Surgical Treatment of Angiofibroma

Endonasal Surgical Treatment of Angiofibroma

CO2, Er:YAG and Pulsed Dye Laser Treatment of Angiofibromas in Patients with Tuberous Sclerosis

Summary Background: Facial angiofibromas in tuberous sclerosis have been treated using various methods, without reproducible efficiency. Laser therapy could be a viable alternative. Objective: The purpose of this study was to evaluate the efficacy and side-effects of pulsed CO2 laser (10600 nm wavelength) or Erbium-YAG laser (2940 nm wavelength), and 595 nm wavelength pulsed dye laser in the treatment of facial angiofibromas. We attempted to make a comparison between these two treatments, and to perform a clinical assessment of pulsed CO2 laser or Erbium-YAG laser and pulsed dye laser treatment of angiofibromas. Methods: We treated 18 patients. We utilised a 595 nm wavelength pulsed dye laser, using a skin cooling system (Spray or Cryo), with a 7 mm spot size, energy density (fluence J/cm2) from 6 to 8 J/cm2, emission modality (repetition rate) at 0.5 Hz to 2, and a short pulse duration of 2 to 0.5 msec or Erbium-YAG laser (1000 mJ, 200 s); KTP 532 nm; Nd:YAG 1064 long pulse (300 J/cm2, 50 ms); and pulsed CO2 laser. Results: Nine patients with fibrous angiofibromas of the entire face were treated using pulsed CO2 laser (ultra or superpulsed). The patients treated showed remarkable cosmetic improvements, without scarring. Mild hyperpigmentation was observed in 2/9 patients, which disappeared within 6 months. Facial erythema persisted for about 2 months in 5/9 patients. The results on fibrous and protuberant angiofibromas were good, with an improvement of 51–75% of the lesions. Seven patients were satisfied after the first pulsed CO2 laser treatment, and only 2 required 1 further treatment at 6-month intervals (mean number of treatments: 1.22). Er:YAG laser was used for 4 patients, with 1 to 2 sessions at 2-month intervals, with good improvement on the fibrous component, and limited adverse effects (1 patient developed hypochromia). Vascular lasers were used for 11 patients. The pulsed Nd:YAG KTP (532 nm) (3 patients) and Nd:YAG-long pulse 1064 nm (1 patient) was used to times and always required the use of another type of laser. With the use of the Nd:YAG KTP (532 nm) a perinasally localised scar occurred. Conclusions: Laser therapy is indicated for angiofibromas in the first instance. Carbon dioxide laser is advocated as the preferable treatment for fibrous or protuberant angiofibromas, while pulsed dye laser is more successful for those with a vascular component. The CO2 laser, however, involves a risk of depigmentation or scarring, so that a combination of lasers may be required to achieve the best results.

Treatment of angiofibroma with the pulsed tunable dye laser

Angiofibromas occur commonly as solitary le­ sions (fibrous papules) on the face of adults, and they also appear extensively on patients with tuberous sclerosis. Fibrous papules are generally smooth, firm, dome-shaped, and skin colored, but they may have a rim of erythema or be slightly pigmented. Although many authors believe that fibrous papules and angiofibromas are identical, others classify them individually.' Angiofibromasoccurinapproximately 90% ofpatients with tuberous sclerosis and are vari­ ably colored, reflecting the amount ofvascular ecta­ SIa. Angiofibromas contain a variable number of spindle-shaped stellate fibroblasts in the papillary dermis and increased numbers of blood vessels that are frequently dilated. 1 The cause of these angiofi­

Nasopharyngeal angiofibromas: hazards of embolization.

A systematic approach to the diagnosis and treatment of angiofibromas is presented. The distinction between true and false internal carotid supply to these tumors as well as identification of vascular anatomical variants that may represent potential hazards of embolization are emphasized. Areterial supply to the cranial nerves, prevention of neurologic complications, and the use of inappropriate embolization materials are also discussed.