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This report describes a PTCH1 c.1804C > T (p.Arg602*) mutation causing a Chinese nevoid basal cell carcinoma syndrome (NBCCS) with multiple basal cell carcinoma (BCC) phenotype. Multiple modalities including microwave ablation, photodynamic therapy, and excision surgery have a good respond to the NBCCS. The current results broaden the spectrum of PTCH1 mutations responsible for NBCCS., (Copyright © 2020. Published by Elsevier B.V.)

Introduction: Conventional methods of basal cell carcinomas (BCC) treatment bring many severe side effects, especially, if they are repeated many times. The aim of this study is to present the clinical effectiveness of photodynamic method in the treatment and prevention of BCC relapses on the face and to propose a management algorithm., Methods: In a patient with Gorlin-Goltz syndrome (NBCCS) lesions on the face were assessed clinically and with photodynamic diagnostics (PDD), initially and in follow-up every 3 months, for a total of 12 months. Detected BCCs were treated with photodynamic therapy three times every week., Results: In whole follow-up period no clinical relapses were shown. However, in PDD after 6 month in one irradiated and in one initially clinically clear area red fluorescence indicating atypical foci was observed and irradiated additional one time., Discussion: Photodynamic therapy is not limited by previous treatments, can be repeated without adverse events, heals multiple lesions at once and prevents new ones. Because BCC in NBCCS will occur constantly, the implementation of PDD to control the condition of the skin in long-term care should be obligatory. We indicate the validity of using the photodynamic diagnostic and therapy, as a medical procedures of choice., (© 2020 Wiley Periodicals LLC.)

Background: Photodynamic therapy (PDT) is a non-scarring alternative for treating basal cell carcinoma (BCC) in patients with Basal Cell Nevus Syndrome (BCNS), also known as Gorlin syndrome. In Europe, red light (635 nm) is the predominant source for PDT, whereas in the United States blue light (400 nm) is more widely available. The objective of this study was to conduct a head-to-head comparison of blue light and red light PDT in the same BCNS patients., Methods: In a pilot study of three patients with 141 BCC lesions, 5-aminolevulinate (20% solution) was applied to all tumors. After 4 h, half of the tumors were illuminated with blue light and the remainder with red light. To ensure safety while treating this many tumors simultaneously, light doses were escalated gradually. Six treatments were administered in three biweekly sessions over 4 months, with a final evaluation at 6 months. Tumor status was documented with high-resolution photographs. Persistent lesions were biopsied at 6 months., Results: Clearance rates after blue light (98%) were slightly better than after red light (93%), with blue light shown to be statistically non-inferior to red light. Eight suspicious lesions were biopsied, 5 after red light (5/5 were BCC) and 3 after blue light (1 was BCC). Blue light PDT was reportedly less painful., Conclusion: Blue light and red light PDT appear to be equally safe and perhaps equally effective for treating BCC tumors in BCNS patients. Further studies to evaluate long-term clearance after blue light PDT are needed., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Topical photodynamic therapy is an effective and safe noninvasive treatment for low-risk basal cell carcinoma, with the advantage of an excellent cosmetic outcome. Efficacy of photodynamic therapy in basal cell carcinoma is supported by substantial research and clinical trials. In this article, we review the procedure, indications and clinical evidences for the use of photodynamic therapy in the treatment of basal cell carcinoma.

Objective: To report our experience of methylaminolevulinate photodynamic therapy (MAL-PDT) in the treatment of multiple basal cell carcinoma (BCC) in adults and children with Gorlin syndrome (GS)., Design: Report of cases., Setting: University of Montpellier, Department of Dermatology., Patients: Seven Gorlin patients (41 superficial or nodular carcinomas), including two children., Interventions: Prior superficial curettage for superficial BBCs or debulking for nodular BCCs was systematically performed. Methylaminolevulinic acid was applied topically to lesions 3 h before illumination with 635 nm red light for 10 min (37 J/cm(2)). To prevent treatment discomfort, analgesics and/or cooling by sprayed water were most often provided, and occasionally 1% lidocaine local anesthesia. A ropivacaine-lidocaine tumescent anesthesia was performed on the youngest patient., Main Outcome Measures: The initial response rate; tolerance, particularly in children; cosmetic outcome., Results: Overall clearance in patients was 60% after one session of MAL-PDT and 78% after three sessions. Resolution of the lesions was accompanied by an excellent cosmetic outcome in all patients. Treatments were well tolerated in adults with moderate pain sensation during illumination. In a child, tumescent anesthesia assured excellent tolerance in all treatment stages., Conclusion: We add our experience to previous articles that consider PDT as an interesting option in the treatment of GS. To our knowledge, this study is the first report of MAL-PDT in GS children using tumescent anesthesia. Specific guidelines for adult and pediatric patients remain to be established., (© 2012 The Authors. Journal of the European Academy of Dermatology and Venereology © 2012 European Academy of Dermatology and Venereology.)

Methyl aminolevulinate photodynamic therapy (MAL-PDT) is utilized in several countries for the treatment of basal cell carcinoma, but allergic sensitization has been reported by the manufacturer. To the best of our knowledge, we report the first case of urticaria following MAL-PDT in a patient with nevoid basal cell carcinoma syndrome. Prophylactic use of antihistamines may allow continued use of MAL-PDT in this setting.

Gorlin syndrome, also called nevoid basal cell carcinoma syndrome, is well known by dermatologists. Since its onset, 10 years ago, photodynamic therapy has found new applications and is now currently used to cure single or multiple basal cell carcinomas, with good results and without residual scars. We recall some of the basic principles of this technique, as well as its indications in Gorlin syndrome, which we illustrate with one case. Plastic surgeons must consider this relatively new technique, developed by dermatologists, as a useful adjunct to surgery in the management of Gorlin syndrome., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Background: In basal cell nevus syndrome, basal cell carcinomas occur in early life. The treatment of basal cell carcinomas requires surgical excisions and may lead to unaesthetic scars. Photodynamic therapy (PDT) is a validated treatment of skin cancers, with good cosmetic outcomes., Objectives: The aim of the study was to evaluate patient's satisfaction, cosmetic outcome and number of surgical excisions before and after PDT, in patients with basal cell nevus syndrome treated with PDT., Methods: A cross-sectional evaluation of all patients with basal cell nevus syndrome, treated with PDT for basal cell carcinomas. A questionnaire evaluated satisfaction, cosmetic outcomes for surgery and PDT. The number of surgeries before and after PDT was noted and efficacy was evaluated., Results: Seven patients were evaluated; 85% of patients were satisfied with PDT vs. 55% for surgery. The average visual analogue score for the cosmetic result was 8.42/10 for PDT vs. 6.3/10 for surgery. The mean number of surgical excisions was 4.4 during the 6 months before the first session of PDT and 0.57 after., Conclusion: Methylaminolevulinate-photodynamic therapy seems an interesting option for the treatment of basal cell carcinomas in patients with basal cell nevus syndrome., (© 2010 The Authors. Journal of the European Academy of Dermatology and Venereology © 2010 European Academy of Dermatology and Venereology.)

Photodynamic therapy is an effective alternative for the treatment of non-melanoma skin cancer, selectively destroying the neoplastic cells through the use of photosensitizer substances that are irradiated with a source of light of adequate wave length. Nevoid Basal Cell Carcinoma Syndrome is a genodermatosis characterized by multiples basal cell carcinomas occurring at an early age, compelling patients to various surgeries, some of them disfiguring. The aim of this article is to show the excellent result of the treatment of multiple basal cell carcinomas on the scalp of a patient suffering from Nevoid Basal Cell Carcinoma Syndrome.

Nevoid basal cell carcinoma syndrome (NBCCS) is a genetic disorder characterized by multiple basal cell carcinomas (BCCs) in addition to skeletal abnormalities and other neoplasms. Difficulty lies in treating the large number of BCCs that develop in these patients. The authors report a case of a patient with NBCCS treated successfully with photodynamic therapy (PDT) using 20% 5-aminolevulinc acid and blue light. The authors also review the literature and summarize past case reports and series using PDT to treat patient with NBCCS. Based on their experience and the reports of others, the authors assert that PDT should become the first-line therapy in the treatment of multiple BCCs in patients with NBCCS.

Aims: The management of the multiple basal cell carcinomas (BCCs) that develop throughout life of patients with Gorlin syndrome can be challenging. Surgical excision can result in significant disfigurement from scarring and tissue defects. Radiotherapy is contraindicated because of enhanced radiation tumourigenesis in these patients. Photodynamic therapy (PDT) is a simple, repeatable out-patient procedure, which is associated with minimal skin deterioration. It is now routinely used to treat superficial sporadic BCCs, using a topically-applied photosensitiser and external light, but its role in the management of Gorlin syndrome-related BCCs has yet to be established. In particular, Gorlin syndrome is often associated thick, nodular lesions which can be resistant to treatment with topical PDT., Materials and Methods: We report our outcome data for 33 Gorlin patients (138 lesions) treated with PDT. Lesion thicknesses were assessed using ultrasound, both prior to treatment and during follow-up, to quantify treatment response and to guide the choice of treatment methods. Topical PDT was used to treat superficial lesions (<2 mm thick) and a systemic photosensitiser +/- light delivered by interstitially-placed optical fibres was employed for thicker lesions (>2 mm)., Results and Conclusions: Local control rates of 56.3% at 12 months were achieved overall. The use of a systemic photosensitiser +/- interstitial light delivery extended the remit of PDT, allowing thicker lesions (>2 mm) to be treated, resulting in local control rates of 59.3% in this group. PDT can be considered as a treatment option for patients with multiple BCCs as a result of Gorlin syndrome. The use of ultrasound to accurately assess lesion thickness helps to select the optimum treatment method. Systemic photosensitisers and interstitial optical fibres can be used to treat thicker lesions, offering a treatment option for patients with thick nodular tumours who wish to avoid surgery.

Background: The naevoid basal cell carcinoma syndrome (NBCCS) is a therapeutic challenge due to the multiplicity of cutaneous tumours. Photodynamic therapy (PDT) is increasingly used as an alternative treatment for superficial and in some countries nodular basal cell carcinomas (BCC)., Objective: To study the safety and efficiency of PDT in NBCCS., Methods: We reviewed retrospectively the evolution of 62 lesions from patients with multiple BCC treated with PDT., Results: The initial response rate (85.4%, 53/62) and recurrence rate (7.5%) appeared comparable to literature values in NBCCS and to those reported in the treatment of sporadic BCC. The clearance rate without recurrence was 79% (49/62), during a mean follow-up period of 13 months. The cosmetic outcome was excellent. Recurrences were found almost 2 years after treatment., Conclusion: PDT is a suitable therapeutic option in the management of NBCCS patients but requires a strict and long follow-up., (Copyright 2009 S. Karger AG, Basel.)

Background: Gorlin Goltz syndrome is a rare, autosomal dominant inherited disease that is characterised by multiple basal cell carcinomas (BCC) including the periorbital region and eye lids. We report a severe infantile manifestation with lid involvement treated by photodynamic therapy (PDT)., Patient: A 13-year-old boy with Gorlin Goltz syndrome presented with multiple confluent BCC on both eye lids and the skin of neck and trunk. Multiple bilateral periorbital confluent and surgically not removable BCC were treated by topical PDT., Results: Numerous superficial BCC were successfully treated by photodynamic therapy with remarkable cosmetic results., Conclusion: In cases of numerous confluent and surgically not removable BCC, PDT represents an effective therapy. Frequent monitoring is necessary to maintain the clinical outcome.

Topical photodynamic therapy (PDT) is used to treat nonmelanoma skin cancers, such as actinic keratoses, Bowen's disease, and basal cell carcinoma (superficial and nodular). This article presents up-to-date, practical, evidence-based recommendations on the use of topical PDT using 5-aminolevulinic acid or methyl aminolevulinate for the treatment (and prevention) of nonmelanoma skin cancers. A systematic literature review was conducted (using MEDLINE), and recommendations were made on the basis of the quality of evidence for efficacy, safety/tolerability, cosmetic outcome, and patient satisfaction/preference. Topical PDT is highly effective in the treatment of actinic keratoses, Bowen's disease, superficial and thin nodular basal cell carcinomas, with cosmesis typically superior to that achieved with existing standard therapies. PDT may also be a means of preventing certain nonmelanoma skin cancers in immunosuppressed patients.

Nodular basal cell carcinomas resistant to multiple forms of treatment in two patients with nevoid basal cell carcinoma syndrome (Gorlin's syndrome) were treated with systemic porfimer sodium-based photodynamic therapy. Interstitially placed optical diffuser fibers were used to deliver light. Good response to treatment was seen on clinical evaluation and on measurement by a 20-MHz high-resolution ultrasound.

Objective: To report the use of wide-area 5-aminolevulinic acid photodynamic therapy to treat numerous basal cell carcinomas (BCCs) and basaloid follicular hamartomas (BFHs)., Design: Report of cases., Setting: Roswell Park Cancer Institute. Patients Three children with BCCs and BFHs involving 12% to 25% of their body surface areas. Interventions Twenty percent 5-aminolevulinic acid was applied to up to 22% of the body surface for 24 hours under occlusion. A dye laser and a lamp illuminated fields up to 7 cm and 16 cm in diameter, respectively; up to 36 fields were treated per session., Main Outcome Measures: Morbidity, patient response, and light dose-photodynamic therapy response relationship and durability., Results: Morbidity was minimal, with selective phototoxicity and rapid healing. After 4 to 7 sessions, with individual areas receiving 1 to 3 treatments, the patients had 85% to 98% overall clearance and excellent cosmetic outcomes without scarring. For laser treatments, a sigmoidal light dose-response relationship predicted more than 85% initial response rates for light doses 150 J/cm(2) or more. Responses were durable up to 6 years. Conclusion 5-Aminolevulinic acid photodynamic therapy is safe, well tolerated, and effective for extensive areas of diffuse BCCs and BFHs and appears to be the treatment of choice in children.

Background: Patients with nevoid basal cell carcinoma syndrome suffer from multiple basal cell carcinomas, requiring numerous surgical procedures that over time leave them with multiple disfiguring scars. Photodynamic therapy with delta-aminolevulinic acid using red light (approximately 630 nm) sources has been reported as effective in treatment of superficial and small nodular basal cell carcinomas. To our knowledge, the blue light source (417 nm peak irradiance) approved by the FDA for treatment of actinic keratoses has not been used for photodynamic therapy with delta-aminolevulinic acid of basal cell carcinoma., Objective: We report treatment of two nevoid basal cell carcinoma syndrome patients, women aged 21 and 47, with 20%delta-aminolevulinic acid solution and 417-nm blue light source (irradiance 10 mW/cm(2))., Methods: delta-Aminolevulinic acid was applied topically on lesions 1 to 5 hr before light treatment. Lesions were illuminated with 417+/-5-nm blue light for 1000 sec (10 J/cm(2)). Two consecutive treatments 1 week apart were administered as a therapeutic course. Each patient underwent two courses of photodynamic therapy with delta-aminolevulinic acid 2 to 4 months apart. The reported assessment was made 8 months after initial treatment. In most sessions the entire face, rather than visible basal cell carcinomas only, was treated. The treated basal cell carcinomas were clinically subdivided to superficial or nodular type guided by their morphologic features. A total of 9 superficial and 16 nodular basal cell carcinomas on the face and 27 superficial basal cell carcinomas on the lower extremities were treated., Results: Complete clinical response was observed in 8 of 9 (89%) superficial basal cell carcinomas and 5 of 16 (31%) nodular basal cell carcinomas on the face and in 18 of 27 (67%) of superficial basal cell carcinomas on the lower extremities. The remaining 21 lesions showed partial clinical resolution. No new basal cell carcinomas were observed during the 8-month follow-up period in areas treated with a broad application technique. Resolution of the lesions was accompanied by an excellent cosmetic outcome and decreased prominence of old surgical scars in the more severely affected patient. Treatments were well tolerated, but associated with moderate to severe stinging during illumination., Conclusion: To our knowledge this is the first use of photodynamic therapy with delta-aminolevulinic acid with 417-nm blue light for treatment of multiple basal cell carcinomas in patients with nevoid basal cell carcinoma syndrome. Our clinical results demonstrate that the blue light reduces cutaneous tumor burden in such patients. Further studies are needed to confirm that broad-area photodynamic therapy with delta-aminolevulinic acid may eradicate subclinical tumors in nevoid basal cell carcinoma syndrome sufferers, as suggested by a strikingly decreased incidence of new basal cell carcinomas in our patients.

A 26-year-old man with basal cell nevus syndrome presented to the Rocky Mountain Cancer Center (Denver, Colorado) for treatment of several basal cell carcinomas with photodynamic therapy using tin ethyl etiopurpurin (SnET2). The patient was of northern European descent, had type I skin (always burns, never tans), and had a 10-year history of multifocal basal cell carcinomas. The patient had a family history of Gorlin's syndrome (basal cell nevus syndrome); the syndrome had been diagnosed in this patient in 1985. The patient was enrolled in a Phase I/II clinical trial. He was given 1.2 mg/kg (94 mg total) of SnET2 via intravenous infusion; he returned to the clinic the following day for red light application. Thirteen lesions, in 12 treatment fields, were illuminated with light totaling 200 J/cm2 at a fluence of 150 mW/cm2. At the 3-month follow-up examination, all tumors were graded as having a complete response by modified AIDS Clinical Trial Guidelines oncologic standards. No evidence of recurrence has been noted during the 6-month follow-up period.

A 74-year-old woman presenting with multiple unilateral localized basal cell carcinomas present for 30 years on the left side of her body is reported. There was no history of arsenic ingestion or X-ray exposure. Typical signs of nevoid basal-cell carcinoma syndrome (Gorlin's syndrome) like jaw keratocysts, skeletal malformations, and small palmoplantar pits were missing. Although treatment with many therapeutic regimens was tried, there were still tumor recurrences. Photodynamic therapy was performed after topical application of 5-aminolevulinic acid. This treatment resulted in good tumor control with an optimal cosmetic result.

We have embarked upon a pilot study of photoradiation therapy (PRT) in the treatment of persistent or recurrent cancer of the head and neck utilizing the photosensitizing agent hematoporphyrin derivative (HPD). This treatment is based upon selective concentration of HPD within malignant tissue with resultant necrosis upon illumination with light of the appropriate wave length (630 nm). Patients entered in this trial have failed all forms of conventional therapy. Twenty-one patients with local recurrence were treated. Sites of recurrence were: tongue (9), nasopharynx (3), floor of mouth (2), soft palate (2), oropharynx (1), buccal mucosa (1), maxilla (1), larynx (1), basal cell nevus (1). There were six complete responses and twelve partial responses (greater than 50% reduction). These responses are clinically significant with some complete responses lasting over one year after a single course of therapy. Ten patients with cutaneous metastases from head and neck primary tumors were also treated. There were two complete responses and three partial responses. However, these patients rapidly developed new tumors in areas adjacent to those previously treated. Less than complete responses could be augmented by repeated applications of this technique. The success of this pilot study combined with the accessibility of head and neck primaries suggest that HPD-PRT should be given a clinical trial in early mucosal cancer of the head and neck region.