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[Skin cancer epidemic in the Netherlands]
Abstract
Despite numerous warnings regarding the dangers of exposure to the sun, the number of skin cancer patients continues to increase rapidly. Dermatologists speak of a 'skin cancer epidemic' and estimate that the number of patients is substantially higher than the number estimated on the basis of cancer registry data. According to the Netherlands and Eindhoven cancer registries, 35.500 Dutch people were newly diagnosed with 'skin cancer' in 2006. Many skin cancer patients develop multiple skin tumours, therefore the total number of skin tumours is much higher than this. One in 6 Dutch people will develop skin cancer in their lifetime, 1 in 50 will develop a melanoma. Despite the good prognosis for most skin tumours, there is a high level of morbidity as a result of treatment. The large number of skin tumours and patients puts a great deal of pressure on the health care system.
... Skin cancer incidence is rising. [1][2][3][4][5] Currently, in the Netherlands, one in six people will develop skin cancer 6 and there is no prospect of an end to the increasing number of skin cancer patients. 7 This skin cancer epidemic will put a heavy burden on health care services and health care costs. ...
... 7 This skin cancer epidemic will put a heavy burden on health care services and health care costs. 4,6,7 In the Netherlands, where the general practitioner (GP) has a gatekeeper role, the majority of lesions are initially evaluated by the GP. The skin lesions GPs are consulted for can be pigmented as well as non-pigmented and, in primary care, most are benign. ...
Background
The dermoscope improves general practitioners' (GP) sensitivity for melanoma. However, diagnostic accuracy (DA) and cost-effectiveness of the dermoscope in primary care for the evaluation of all skin lesions suspected of malignancy remains unknown. Objectives
To determine the DA and cost-effectiveness of the dermoscope in primary care for skin lesions suspected of malignancy. Methods
In a cluster randomized clinical trial, 48 Dutch general practices were randomized to either intervention group using a dermoscope or control group using only naked-eye examination. A total of 194 lesions from 170 patients in the intervention group and 222 lesions from 211 patients in the control group were analysed for DA and cost-effectiveness. ResultsThe percentage of correctly diagnosed lesions in intervention group and control group was 50.5% and 40.5% respectively. This was 61.5% and 22.2% for melanomas. In the intervention group, three malignancies were treated with the expectative treatment option compared to none in the control group. The odds ratio (OR) of a correct diagnosis in the intervention group, compared to control group, was 1.51 (95% CI: 0.96–2.37) P = 0.07. Consequently, the relative risk was 1.25. The incremental cost-effectiveness ratio was €89 (95% CI −€60 to €598), indicating that using a dermoscope costs an additional €89 for one additional correctly diagnosed patient. Additional analyses showed better effects of dermoscopy compared to the control group for 98% of the bootstrap resamples. Conclusions
The probability of a correct diagnosis was 1.25 times higher using a dermoscope than without a dermoscope. Although this difference is marginally not statistically significant, dermoscopy in general practice appears to be cost effective. We therefore think that GPs should be trained to use a dermoscope, although they should realize that even with the use of a dermoscope not all lesions will be diagnosed correctly.
... The skin cancer foundation in the United States has been stated that the maximum frequent type of cancer is the skin cancer. Skin cancer increased over amount of time in many region in the world for instance Singapore [10], Slovakia [17], and the Netherland [14]. To date the easiest way to stop spreading of skin cancer is through detecting the skin cancer in early stage to manage to identify the abnormalities of skin cancer. ...
Markov Random Field (MRF) theory has a significant potential role in image segmentation field. It uses (pixels, regions, edges)-based on MRF models to detect objects, boundaries and other relevant information in an image. This paper proposes an extension of Unified Markov Random Field (UMRF) model to include edge-based features. Firstly, the proposed technique employs the likelihood function to combine the advantages of the pixel-based, region-based and edge-based MRF model, by computing the product of the pixel likelihood function, regional likelihood function and edge likelihood function. Secondly, the region-based macro texture features are extracted using the UMRF model. Then the edge-based features are extracted using the maximum gradient method to recover any significant lost information. A principled probabilistic inference is implemented to integrate various types of likelihood information and spatial constraints by iteratively updating the posterior probability of the proposed model. The segmentation process is completed when the iterations converge. The proposed enhanced UMRF technique which combines pixel-based, region-based and edge-based features achieved a higher skin lesion segmentation accuracy than MRF model which combines pixel-based and region-based only.
... The skin cancer foundation in the United States has stated that the greatest frequent form of cancer is skin cancer. Skin cancer increase over time in many regions in the world such as Singapore [13], Slovakia [4], and The Netherland [14]. To date, the best way to stop spreading of skin cancer is through detecting the skin cancer in the early stage to be able to identify the abnormalities of skin cancer. ...
Most of the medical institutions still use manual methods to detect the skin cancers tumors. However, melanoma detection using human vision alone can be subjective, inaccurate and poorly reproducible even among experienced dermatologists. This is attributed to the challenges in the automatic segmentation of skin cancer due to many factors, such as different skin colors and the presence of hair, diverse characteristics including lesions of varying sizes and shapes, lesions that may have fuzzy boundaries. To address these factors, a Unified Markov Random Field (UMRF) is used to segment both pixel information and regional information corresponding to skin lesions from the images, where UMRF model lies in two aspects. First, it combines the benefits of the pixel-based and the region-based Markov Random Field (MRF) models by decomposing the likelihood function into the product of the pixel likelihood function and the regional likelihood function. The experimental results show that the employed method has high precision (Jaccard Index).
... Skin cancer incidence is rising [1][2][3][4][5][6]. In the Netherlands, one in six people are expected to develop skin cancer [7]. Public awareness is also rising as a result of many public information campaigns [8][9][10][11] and this may lead to an increased consultation rate. ...
Skin cancer is believed to impose a heavy burden on healthcare services, but the burden of skin lesions suspected of malignancy on primary healthcare has never been evaluated. Therefore the aim of this study was to determine the demand for care in general practice due to these suspected skin lesions (i.e. lesions that are suspected of malignancy by either the patient or the GP).
Registry study based on data (2001-2010) from the Registration Network Groningen. This is a general practice registration network in the northern part of the Netherlands with an average annual population of approximately 30,000 patients. All patient contacts are coded according to the International Classification of Primary Care (ICPC). Consultations for skin lesions suspected of malignancy were selected according to the assigned ICPC codes. Subsequently, the number of consultations per year and the annual percent change in number of contacts (using the JoinPoint regression program) were calculated and analysed. Additionally, the percentage of patients referred to secondary care or receiving minor surgery within one year after the first contact were calculated.
From 2001 onwards we found an annual increase in demand for care due to skin lesions suspected of malignancy of 7.3% (p < 0.01) and in 2010 the benign:malignant ratio was 10:1. In total 13.0% of the patients were referred and after 2006, minor surgery was performed on 31.2% of the patients. Most surgeries and referrals took place within 30 days.
Suspected skin lesions impose an increasing burden on primary healthcare and most likely on healthcare costs as well. General practitioners should therefore be trained in diagnosing skin lesions suspected of malignancy, as a high diagnostic accuracy can save lives in the case of melanoma, and may also prevent unnecessary, costly, excisions and referrals to secondary healthcare.
... Among the B15 million Dutch citizens, more than 350,000 skin malignancies have occurred between 1989 and 2005. It was estimated that one out of six Dutch citizens will develop a skin tumor in their lifetime (de Vries et al., 2009). The keratinocytic skin cancers (BCC and SCC) and melanoma together comprise more than 98% of all skin tumors. ...
Epidemiology of rare cutaneous malignancies in the general population is poorly documented. This descriptive study aimed to estimate the incidence and trends of all skin malignancies between 1989 and 2005. Data on skin tumors were extracted from the Netherlands Cancer registry (except for basal cell carcinoma (BCC) data—only available from Comprehensive Cancer Centre South) and categorized according to the International Classification of Diseases for Oncology, third edition, codes. Age-standardized incidence rates (European standardized population rate, ESR) per 100,000 person-years were calculated per year and for the period between 2001 and 2005. Estimated annual percentage changes (EAPCs) were estimated by Poisson regression models. A total of 356,620 skin tumors were diagnosed between 1989 and 2005. Excluding BCC, squamous cell carcinoma (SCC), and melanoma, the remaining skin tumors constituted about 2% of all skin malignancies. The incidence of melanoma showed the steepest increase (EAPC, 4.0%), and ESR was close to that observed for SCC (EAPC, 2.3%) between 2001 and 2005 (17.1 versus 19.6). Hematolymphoid tumors (ESR ¼ 0.74) were mainly cutaneous T-cell lymphomas (60.8%). No significant increases in incidence were observed for lymphomas, and appendageal, fibromatous, and myomatous carcinomas during 1989–2005. In addition to keratinocytic cancers and melanoma, there is a wide variety of skin tumors that constitute o2% of all skin malignancies. The incidence of UV-related skin tumors increased significantly and more steeply than did those of other skin malignancies.
... Among the B15 million Dutch citizens, more than 350,000 skin malignancies have occurred between 1989 and 2005. It was estimated that one out of six Dutch citizens will develop a skin tumor in their lifetime (de Vries et al., 2009). The keratinocytic skin cancers (BCC and SCC) and melanoma together comprise more than 98% of all skin tumors. ...
Epidemiology of rare cutaneous malignancies in the general population is poorly documented. This descriptive study aimed to estimate the incidence and trends of all skin malignancies between 1989 and 2005. Data on skin tumors were extracted from the Netherlands Cancer registry (except for basal cell carcinoma (BCC) data-only available from Comprehensive Cancer Centre South) and categorized according to the International Classification of Diseases for Oncology, third edition, codes. Age-standardized incidence rates (European standardized population rate, ESR) per 100,000 person-years were calculated per year and for the period between 2001 and 2005. Estimated annual percentage changes (EAPCs) were estimated by Poisson regression models. A total of 356,620 skin tumors were diagnosed between 1989 and 2005. Excluding BCC, squamous cell carcinoma (SCC), and melanoma, the remaining skin tumors constituted about 2% of all skin malignancies. The incidence of melanoma showed the steepest increase (EAPC, 4.0%), and ESR was close to that observed for SCC (EAPC, 2.3%) between 2001 and 2005 (17.1 versus 19.6). Hematolymphoid tumors (ESR=0.74) were mainly cutaneous T-cell lymphomas (60.8%). No significant increases in incidence were observed for lymphomas, and appendageal, fibromatous, and myomatous carcinomas during 1989-2005. In addition to keratinocytic cancers and melanoma, there is a wide variety of skin tumors that constitute <2% of all skin malignancies. The incidence of UV-related skin tumors increased significantly and more steeply than did those of other skin malignancies.
Objectives
In 2016, the SKINCATCH Trial, a clustered multi-centre randomised trial, was initiated to assess whether low-risk basal cell carcinomas (BCCs) can be treated by general practitioners (GPs) without loss of quality of care. The trial intervention consisted of a tailored 2-day educational course on skin cancer management. The aim of this process evaluation was to investigate GPs’ exposure to the intervention, implementation of the intervention and experiences with the intervention and trial.
Research design and methods
Data on exposure to the intervention, implementation and experiences were obtained at several points during the trial. Complementary quantitative components (ie, surveys, database analysis, medical record analysis) and qualitative components (ie, interviews and focus groups) were used. Quantitative data were analysed using descriptive statistics; qualitative data were summarised (barrier interviews) or audiorecorded, transcribed verbatim and thematically analysed using Atlas.Ti (focus groups).
Results
Following a 100% intervention exposure, results concerning the implementation of the trial showed that aside from the low inclusion rate of patients with low-risk BCCs (n=54), even less excisions of low-risk BCCs were performed (n=40). Although the intervention was experienced as highly positive, several barriers were mentioned regarding the trial including administrative challenges, lack of time and high workload of GPs, low volume of BCC patients and patients declining to participate or requesting a referral to a dermatologist.
Conclusions
Although GPs’ participation in the highly valued training was optimal, several barriers may have contributed to the low inclusion and excision rate of low-risk BCCs. While some of the issues were trial-related, other barriers such as low patient-volume and patients requesting referrals are applicable outside the trial setting as well. This may question the feasibility of substitution of surgical excisions of low-risks BCCs from secondary to primary care in the current Dutch setting.
Trial registration number
Trial NL5631 (NTR5746).
Background
The high prevalence of actinic keratosis (AK) requires the optimal use of healthcare resources.
Objectives
To gain insight in to the healthcare utilization of people with AK in a population‐based cohort, and the management of AK in a primary and secondary care setting.
Methods
A retrospective cohort study using three complementary data sources was conducted to describe the use of care, diagnosis, treatment and follow‐up of patients with AK in the Netherlands. Data sources consisted of a population‐based cohort study (Rotterdam Study), routine general practitioner (GP) records (Integrated Primary Care Information) and nationwide claims data (DRG Information System).
Results
In the population‐based cohort (Rotterdam Study), 69% (918 of 1322) of participants diagnosed with AK during a skin‐screening visit had no previous AK‐related visit in their GP record. This proportion was 50% for participants with extensive AK (i.e. ≥ 10 AKs; n = 270). Cryotherapy was the most used AK treatment by both GPs (78%) and dermatologists (41–56%). Topical agents were the second most used treatment by dermatologists (13–21%) but were rarely applied in primary care (2%). During the first AK‐related GP visit, 31% (171 of 554) were referred to a dermatologist, and the likelihood of being referred was comparable between low‐ and high‐risk patients, which is inconsistent with the Dutch general practitioner guidelines for ‘suspicious skin lesions’ from 2017. Annually, 40 000 new claims representing 13% of all dermatology claims were labelled as cutaneous premalignancy. Extensive follow‐up rates (56%) in secondary care were registered, while only 18% received a claim for a subsequent cutaneous malignancy in 5 years.
Conclusions
AK management seems to diverge from guidelines in both primary and secondary care. Underutilization of field treatments, inappropriate treatments and high referral rates without proper risk stratification in primary care, combined with extensive follow‐up in secondary care result in the inefficient use of healthcare resources and overburdening in secondary care. Efforts directed to better risk differentiation and guideline adherence may prove useful in increasing the efficiency in AK management.
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Het basaalcelcarcinoom (BCC) is de meest voorkomende vorm van kanker in Nederland (80% van alle huidkankers). Het aantal BCC’s is in ruim 35 jaar tijd enorm toegenomen en deze stijging zet door. Geschat wordt dat in 2020 de incidentie zelfs zal stijgen naar 234 per 100.000 persoonsjaren voor mannen en 226 per 100.000 persoonsjaren voor vrouwen. Vooral de vergrijzing in combinatie met de toegenomen hoeveelheid zonlichtexpositie vergroot het risico op het krijgen van een BCC. Dit artikel geeft een overzicht van de diagnostische procedures en de verschillende behandelmogelijkheden.
Er zijn diverse mogelijkheden om huidkanker of de voorstadia daarvan te behandelen. De keuze van de therapie is afhankelijk van onder meer de diagnose, diepte van de tumor, (histologische) groeiwijze, grootte, lokalisatie, primaire tumor of recidief, eventuele uitzaaiingen, leeftijd en conditie van de patiënt. In de huisartsenpraktijk bestaat het grootste deel van de dermato-oncologie uit drie maligne aandoeningen (basaalcelcarcinoom, plaveiselcelcarcinoom, melanoom) en de premaligne actinische keratosen. Bij de overgrote meerderheid van de patiënten kan de huisarts de (eerste) behandeling zelf uitvoeren, waarbij chirurgische excisie voor de maligne tumoren en cryotherapie voor actinische keratosen als standaardtherapie beschouwd worden. Bij goede indicatiestelling en zorgvuldige lege artis uitvoering daarvan, is de eerstelijnszorg voor dermato-oncologische patiënten effectief, efficiënt, patiëntvriendelijk en goedkoop. Een groot voordeel van chirurgie is dat histologische verifi catie van de diagnose en van radicaliteit kan plaatsvinden. Andere behandelmodaliteiten komen alleen in aanmerking wanneer de standaardtherapie door tumor- of patiëntkarakteristieken minder wenselijk of geschikt is. Hierna worden zowel de behandelmethoden die door de huisarts kunnen worden uitgevoerd als enkele therapiemogelijkheden voor de tweede lijn kort besproken. Therapie voor zeldzame aandoeningen en gemetastaseerde kanker valt buiten het bestek van dit boek. Behandelingen voor individuele diagnosen zijn beschreven bij de betreffende ziektebeelden.
Huidkanker is in principe grotendeels een vermijdbare aandoening, aangezien de meeste huidtumoren het gevolg zijn van overmatige blootstelling aan ultraviolette (UV) straling bij mensen met een zongevoelige huid. Vermijden van die straling of vermindering daarvan is logischerwijs een belangrijk aspect van de preventie van huidkanker.
This article gives us the results of an open house project focusing on skin cancer. It was conducted at the dermatology department Deventer hospital. The public was informed about the clinical picture, the types and the treatment of skin cancer. All participants were given the opportunity to be examined by a dermatologist for suspected lesions. A total of 245 people were examined. In 11 of the 245 participants a skin cancer was diagnosed and confirmed histologically (10 basal cell carcinoma and 1 melanoma). This represents a yield of 4.5%. Furthermore 52 cases of actinic keratosis (in 12 of them histologically diagnosed) and 2 cases of histologically diagnosed dysplastic nevi were found. This open house shows how important it is to check suspicious spots professionally. A 'check your spot' day, where suspected lesions can be screened anonymously and free of any barrier (including costs), contributes to early detection and treatment of skin cancer. In addition, the combination of information and screening on request will contribute to the self-awareness for skin cancer in the general population.
Huidkanker neemt in Nederland epidemische vormen aan en het einde is voorlopig niet in zicht. Tussen 1989 en 2006 is het aantal
patiënten bij wie een vorm van huidkanker werd geconstateerd met 140% toegenomen. Door de goede prognose van de meeste huidtumoren
leidt deze aandoening niet zozeer tot sterfte als wel tot ziektelast (morbiditeit), cosmetische problemen en zorgconsumptie
(met daaraan gerelateerde kosten). Huidkanker is daardoor een groot volksgezondheidsprobleem geworden, zowel in termen van
incidentie en prevalentie, als ook in termen van beslag op de medische middelen en menskracht. Plaveiselcelcarcinomen en basaalcelcarcinomen
komen vaak in het gezicht voor, waar de behandeling soms voor grote littekens zorgt, met bijbehorende functionele en cosmetische
morbiditeit. Bijna de helft van de patiënten met een basaalcelcarcinoom – met 75% van het totaal verreweg de meest voorkomende
vorm van huidkanker – krijgt meerdere huidtumoren, met navenante zorg. In de praktijk worden bij deze patiënten vaak verschillende
basaalcelcarcinomen op dezelfde dag gevonden, soms meer dan tien. In verband met de kans op recidieven, multipele tumoren
en – in het geval van plaveiselcelcarcinomen en melanomen – metastasering, staan veel patiënten met huidkanker onder langdurige
medische controle. Ook is huidkanker, ondanks de goede prognose van de meeste tumoren, vaak aanleiding tot hospitalisatie:
in de periode 2003-2007 steeg het aantal ziekenhuisopnamen voor huidkanker van 6900 naar 17 900. De opnamen vinden vooral
plaats in de hogere leeftijdsgroepen met een sterke stijging bij mannelijke 75-plussers. De groei wordt nagenoeg geheel veroorzaakt
door dagopnames voor fotodynamische therapie van huidkanker. Door al deze factoren neemt de druk op de zorgverleners in de
preventieve en curatieve zorg sterk toe. Bij huisartsen, dermatologen en (plastisch) chirurgen dreigt zo een toenemende discrepantie
tussen vraag naar en aanbod van zorg te ontstaan.
Het basaalcelcarcinoom (synoniemen: basalecelcarcinoom, basocellulair carcinoom, basalioom) is de meest voorkomende maligne
tumor bij de mens. Dit carcinoom ontleent zijn naam aan de gelijkenis van de tumorcellen met de cellen in de basale cellaag
van de epidermis en ontstaat uit onvolledig gedifferentieerde immature keratinocyten van de epidermis of de huidaanhangselen.
Behavioural interventions to reduce exposure to ultraviolet radiation (UVR) can reduce risk of skin cancer.
To integrate the data and to evaluate the impact of interventions to limit exposure to UVR on skin cancer incidence in four selected countries.
Using PREVENT, a dynamic simulation model, we modelled the potential for skin cancer prevention in four European countries under various scenarios to avoid damage by UVR.
In general, the most effective interventions were those aimed at protecting people during outdoor work and outdoor hobbies against the harmful effects of UVR, and combinations of several interventions. These could in theory lead to reductions of up to 45% in skin cancer cases projected for the year 2050.
The scope for prevention depends on the prevalence of the risk factors in the different countries, as well as the associated risk factors and time lags modelled.
Junctional epidermolysis bullosa, type Herlitz (JEB-H) is a rare, autosomal recessive disease caused by absence of the epidermal basement membrane adhesion protein laminin-332. It is characterized by extensive and devastating blistering of the skin and mucous membranes, leading to death in early childhood.
To present the results of the long-term follow-up of a cohort of patients with JEB-H, and to provide guidelines for prognosis, treatment and care.
All patients with JEB-H included in the Dutch Epidermolysis Bullosa (EB) Registry between 1988 and 2011 were followed longitudinally by our EB team. Diagnosis was established using immunofluorescence antigen mapping, electron microscopy and DNA analysis.
In total, we included 22 patients with JEB-H over a 23-year period. Their average age at death was 5.8 months (range 0.5-32.6 months). The causes of death were, in order of frequency: failure to thrive, respiratory failure, pneumonia, dehydration, anaemia, sepsis and euthanasia. The pattern of initial weight gain was a predictor of lifespan in these patients. Invasive treatments to extend life did not promote survival in our patients.
It is important to diagnose JEB-H as soon as possible after birth so that the management can be shifted from life-saving to comfort care. The palliative end-of-life care can take place in hospital, but is also safe in the home setting. Suffering in patients with JEB-H can become so unbearable that in some patients who do not respond to adequate analgesic and sedative treatment, newborn euthanasia, performed according to the Groningen protocol, is legally permitted in the Netherlands.
Some subtypes of the heterogeneous genetic blistering disease epidermolysis bullosa (EB) lead to lethality in childhood. The severity and extent of blistering leaves these patients living in excruciating pain and distress their entire lives. Parents of these patients experience some specific problems, such as the unfamiliarity of EB amongst healthcare professionals and the suffering and loss of their child.
To identify the needs of parents who have lost their child to lethal EB.
A qualitative study was performed, comprising semistructured, in-depth interviews with 16 parents. The transcripts were analysed and common themes were identified.
Parents indicated that they have the need (i) for a fast and correct referral to a specialized EB clinic, (ii) to be informed as honestly as possible about the diagnosis and lethal prognosis, (iii) to have a structured network of caregivers in the palliative care, (iv) to be involved in the care and the medical decisions involving their child, (v) to be informed about the end of life and to discuss euthanasia, (vi) for guidance and to have remembrances of their child, and (vii) for genetic counselling.
Our job as healthcare professionals is to provide the best care not only for children suffering from lethal EB, but also for their parents. In this study, parents have provided us with some guidelines to care for them. However, it is important to keep in mind that every parent is different, and that the guidance should be tailored to their individual needs.
The incidence of multiple basal cell carcinomas (BCCs) is not well documented.
To calculate the cumulative risks, rates and risk factors for the development of subsequent histologically confirmed BCCs.
For this cohort study the Dutch nationwide network and registry of histopathology and cytopathology (PALGA) was used. The first 2483 patients diagnosed with a first histologically confirmed BCC in the year 2004 were followed for 5years. Multifailure survival models were used to study whether gender or age affected the risk of developing subsequent tumours.
During our observational period, the 2483 patients developed a total of 3793 histologically confirmed BCCs. The 5-year cumulative risk of developing one or more subsequent BCCs was 29·2%. Incidence rates were 25,318 per 100,000person-years in the first 6months after first BCC diagnosis, decreasing to 6953 per 100,000person-years after 5years of follow-up. Males compared with females had a 30% [adjusted hazard ratio (HR) 1·30, 95% CI (confidence interval) 1·11-1·53] higher risk of developing multiple BCCs and those aged 65-79years had more than 80% (adjusted HR 1·81, 95% CI 1·37-2·41) higher risk of having subsequent tumours compared with patients younger than 50years.
The high incidence rate of subsequent BCCs among patients with a first BCC is highest in the first months after diagnosis of the first BCC but persists long term, indicating that patients with BCC should undergo full-body skin examinations at first presentation and subsequent follow-up visits. Special attention should be paid to males and persons of older age at index lesion.
To assess the long-term outcome after sentinel lymph node biopsy (SLNB) in melanoma patients.
Between 1995-2009 450 melanoma patients underwent SLNB in a single center. Survival and prognostic factors were analyzed for 429 patients.
Median age was 53 (range 11-84) years. Median Breslow thickness was 2.4 (range 1-20) mm and 36% were ulcerated melanomas. Median follow-up time was 64.8 (range 2-174) months. A tumor-positive SLN was present in 140 patients (31%). Completion lymph node dissection (CLND) was performed in 119 patients and these patients were analyzed for recurrence and survival. 124 Patients (29%) relapsed during follow-up; 55 in the node-positive group who underwent CLND (55/119; 46%) and 69 in the node-negative group (69/310; 22%; p < 0.001). In the node-negative group 17 patients developed recurrence in the regional node field; false-negative rate 11%. On multivariate analysis strongest prognostic factors for disease free survival (DFS) were primary melanoma ulceration and SLN positivity (Hazard Ratio (HR) of 2.2 and 2.3; p < 0.001). For disease specific survival (DSS) the same was found to be true with an HR of 2.1 for ulceration and 2.0 for SLN positivity (p = 0.001 and p = 0.002 respectively). 10-Year DFS was 71% for node-negative patients compared with 48% for node-positive patients (p < 0.001). 10-Year DSS was 77% for node-negative patients compared to 60% for node-positive patients (p < 0.001).
This study shows a remarkably high percentage of tumor-positive SLN. The long-term follow-up data confirm that tumor-positive SLN patients have a worse DFS and DSS than tumor-negative SLN patients. Ulceration and SLN status proved to be the strongest prognostic factors for long-term DFS and DSS.
Squamous cell carcinoma (SCC) is the most severe complication and most common cause of death in patients with recessive dystrophic epidermolysis bullosa. The risk of developing SCC among patients with junctional epidermolysis bullosa (JEB) is unclear from the literature; however, in our center we noticed an unexpected number of SCCs among adult patients with JEB.
To review all documented patients with JEB in whom an SCC developed, both from our epidermolysis bullosa (EB) center and those reported in the literature.
A search in our EB registry documenting all JEB patients visiting our EB referral center from 1990 through 2010 revealed 7 JEB patients who developed 1 or more SCCs. A systematic literature search revealed 8 relevant articles documenting a total of 7 patients who developed an SCC.
In our EB registry we found 7 patients with JEB who developed an SCC; these were all adults classified with non-Herlitz type JEB. The frequency of developing an SCC among adult JEB patients (n = 28) in our center was therefore 25%. In the literature, we found 7 case reports of JEB complicated by SCC (also classified as JEB, non-Herlitz type), bringing the total number of documented cases to 14. The first SCC in JEB patients developed at an average age of 50 years (median, 52 years; range, 28-70 years). In 9 of 14 cases, multiple primary SCCs occurred, with a total of 45 SCCs. The SCCs are most often located on the lower extremities, in areas of chronic blistering, long-standing erosions, or atrophic scarring. Three patients (21%) developed metastases and died on average 8.9 years after diagnosis of the initial SCC.
This study was retrospective and the statistical analyses were based on a small number of patients.
From their third decade, adult patients with JEB have an increased risk (1:4) of developing SCC on their lower extremities. The SCCs have a high recurrence rate and follow an aggressive course that results in death in 1 of 5 patients. We recommend annual checks of all JEB patients for SCC starting at 25 years of age.
Junctional epidermolysis bullosa of late onset (JEB-lo) is a rare disease characterized by blistering of primarily the hands and feet starting in childhood. The pathogenesis remains unclear.
To clarify the pathogenesis of JEB-lo.
Two patients with JEB-lo, a brother and a sister, were examined using electron microscopy (EM), immunofluorescence (IF) antigen mapping and molecular analysis.
We found subtle changes in IF antigen mapping and EM. The most remarkable changes were loss of the apical-lateral staining of monoclonal antibodies (mAbs) against type XVII collagen (Col17), and a broadened distribution of mAb staining against the ectodomain of Col17, laminin-332 and type VII collagen. Mutation analysis of COL17A1, encoding Col17, showed a compound heterozygosity for a novel mutation c.1992_1995delGGGT and the known mutation c.3908G>A in both patients. The deletion c.1992_1995delGGGT results in a premature termination codon and mRNA decay, leaving the patients functionally hemizygous for the missense mutation c.3908G>A (p.R1303Q) in the noncollagenous 4 domain of Col17.
JEB-lo is an autosomal recessive disorder caused by mutations in COL17A1, and subtle aberrations in EM and IF antigen mapping are clues to diagnosis.
Er zijn veel risicofactoren voor het ontwikkelen van huidkanker, die per tumortype verschillend kunnen zijn of verschillend
zwaar wegen (tabel 2.1). Zongevoelige huid (huidtypen I en II) en overmatige blootstelling aan ultraviolette straling spelen
in de cutane carcinogenese een zeer belangrijke rol.
During the period 1986-2001, a metastasised basal-cell carcinoma of the head was diagnosed in five patients (a 35-year-old woman and four men aged 40, 44, 54 and 54 years) at the Utrecht University Medical Centre, the Netherlands. Metastases were found in the cervical lymph nodes, the skeleton, the parotid region and the lungs. The tumours were all of the morphoeic or 'wispy' type. The treatment consisted of excision and sometimes radiotherapy. Two patients died, one of whom of a cause unrelated to the tumour, two patients were free of symptoms 24 months after the last treatment and one patient was still being treated with radiotherapy. It is often assumed that basal-cell carcinomas do not metastasised, but a frequency of 0.0028-0.55% is reported in the literature. An important risk factor is the size of the tumour. Surgical excision or Mohs' micrographic surgery is the preferred method of treatment because this allows histological inspection of the excised margins. Due to the low incidence, there are no clear therapeutic guidelines for the treatment of patients with metastasised basal-cell carcinoma.