Number Of Basal Cell Carcinomas Research Articles

A new nonsense mutation of PTCH1 gene in mother and daughter with late-onset nevus basal cell carcinoma syndrome: Case report.

Nevoid basal cell carcinoma syndrome (NBCCS) is a rare clinical disease characterized by a disproportionate number of basal cell carcinoma to sun exposure and skin types. Patched 1 (PTCH1) gene is proposed to be implicated in the pathogenesis of NBCCS. This study aimed to investigate whether PTCH1 gene is the causative gene in Chinese patients with NBCCS. Here we detected the first nonsense mutation in PTCH1 gene by Sanger sequencing of blood samples from a mother and her second daughter (NM000264: exon14: c.2080C>T: p.Q694X). Both of the mother and her second daughter had ovarian mature teratomas. The mother received liquid nitrogen cryotherapy, surgical resection, and radiation therapy, while her second daughter was treated with a GX-III multifunctional ion surgical therapy machine and surgery. New rashes continued to appear and contractures of the right eyelid healed in the mother, while her second daughter had multiple pitting depressions on the palms and soles of both hands and feet. We detected a new mutation in PTCH1 gene in 2 patients with NBCCS, and both of them had ovarian mature teratomas, which are related to NM000264: exon14: c.2080C>T: p.Q694X.

Basal cell carcinoma: Epidemiological impact of clinical versus histopathological diagnosis.

Low-risk basal cell carcinomas (BCCs) are to an increasing extent diagnosed by dermatologists through dermoscopic examination only and treated with non-surgical methods. Reports on increasing incidence as well as trends regarding subtypes, anatomical sites and differences related to gender are based solely on histopathologically verified tumours. How unreported clinically diagnosed BCCs affect the epidemiological data has not been sufficiently investigated. To analyse the tumour and patient characteristics of clinically diagnosed versus histopathologically confirmed primary BCCs and to make a gross estimate on how unreported BCCs could influence the total number of new cases. We retrospectively reviewed all primary BCCs diagnosed in 2016 at the Department of Dermatology, Sahlgrenska University Hospital in Gothenburg, Sweden. We also reviewed all histopathologically verified primary BCCs at the two largest pathology laboratories in Western Sweden during the same year to estimate the proportion of BCCs diagnosed by dermatologists. In total, 2365 primary BCCs were diagnosed at our centre. More than half of these tumours were clinically diagnosed (55.8%). Superficial subtype (41.7%), location on the trunk (46.3%) and destructive treatment methods (60.0%) were most common. The reports from the two pathology laboratories showed that histopathologically verified BCCs (n=5837) were more commonly of the infiltrative or nodular subtype and located in the head and neck area. Dermatologists managed 56.0% of them. This study indicates that a substantial number of BCCs are not visualized in the official statistics which are solely based on reports from pathology laboratories. When taking clinically diagnosed tumours into account, truncal location and superficial subtype are more common than previously believed. Further, based on the regional calculations, the real burden of BCC in Sweden might be up to 70% higher than what is reported in official statistics.

546 A Longitudinal Case History of Skin Cancer Presentations from One of the UK's Longest Surviving Renal Transplant Patients

Abstract Introduction The immunosuppression required by solid-organ transplant patients not only increases their risk of severe infections but also pre-disposes them to post-transplant malignancy, with skin cancers being the most frequent type. We present a longitudinal history of skin cancers in a patient who received their kidney transplant as a child and has been on immunosuppression for 50 years. Case Report The patient developed renal failure at 12 years of age and received a living donor renal transplant in 1970. He developed his first skin cancer in 2004, 34 years later. To date he has had a total of 173 lesions excised of which 123 were a non-melanoma skin cancer (NMSC) (108 basal cell carcinomas (BCCs) and 15 squamous cell carcinomas (SCCs)). None of these lesions had any high-severity features such as poor differentiation or lymphovascular invasion. Numbers of BCCs have not varied, but numbers of SCCs, Bowen's disease and Actinic Keratoses have risen gradually over time. Discussion The patient developed his first skin cancer later than has typically been reported in transplant patients and has had a ratio of BCCs to SCCs more similar to the non-transplant population. Conclusion Whilst the patient has had a large number of NMSCs over time, a recognised association for those taking immunosuppressive medications, the types and nature of his lesions mirrored that of the non-transplant population, which could relate to the fact he received his transplant as a child.

Update on Hedgehog Pathway Inhibitor Therapy for Patients with Basal Cell Naevus Syndrome or High-frequency Basal Cell Carcinoma.

Some patients with basal cell carcinoma develop a large number of basal cell carcinomas during their lives. The most common underlying genetic disease that causes multiple basal cell carcinomas is basal cell naevus syndrome. Basal cell naevus syndrome is caused by a germline mutation in patched-1 (PTCH1), a tumour suppressor gene of the hedgehog signalling pathway. However, in a significant portion of patients with multiple basal cell carcinomas, no underlying genetic cause is found. Nevertheless, these patients can experience a treatment burden comparable to that of patients with basal cell naevus syndrome. They are referred to as high-frequency basal cell carcinoma patients. Hedgehog pathway inhibitors were the first group of targeted therapy for basal cell carcinomas. This study reviews the literature on hedgehog pathway inhibitor therapy for patients with basal cell naevus syndrome and high-frequency basal cell carcinoma, to provide an overview on efficacy, safety, dosing regimens, tumour resistance and reoccurrence, and health-related quality of life.

Abstract 716: Silibinin: A novel potential therapeutic agent against UVB-induced basal cell carcinoma

Abstract Herein, we report silibinin (a natural flavonolignan from milk thistle seeds) efficacy against basal cell carcinoma (BCC), the major non-melanoma skin cancer. To determine the preventive/therapeutic activity of silibinin on the progression of UVB-induced microscopic BCC lesions to more advanced BCC, both male and female 8 weeks old Ptch1+/- mice were irradiated with 240 mJ/cm2 UVB dose 3 times/week (M, W, F) for 26 weeks, and thereafter, UVB irradiation was stopped. At this point (after initial UVB exposure for 26 weeks was stopped), these Ptch1+/- mice were randomized into 3 groups: Baseline group (mice euthanized for baseline data); Vehicle group: mice treated topically with acetone for 20 more weeks, and Silibinin group: mice treated topically with silibinin (9 mg in 200 µL acetone), once a day for five days/week for 20 more weeks i.e., till 54 weeks of mice age. Assessment of BCC and non-BCC lesion pathology was performed following β-galactosidase and H&E based histopathological analysis. Results indicated that compared to BCC-associated pathologies observed at baseline, acetone exposure for another 20 weeks resulted in a significant increase in the number (~2 folds, P<0.001) and area (~3.4 folds, P<0.001) covered by BCC lesions. There was also a significant increase in epidermal dysplasia, fibrosarcoma, and squamous cell carcinoma incidence. Notably, topical application of silibinin during this phase significantly decreased BCC numbers as well as area covered by BCC lesions by ~76% (P<0.001) and ~88% (P<0.001), respectively. This protective effect of silibinin was associated with decreased proliferation of basal cells and decreased expression of Hh signaling molecules (Smo and Gli1). To further delineate the changes associated with silibinin’ s protective effect at the transcriptomic level, RNA sequencing studies were performed in skin samples from all three groups as well as non-UVB exposed control mice. Clustering of mRNA profile by sparse Partial Least Squares - Discriminant Analysis (sPLS-DA) showed that UVB exposed tissues (not treated with silibinin) were significantly different from non-UVB controls; most notably, silibinin treatment (after UVB exposure) reversed this phenomenon and the transcriptomic profile of silibinin group was almost similar to non-UVB controls. This indicated that topical silibinin was not only able to protect against progression to advanced BCC but had the potential to even normalize the aberrant gene expression driving BCC formation. In addition, Gene ontology enrichment analysis as well as Pathway enrichment analysis of differentially expressed genes showed silibinin-associated enrichment in the calcium and CX3CR1-mediated signaling pathway, and TGF-β-mediated regulation of the extracellular matrix. Taken together, these results highlight the potential of silibinin to be an effective preventive and/or therapeutic modality against BCC growth and progression. Citation Format: Komal Raina, Sandeep Paudel, Neha Mishra, Sushil Kumar, David J. Orlicky, Zhiying You, Rama Kant, Chapla Agarwal, Rajesh Agarwal. Silibinin: A novel potential therapeutic agent against UVB-induced basal cell carcinoma [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2022; 2022 Apr 8-13. Philadelphia (PA): AACR; Cancer Res 2022;82(12_Suppl):Abstract nr 716.

Antidiabetic Treatment in Patients at High Risk for a Subsequent Keratinocyte Carcinoma.

Metformin and sulfonylureas are the most commonly prescribed drugs used for the treatment of type II diabetes. Type II diabetes has been linked to the development of keratinocyte carcinoma (KC), consisting of basal cell carcinoma (BCC) and squamous cell carcinoma (SCC). Previously we have demonstrated lower risk for a subsequent KC in metformin users. In this study, we aim to investigate the association between sulfonylureas use and the development of KC in patients with KC history. We performed a retrospective cohort study of the Veterans Affairs Keratinocyte Carcinoma Chemoprevention Trial, which was a randomized double-blind vehicle-control cream originally investigating the effect of 5-fluorouracil on KC development. 932 patients with a history of KC were enrolled (98% male, 99% white, median age of 70 years) and followed for a median duration of 2.8 years. 153 patients were on metformin and 94 on sulfonylureas. We performed a survival analysis with cox regression and controlled for body mass index and known predictors: number of prior BCCs and age (for BCC) and for number of prior SCCs (invasive and in situ), number of actinic keratoses at baseline (for SCC). Sulfonylurea-users com-pared to non-users had a HR of 0.67 (CI: 0.40–1.56; P=0.49) and 0.94 (CI: 0.63–1.40; P= 0.77), for SCC and BCC, respectively. Diabetic patients at high risk for KC might benefit from the use of metformin versus sulfonylureas. J Drugs Dermatol. 2022;21(5):502-505. doi:10.36849/JDD.6087.

COVID-19 Pandemisinin Nonmelanom Deri Kanserleri Tanısı Üzerindeki Etkisi: Retrospektif Vaka Kontrol Çalışması

Objective: The coronavirus disease-2019 (COVID-19) pandemic has caused delays in the diagnosis and treatment of some oncological diseases. This study aimed to reveal the effect of the COVID-19 pandemic on the frequency of diagnosis of non-melanoma skin cancers (NMSC) and the demographic characteristics of patients. Material and Methods: Patients aged over 18 years, who were histopathologically diagnosed with basal cell carcinoma (BCC) or squamous cell carcinoma (SCC) between March 12, 2019 and March 11, 2021 were included in the study. The demographic characteristics of these patients, the number of total skin biopsies, and the number of BCC and SCC biopsies were compared between the pre-pandemic and pandemic periods, based on the date of March 12, 2020, when the pandemic measures began to be implemented in Turkey. Results: With the pandemic, there was a 26.7% decrease in the total skin biopsies and a 21% decrease in the total NMSC biopsies. Despite a 34% decrease in the number of biopsies for the diagnosis of BCC, a 15.8% increase was observed in the number of biopsies for the diagnosis of SCC. The frequency of excisional biopsies was observed to increase among the patients with BCC and SCC during the pandemic period (p=0.004 and p=0.001, respectively). Conclusion: The number of total skin biopsies and total NMSC biopsies significantly decreased during the pandemic period. The health system should be reorganized to effectively handle pandemics and similar crisis periods, and new strategies should be determined regarding patient education, early diagnosis, and treatment approaches for NMSC.

Histomorphological Pattern of Malignant Skin Tumors – A Cross-sectional Study in a Teaching Hospital

Introduction: The incidence of skin cancer is increasing exponentially around the world. This study aims to analyze different malignant skin tumors concerning age pattern, gender, and site-wise distribution.
 Materials and Methods: This is a descriptive cross-sectional hospital-based study carried out in the Department of Pathology, a tertiary care center for over ten years from April 2011 to March 2021. All malignant skin tumors arising from the epidermis along with melanocytic and adnexal tumors were included in the study.
 Results: A total of 208 cases of skin malignancies among which 117 (56.3%) cases were males and 91 (43.8%) females with a male to female ratio of 1.3:1. Overall, the majority were seen in the sixth decade 49 (23.6%) with head and neck region 113 (54.3%) being the commonest site of involvement. Basal cell carcinoma 79 (38%) was the most frequent non-melanoma skin cancer followed by squamous cell carcinoma 75 (36.1%). A maximum number of basal cell carcinoma were observed in the sixth decade 19 (24.1%) whereas squamous cell carcinoma in the seventh decade 21 (28%) cases. Other skin cancers were malignant melanoma 31 (14.9%), verrucous carcinoma 13 (6.3%), trichilemmal carcinoma five (2.4%), sebaceous carcinoma two (1%), and one case each of eccrine carcinoma, malignant nodular hidradenoma, and malignant proliferating trichilemmal tumor.
 Conclusions: Skin malignancies were seen in the sixth decade with male preponderance. Overall, the head and neck region was the commonest site of involvement. Basal cell carcinoma was the most frequent non-melanoma skin cancer followed by squamous cell carcinoma.

Clinical and genetic profiling of nevoid basal cell carcinoma syndrome in Korean patients by whole-exome sequencing

Nevoid basal cell carcinoma syndrome (NBCCS) is mainly characterised by multiple basal cell carcinomas (BCCs) caused by PTCH1, PTCH2, and SUFU. However, clinical and genetic data on Asian NBCCS patients are limited. We aimed to analyse the clinical phenotypes and genetic spectrum of Korean patients with NBCCS. Fifteen patients with NBCCS at Seoul National University Hospital were included, and their clinical data were analysed. Whole-exome sequencing and/or multiplex ligation-dependent probe amplification using peripheral blood were performed to identify genetic causes. Genetic analysis revealed that 73.3% (11/15) of the patients carried 9 pathogenic variants, only in the PTCH1 gene. Variants of uncertain significance (VUS) and likely benign were also detected in 2 (13.3%) and 2 (13.3%) patients, respectively. BCCs were found in the majority of the cases (93.3%) and the number of BCCs increased with age (ρ = 0.595, P = 0.019). This study revealed that PTCH1 pathogenic variants were the main cause of NBCCS in Korean patients. As BCCs are commonly detected, a periodic dermatologic examination is recommended. Finally, our results support the addition of genetic screening to the existing criteria for NBCCS diagnosis.

Methyl aminolevulinate photodynamic therapy after partial debulking in the treatment of superficial and nodular basal cell carcinoma: 3-years follow-up.

The increase in the number of basal cell carcinoma (BCC) lesions has prompted use of minimally invasive therapies, including Photodynamic therapy (PDT). . The objective of the present work was to analyze the efficacy of methyl aminolevulinate-mediated photodynamic therapy (MAL-PDT) in patients suffering from superficial or nodular BCCs. A total of 220 BCC lesions (76 superficial and 144 nodular), clinically diagnosed and confirmed by histopathology analysis, were treated in 174 patients (mean age 72.5). Debulking using curettage was performed before two or three MAL-PDT sessions (λ = 630 nm; 90 J/cm2; 23 min) at 4-week intervals. Analyses of clinical clearance and cosmetic outcome were carried out by direct examination, dermoscopy, photographs, as well as by fluorescence diagnosis using a Wood's lamp. Evaluations were carried out at the different PDT sessions and follow-ups over a 3-year period. MAL-PDT was safe and highly tolerated. After an average of 2.6 MAL-PDT sessions, the overall clearance rate at 3-year follow-up was 96.1 % (95 % confidence interval [CI] 100 %-92 %) for superficial BCCs and 95.2 % (95 % [CI] 99 %-92 %) for nodular BCCs after an average of 2.7 sessions. Minimal side effects such as crushing, erythema and edema were reported. All BCC lesions showed excellent or good cosmetic results. The protocol followed in the present study has shown that MAL-PDT is a safe and effective treatment for superficial and nodular types of BCC.

Histopathological Spectrum of Cutaneous Basal Cell Carcinoma- An 11 Year Retrospective Study in a Tertiary Care Hospital in Southern Karnataka

Introduction: Basal Cell Carcinoma (BCC) is a slow growing, locally invasive, malignant skin tumour with increasing incidence in recent decades. Various histological subtypes of BCC have been described which include nodular, superficial, adenoid, keratotic, basosquamous, and morpheiform. Aim: To analyse the clinical data of patients with BCC and the histomorphological spectrum of BCCs in a population of Southern Karnataka. Materials and Methods: This was an 11 year retrospective descriptive observational study of all histologically confirmed BCCs diagnosed in the Department of Pathology in a tertiary care centre in Southern Karnataka from January 2010 to January 2021. Detailed clinical data of 64 patients including age, gender, clinical diagnosis and anatomic location were analysed. Results: The maximum number of BCCs occurred in the sixth decade with a slight female preponderance. Head and neck lesions were the most common and uncommon sites noted included the vulva and axilla. Most cases presented as an irregular plaque followed by presentation as an ulcerative lesion. Pigmentation was noted in 18 (28%) cases clinically. Majority of patients (63/64 or 98.4%) had a single lesion. Histological types included nodular, superficial, adenoid, basosquamous and BCC with sebaceous differentiation. Nodular BCC was the most commonly encountered type in our setting, followed by superficial BCC. Only one case of basosquamous carcinoma showed evidence of metastasis to lymph nodes. Conclusion: Histological evaluation of BCC is of paramount importance not only to establish the diagnosis but also to predict behaviour and risk of recurrence. In addition to the diagnosis, pathologist should also describe subtypes of the tumour which has a prognostic implication. This study reveals the morphological spectrum of BCC in the population of southern Karnataka and reveals significant patterns in anatomical distribution of BCC. It also highlights a significant percentage of BCCs presenting as pigmented lesions in the Indian population.

Expression of Glioma-associated oncogene homolog 1 as biomarker with sonidegib in advanced basal cell carcinoma.

The pivotal BOLT (Basal cell carcinoma Outcomes with LDE225 [sonidegib] Treatment) study established the durable efficacy and manageable toxicity of sonidegib 200 mg once daily (QD) through 42 months in patients with advanced basal cell carcinoma (BCC). This secondary analysis used expression of Glioma-associated oncogene homolog 1 (GLI1) as a biomarker to assess the extent of Hedgehog pathway inhibition by sonidegib in patients with locally advanced BCC (laBCC) and metastatic BCC (mBCC). The study enrolled 230 patients, 79 and 151 receiving sonidegib 200 and 800 mg QD, respectively. At week 17, GLI1 expression was reduced from baseline by a median percentage (95% confidence interval) of 88.7% (54.6%–93.0%) and 97.0% (77.5%–98.9%) for aggressive laBCC, 97.5% (80.3%–98.8%) and 95.0% (80.7%–97.5%) for nonaggressive laBCC, and 99.1% (96.4%–99.6%) and 99.3% (95.9%–99.9%) for mBCC in the 200 and 800 mg groups, respectively. Substantial repression of GLI1 was observed in patient subgroups stratified by age, sex, BCC cytological subtype, Eastern Cooperative Oncology Group performance status, lesion site, baseline number of BCCs, and prior radiotherapy. Results support further studies on the inhibition of Hedgehog pathway genes by sonidegib in patients with laBCC and mBCC.

388 Natural history and management of basal cell nevus syndrome: Updates from the gorlin syndrome registry

Background: Patients with basal cell nevus syndrome (BCNS) are at increased risk of developing basal cell carcinomas (BCCs). Long-term data on tumor burden, co-morbidities, and management of BCNS is limited. Method: A prospective, cross-sectional study of self-reported questionnaire responses collected from BCNS patients from Feb 2012 to Oct 2016 through the national Gorlin Syndrome Registry. BCC burden was characterized based on frequency and anatomic distribution. Logistic regression analysis was performed to determine the association of BCC development with risk factors such as sex, family history, age of diagnosis/symptoms, and sun exposure. Treatment of BCCs and other co-morbid tumors are additionally characterized. Results: 87 BCNS participants (current age: 39.8 ± 20.0 years; age at diagnosis: 16.5 ± 12.4 years) reported a median of 172 BCCs (range 1 – 1715 BCCs) developing over their lifetime. The number of lifetime BCCs significantly associated with family history of BCNS (p = 0.02) and age (Lifetime BCCs = 5.4*Age, p < 0.0001). A median of 100 BCCs presented on the head, 56 BCCs on the trunk and extremities, and 10 BCCs on the breast and groin. Of the 27/87 (31%) participants with locally advanced or metastatic BCCs, roughly half (13/27) had tried a hedgehog inhibitor such as vismodegib, sonidegib, or itraconazole. Participants with BCNS are found to have an increased prevalence of tumors of the skin (KCOT, actinic keratosis, SCC, melanoma), brain (meningioma, medulloblastoma), and ovaries (fibroma, cyst). Conclusion: The results of this study demonstrate the high burden of BCCs among patients with BCNS. BCCs predominantly developed on sun-exposed area and strongly correlated with increased age. Additional interventions to prevent and treat BCCs are needed. This study establishes a clinical baseline for emerging therapies such as hedgehog inhibitors in the BCNS population.

It's time for Mohs: Micrographic surgery for the treatment of high-risk basal cell carcinomas of the head and neck regions.

Basal cell carcinoma (BCC) is the most common variety of non-melanoma skin cancer and its incidence is increasing worldwide. The centrofacial sites (area H) are considered a high-risk factor for BCC local recurrence. Mohs micrographic surgery (MMS) is a technique that allows intraoperative microscopic control of the surgical margins and is a good treatment option when tissue conservation is required for esthetic or functional reasons or for high-risk lesions. The present study aimed to evaluate the recurrence rate of head and neck high-risk BCCs comparing MMS vs conventional surgical excision. Clinical data of patients diagnosed from September 2014 to March 2017, referring to the Dermatology Unit of the Policlinico Sant'Orsola-Malpighi, University of Bologna, were retrospectively evaluated (285 treated with MMS and 378 treated with traditional surgery). Of the 285 patients treated with MMS, 9 experienced a recurrence (3.1%). Of the 378 patients treated with traditional surgery, 53 relapsed (14%), 13 of whom presented residual tumor on the deep or lateral margins of the main surgical specimen. Our study confirms the trend reported in the literature that MMS represents the best treatment option for high-risk BCCs arising in the head and neck region or presenting as a recurrence (P < .00001). Many more MMS centers and more trained dermatologists are needed worldwide in order to deal with the increasing number of BCC diagnosed every year.

Efficacy and Safety of Sonidegib in Adult Patients with Nevoid Basal Cell Carcinoma Syndrome (Gorlin Syndrome): Results from a Phase 2, Double-Blind, Randomized Trial.

Nevoid basal cell carcinoma syndrome (NBCCS), or Gorlin syndrome, is a rare hereditary disease characterized by the development of multiple cutaneous basal cell carcinomas (BCCs) from a young age.1 Loss-of-function germline mutations in the hedgehog-related patched 1 (PTCH1) tumor suppressor gene are the most common cause of NBCCS.1 The hedgehog signaling pathway plays a major role in embryonic development, and in adulthood, is involved in the renewal and maintenance of distinct tissues, including hair follicles, muscle stem cells, and gastric epithelium.2 Its abnormal activation is thought to drive the formation of both sporadic BCCs and those resulting from NBCCS.1 Patients with NBCCS inherit one inactive copy of PTCH1 and then acquire a “second-hit” mutation, resulting in hedgehog pathway activation and BCC formation.1 Mutations in Suppressor of fused (SUFU) or the PTCH1 homolog PTCH2 have also been found in a subset of patients meeting criteria for NBCCS.1,3 Treatment of BCCs in patients with NBCCS can be extremely difficult due to the large number of tumors. Patients with a limited number of BCCs may benefit from surgical excision.3 However, patients with aggressive or recurrent tumors, as well as tumors in delicate or high-risk areas, may benefit from Mohs micrographic surgery, which is lengthy and costly.3 Multiple surgical procedures can be a source of discomfort, pain, and disfigurement for patients with NBCCS, leading to an unmet need for nonsurgical and minimally invasive treatment options. Sonidegib (Odomzo®; Sun Pharmaceutical Industries, Inc.; Cranbury, NJ), a hedgehog pathway inhibitor (HPI), is approved for the treatment of adult patients with locally advanced BCC (laBCC) that has recurred following surgery or radiation therapy, or those who are not candidates for surgery or radiation therapy.4 Approval was based on results from the BOLT (BCC outcomes with LDE225 [sonidegib] treatment) study.5,6 Here we report the results of an exploratory study evaluating the safety, preliminary efficacy, and pharmacokinetics of sonidegib in patients with NBCCS after 12 weeks of treatment.

Trends of basal cell carcinoma at the Centre of Dermatovenereology of Vilnius University.

Basal cell carcinoma is the most common locally invasive malignant epidermal neoplasm in humans and its incidence has increased over the last decades worldwide, especially among the Caucasian population. Basal cell carcinoma accounts for about 75% of all skin cancers. Incidence data on basal cell carcinoma is sparse because traditional cancer registries often do not register these tumours. In Lithuania, patients with skin cancer and melanoma were traditionally treated in centralized oncological institutes. From 2006, the Centre of Dermatovenereology at Vilnius University Hospital Santaros Klinikos (Vilnius, Lithuania) provides modern diagnostic and treatment facilities to oncodermatological patients. The objective of the study was to evaluate epidemiological and clinical data of basal cell carcinoma at the Centre of Dermatovenereology during the last 15 years. Medical documentation of the cases of histologically-proven basal cell carcinoma diagnosed between 2000 and 2015 was analyzed. Epidemiological and clinical evaluation according to the patients' age, sex, and place of residence, as well as tumour localization, its histological type, and treatment options was performed. After the skin lesion biopsy and histopathological examination, a total of 847 basal cell carcinomas were confirmed to 782 patients. During the study period, the total annual number of newly diagnosed basal cell carcinomas rose steadily in our centre: 2.7% between 2000 and 2003, 6.5% between 2004 and 2006, 13.6% between 2007 and 2009, 27.6% between 2010 and 2012, and 49.6% between 2013 and 2015. The biggest part of patients (28.4%) were 70-79 years old, 4.6% - younger than 40, 7.3% - 40-49, 17.1% - 50-59, 27.2% - 60-69, 14.1% - 80-89, and 1.3% ≥90 years old. The average patient age was 66.0 (±13.6). Of these patients, 62.0% were female and 38.0% male; 63.6% were from the capital city, 18.3% from other cities, and 18.2% from rural areas. Basal cell carcinomas occurred most often in the face region 49.0%, followed by the trunk - 29.4%, the scalp and neck - 10.9%, arms and legs - 7.7%, in 2.9% location was not specified and the whole body - 0.1%. The predominant histological type of basal cell carcinomas was nodular (60.6%), other diagnosed types were superficial (22.9%), infiltrative/morpheaform (8.0%), mixed nodular and infiltrative (1.7%), pigmented (0.2%), rare types (micronodular, infundibulocystic, ductal and mixed) - 0.6%; the type was not specified in 6.0% of cases. Nodular, superficial, and infiltrative types were the most common morphological types in all body sites: respectively, in the face - 67.5%, 12.5%, 9.4%; in the scalp and neck region - 77.2%, 14.1%, 5.4%; in the trunk - 49.8%, 37.3%, 7.2%; in extremities - 41.5%, 43.1%, 7.7%. The nodular type was more common among the elderly and its incidence increased with age (p = 0.009), meanwhile, superficial basal cell carcinomas prevailed among younger patients (<40 years), and its incidence decreased with age (p < 0.001). Also, the nodular type was usually found in the areas of the face (p < 0.001) and the scalp and neck (p = 0.045), and the superficial type - in the areas of the trunk (p < 0.001) and extremities, specifically in the hand (p = 0.022). Basal cell carcinomas were mostly treated with surgical excision (79.6%), photodynamic therapy (5.5%; in our centre, this treatment option is available from 2007), and other/combined methods (14.9%). The results showed that the number of newly diagnosed basal cell carcinomas increased continuously between 2000 and 2015. Basal cell carcinomas in our centre occurred most often among the patients aged 70-79 years. This tumour was more often diagnosed in female than male patients. Most of basal cell carcinomas were located on the chronically sun-exposed skin, such as the face region, and were of the nodular histological type, which is more common among the elderly, while the superficial type is more prevalent among younger patients. The most commonly used treatment option for basal cell carcinoma was surgical excision. Acknowledgment of the tendency of the rising number of basal cell carcinomas, healthcare resources, highlights the need for an effective skin cancer prevention strategy in Lithuania.

High-risk basal cell carcinoma excision in primary care: a retrospective observational study of compliance with NICE guidance

ObjectivesTo assess compliance with 2010 National Institute for Health and Care Excellence (NICE) guidance on cancer services relating to the management of basal cell carcinomas (BCC) in the community, where...

Gorlin Syndrome

Gorlin syndrome is a rare autosomal dominant disease caused by mutations in the sonic hedgehog signaling pathway. Of particular importance is the PTCH1 gene. The disease is characterized by the development of multiple basal cell carcinomas at young ages. These tumors may present with other skin manifestations such as palmoplantar pits and with extracutaneous manifestations such as odontogenic keratocysts and medulloblastoma. Although the dermatologist may be key for recognizing clinical suspicion of the syndrome, a multidisciplinary team is usually necessary for diagnosis, treatment, and follow-up. Skin treatment may be complicated due to the large number of basal cell carcinomas and the extent of involvement. In recent years, new drugs that inhibit targets in the sonic hedgehog pathway have been developed. Although these agents appear promising options for patients with Gorlin syndrome, their efficacy is limited by adverse effects and the development of resistance.

Síndrome de Gorlin

Gorlin syndrome is a rare autosomal dominant disease caused by mutations in the sonic hedgehog signaling pathway. Of particular importance is the PTCH1 gene. The disease is characterized by the development of multiple basal cell carcinomas at young ages. These tumors may present with other skin manifestations such as palmoplantar pits and with extracutaneous manifestations such as odontogenic keratocysts and medulloblastoma. Although the dermatologist may be key for recognizing clinical suspicion of the syndrome, a multidisciplinary team is usually necessary for diagnosis, treatment, and follow-up. Skin treatment may be complicated due to the large number of basal cell carcinomas and the extent of involvement. In recent years, new drugs that inhibit targets in the sonic hedgehog pathway have been developed. Although these agents appear promising options for patients with Gorlin syndrome, their efficacy is limited by adverse effects and the development of resistance.

Correlation Between the Sites of Onset of Basal Cell Carcinoma and the Embryonic Fusion Planes in the Auricle.

Objectives:This study aims at the identification of the distribution of basal cell carcinomas (BCCs) in the auricle in correlation with the currently most credited sites of the embryonic fusion planes of the auricle.Methods:An overall number of 69 patients with 72 BCCs of the auricle were enrolled in the study over a period of 14 years, from June 2003 to October 2017. All the cases underwent medical preoperative digital photography and the specific location of each BCC was coded on an original full-size anatomical diagram of the auricle derived from the reports by Streeter, Wood-Jones, Park, Porter, and Minoux showing the currently most credited sites of the embryonic fusion planes arbitrarily featured as two 5-mm-wide ribbon-like areas: (1) the hyoid-mandibular fusion plane (HM-FP) running from the upper margin of the tragus toward the concha and then deflecting toward the lower margin of the tragus and (2) the free ear fold-hyoid fusion plane (FEFH-FP) running from the cranial-most portion of the helix to the mid-portion of the ascending helix. The latter fusion planes were comprehensively termed embryological fusion planes (EFP) while all of the remaining surface of the auricle was comprehensively termed non-fusion area (NFA). The surfaces of all of the latter areas were calculated using the ImageJ software.Results:According to our data, the greatest number of BCCs was observed within the currently most credited sites of the embryonic fusion planes of the auricle. The latter sites displayed a 12-fold increased tumor incidence in comparison with the remaining surface of the ear.Conclusions:A correspondence between the sites of onset of BCCs and the sites of merging and/or fusion of embryonal processes was demonstrated in the auricle. Therefore, the latter sites might be considered as high-risk areas for the development of a BCC. Such an evidence provides further support to the hypothesis of an embryological pathogenesis of BCC.