Dysplastic nevus syndrome with development of multiple melanomas. A surgical concept for prophylaxis.

A 58-year-old man presented in 2001 with dysplastic nevus syndrome with at least 300 nevi and about 100 clinically highly atypical nevi. Three melanomas had been excised by a private dermatologist within the past year. We then removed two additional melanomas. Between 2002 and 2003, 117 nevi which were atypical on dermatoscopy were removed; most by horizontal (shave) excision. Adequate clinical and dermatoscopic control was not feasible due to the large number of atypical nevi. In order to reduce the number of nevus cells, we treated test sites with both dermabrasion and split-thickness excision. The latter proved to be more efficient and was used to completely excise the skin of the back, as well as parts of the arms and chest in two sessions under general anesthesia. Wound healing was uneventful. Following these procedures, adequate clinical and dermatoscopic monitoring of the remaining nevi was possible and only a few had to be removed. No more melanomas appeared in the treated areas.

Skin self-examination of persons from families with familial atypical multiple mole melanoma (FAMMM).

OBJECTIVE: Early detection of melanomas might increase survival chance. Patients can usually see primary lesions while inspecting their skin. Skin self-examination (SSE) is therefore the recommended pre-screening method. To maximize the likelihood that SSE will be performed, it is important to distinguish those factors that increase the chance of performance. The aim is to examine motivational differences between (a) melanoma-prone persons who perform SSE once every 2-3 months, and (b) melanoma-prone persons with a lower SSE frequency. METHODS: A survey to assess socio-demographic factors, attitude, social influence, self-efficacy and intention to perform SSE. RESULTS: Members (n=71) of 18 familial atypical multiple mole melanoma (FAMMM) families participated; 70% performed SSE at least once every 2-3 months. Adequate performers were more likely to have a partner, had a more positive attitude toward SSE, perceived SSE as less difficult to perform and had a stronger intention to perform SSE compared to poor performers. Logistic regression indicated attitude as the only reliable predictor of SSE performance. CONCLUSION: We found that about one third of our genetically predisposed population did not report an adequate frequency of performing SSE. PRACTICE IMPLICATIONS: Deficiencies in SSE practices in a genetically predisposed population are indicated.

Monitoring of kindreds with hereditary predisposition for cutaneous melanoma and dysplastic nevus syndrome: results of a Swedish preventive program.

PURPOSE: To evaluate a program initiated in 1987 by the Swedish Melanoma Study Group aiming to provide preventive surveillance to kindreds with hereditary cutaneous melanoma and dysplastic nevus syndrome. PATIENTS AND METHODS: Overall, 2,080 individuals belonging to 280 melanoma families were followed for 14 years between 1987 and 2001 at 12 participating centers. Data were registered in a central database. RESULTS: Among 1,912 skin lesions excised during follow-up, 41 melanomas were removed in 32 individuals. Of these, 15 (37%) were in situ melanomas and 26 (63%) invasive melanomas. The median tumor thickness of invasive melanomas was 0.5 mm. Ulceration was absent in 24 of 26 invasive melanomas (92%) and 12 (46%) lacked vertical growth phase. Compared with melanomas in the general Swedish population, the melanomas identified in these kindreds during follow-up had better prognostic characteristics. All melanomas except one were diagnosed in families with two or more first-degree relatives with melanoma. Diagnosis of melanoma occurred in three of eight kindreds with germline CDKN2A mutations, supporting that families with such mutations are at increased risk for melanoma development. Of the 32 individuals who developed melanoma during follow-up, 21 (66%) had had at least one previously diagnosed melanoma. CONCLUSION: This study shows that a coordinated program aimed at detecting and offering skin surveillance in kindreds with hereditary cutaneous melanoma results in a low incidence of melanomas during the follow-up period and that the tumors that do arise have favorable prognostic characteristics.

Improving screening for melanoma by measuring similarity to pre-classified images.

A new method that can dramatically improve the sensitivity and especially the specificity of skin naevi screening for melanoma by physicians is introduced. The method is based on measuring the similarity to previously classified naevi images. As more naevi are being classified, the power of the method is enhanced. Thus physicians can benefit from experience accumulated by others and the screening can be performed effectively by physicians with less experience.

Participant satisfaction and value in American Academy of Dermatology and American Cancer Society skin cancer screening programs in Massachusetts.

BACKGROUND: More than 1 million Americans have attended the American Academy of Dermatology's Melanoma/Skin Cancer Screening Programs since 1985. However, there have been no reports of the participants' perceived value, satisfaction, and benefits of skin cancer screening. OBJECTIVE: We attempted to measure the benefits and subsequent screening practices of persons with presumptive positive screening diagnoses. METHODS: A self-administered questionnaire was sent to participants with positive screening diagnoses in Massachusetts. RESULTS: Of the 643 respondents, 81% rated their satisfaction as high and 84% had similar ratings for the value of the screening. Screenings apparently led to an increase in self-screening (60% before screening compared with 84% after screening). CONCLUSION: Although screening appears to have relatively strong benefits, further studies should be conducted nationally.

Research note: the Lions Cancer Institute and the Western Australian Society of Plastic Surgeons skin cancer screening programme.

BACKGROUND: The Lions Cancer Institute, Perth, and the Western Australian Society of Plastic Surgeons have been investigating the feasibility of community based skin cancer screening. Members of the community responded to newspaper advertisements to attend free skin cancer screening events. This report presents preliminary results obtained from the methods development programme. METHODS: Seventeen screening clinics were conducted in Perth (4) and country towns (14) in Western Australia between January 1991 and October 1993. The participants were interviewed and given promotional literature and their personal profiles were determined. A total body skin examination was performed by a specialist plastic surgeon and any suspicious lesions were recorded and, if necessary, recommendations for further treatment was documented. RESULTS: The number of individuals screened was 3397. Of these, 572 patients were referred to general practitioners for further medical attention of suspicious lesions, 79 patients were clinically diagnosed as having suspicious pigmented skin lesions (13.8% of total lesions and 2.3% of total sample screened). Of these, 53 individual patient pathology reports were obtained. Four invasive malignant melanomas and two in situ melanomas arising in Hutchinson's melanotic freckles were detected. CONCLUSIONS: Debates concerning the efficacy of screening for skin cancer have recently received great attention from many medical disciplines. However, as yet the effectiveness of population based skin cancer screening programmes have not been adequately evaluated with randomized controlled studies. The study reported here provides some findings that may be used to enhance future screening studies.

Atypical mole syndrome: risk factor for cutaneous malignant melanoma and implications for management.

The incidence of malignant melanoma is increasing faster than that of any other cancer. It is important to identify subsets of the population at high risk of its development so that they can be observed more closely to identify early melanomas when they are curable. It has been reported worldwide that persons with the atypical mole (dysplastic nevus) syndrome are such a subset at increased risk. A risk gradient for the development of melanoma exists and varies from persons with one or two atypical moles and no family history of melanoma at one end of the spectrum to persons with the familial atypical multiple-mole melanoma syndrome at the other. Guidelines for the management of atypical mole syndrome are presented.

A questionnaire survey of Midwest dermatologists on the clinical-genetic aspects of patients with multiple atypical nevi.

Midwest dermatologists minimally initiate or carry on in-depth genealogical investigations of patients with the cancer associated genodermatosis, the familial atypical multiple mole melanoma (FAMMM) syndrome. Their conceptualizations of the FAMMM syndrome varies over a wide spectrum and results in a range of clinical behaviour patterns in the care of these patients. If the clinical behavior of physicians with respect to patients with the FAMMM syndrome is a valid model for deducing patterns of clinical behavior in their practices then medical schools' curricula and continuation educational programs need significant revisions to correct these deficiencies in the delivery of health care.

Melanoma/skin cancer screening clinics: experiences in The Netherlands.

In 1989 and 1990 we conducted two free melanoma/skin screening clinics in Oss and Arnhem in the Netherlands. The study was carried out along the lines of the recent campaigns supported by the American Academy of Dermatology. Of 2564 persons screened, 53 had melanoma or nonmelanoma skin cancer (2.1%). Compliance with follow-up for persons with suspected melanoma/skin cancer was adequate (93 of 103; 90.3%).

[Prevention of familial tumor diseases using genetic counseling and early diagnosis]. / Prävention familiärer Tumorkrankheiten durch genetische Beratung und Frühdiagnostik.

Several types of familial cancer can be prevented from progressing into an advanced stage through genetic counselling and regular medical control of persons at risk. Measures of secondary cancer prevention and their efficacy are described for retinoblastoma, MEN2 syndrome, familial colorectal carcinoma with (FAP) or without (HNPCC) pre-existing polyposis coli, dysplastic naevi syndrome, basal cell naevi syndrome and von Hippel-Lindau syndrome. The prevention of malignancies is promising primarily for the familial forms, since persons at risk are motivated to employ preventive measures, especially if they can be identified through DNA diagnosis and counselled with concrete information. The establishment of familial cancer registries should be considered which improve the continuous medical care for all family members, even in the case of a change of the family physician or a loss of contact.

The familial dysplastic nevus syndrome. Natural history and the impact of screening on prognosis. A study of nine families in the Netherlands.

Since 1982, nine families with the dysplastic nevus syndrome have been identified in the Leiden area (The Netherlands). A total of 50 primary melanomas were diagnosed in 38 persons. Nineteen of these melanomas had been diagnosed before the start of the screening programme (category I), 11 were detected at the initial examination of the families (category II), and 20 were found during the course of follow-up (category III). To assess the effect of screening, we compared these categories with respect to the developmental stage of the melanomas. One of the 19 melanomas in category I, two of the 11 in category II and seven of the 20 in category III were melanoma in situ. The average thickness of the invasive melanomas in categories I, II and III was 1.75, 0.80 and 0.54 mm respectively. Sixteen of the 19 melanomas in category I (84%) were Clark III or IV, whereas 15 of the 20 melanomas in category III (75%) were Clark I or II. From these findings it may be concluded that screening can lead to the detection of melanomas at an earlier stage, which in turn can permit curative treatment and improvement of both prognosis and life expectancy. The need for supervision based on central registration of affected families to guarantee the continuity of screening is discussed.

Skin cancer: a primer for the nondermatologist.

The rising incidence of skin cancer in the United States has become a significant public health concern. The morbidity and mortality associated with these conditions can be reduced significantly by earlier detection and treatment. All physicians, not just dermatologists, can play a role in this regard. This article is intended to alert practicing physicians to the extent of the problem and to familiarize them with the various forms of skin cancer.