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Cutaneous melanoma in children and adolescents and aspects of naevus phenotype in melanoma risk assessment
Linköping University, Department of Clinical and Experimental Medicine, Dermatology and Venerology . Linköping University, Faculty of Health Sciences.
2006 (English)Doctoral thesis, comprehensive summary (Other academic)
Abstract [en]

Cutaneous malignant melanoma (CMM) is one of the most rapidly increasing cancers in the Swedish population. The aetiology of melanoma is a complex interplay between genetics, host characteristics and environmental factors. The host characteristic with the strongest association with CMM is a phenotype with high numbers of common naevi and with dysplastic naevi. The principal environmental factor is sun exposure.

Melanoma risk assessment (paper I)

In a multi-national study including 986 subjects from Sweden, Denmark, the UK, Germany and the Netherlands, the ability of primary care physicians and nurses to identify individuals at increased melanoma risk was assessed. The atypical mole syndrome (AMS) scoring system for melanoma risk was used. The AMS scoring system consists of a five point check list incorporating total body naevus counts, clinically dysplastic naevi and body distribution of naevi. After brief training, the overall agreement in diagnosis between the trained personnel and experienced dermatologists was 94.5% (kappa value 0.70, p<0.05). The study showed that the scoring system successfully can be taught to personnel in primary care.

The naevus phenotype in a population in northern Sweden (paper II)

The naevus phenotype was investigated in a population living in the inland of northern Sweden with a low melanoma incidence. Two hundred and one participants from the community of Storuman were included. The median naevus count was15 common naevi/individual, and the prevalence of dysplastic naevi was 11%. The median naevus count and prevalence of dysplastic naevi were significantly lower than previously described in populations with higher melanoma incidence and higher ambient ultraviolet exposure in southern Sweden. This geographical variation in naevus phenotype might be explained by differences in levels of sun exposure and in genotype.

Cutaneous malignant melanoma in children and adolescents (papers III–V)

During the years 1973 to 2002, 250 cases of primary CMM in individuals aged 0-19 years were reported to the Swedish Cancer Registry. Histological material was available for review in 87% of the cases registered during the two first decades (1973–1992). The diagnostic accuracy in the reviewed material was 88%.

The melanoma incidence doubled in teenagers between the first decade (1973–1982) and the second (1983–1992). During the third decade (1993–2002) the increasing trend was broken. A decrease in incidence was noted in boys during 1993–1997, and in girls during 1998–2002. In younger children the incidence remained extremely low, only 4 cases in children aged 0–9 years were reported during the studied 30-year period. The trunk was the most common melanoma site in boys, and legs and trunk were the most common sites in girls. Superficial spreading melanoma was the most frequent subtype, followed by nodular melanoma. During the two first decades (1973–1992), the median melanoma thickness decreased from 1.5 to 0.9 mm. The melanoma-specific 5-year survival rate was 93%. The most important prognostic factor was melanoma thickness. The prognosis for thin lesions was excellent, during a median follow up time of 12 years, no tumour less than 0.8 mm was lethal according to the Registry.

The results indicate that CMM in teenagers has many features in common with adult onset melanoma. The tudy also underlines the importance of not neglecting lesions suspected for malignant change in children and adolescents, as early detection and removal is crucial for the prognosis also in this young age group.

Place, publisher, year, edition, pages
Institutionen för biomedicin och kirurgi , 2006.
Series
Linköping University Medical Dissertations, ISSN 0345-0082 ; 961
Keyword [en]
melanoma, naevus, childhood, adolescence, prevention, epidemiology
National Category
Dermatology and Venereal Diseases
Identifiers
URN: urn:nbn:se:liu:diva-7703ISBN: 91-85643-73-4 (print)OAI: oai:DiVA.org:liu-7703DiVA: diva2:22702
Public defence
2006-11-03, Eken, Campus US, Linköpings Universitet, Linköping, 09:00 (English)
Opponent
Supervisors
Note
The electronic version of the thesis is a corrected version of the printed version.Available from: 2006-11-09 Created: 2006-11-09 Last updated: 2009-08-22
List of papers
1. Teaching non-specialist health care professionals how to identify the atypical mole syndrome phenotype: a multinational study
Open this publication in new window or tab >>Teaching non-specialist health care professionals how to identify the atypical mole syndrome phenotype: a multinational study
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2000 (English)In: British Journal of Dermatology, ISSN 0007-0963, Vol. 142, no 2, 331-337 p.Article in journal (Refereed) Published
Abstract [en]

The atypical mole syndrome (AMS) phenotype is the strongest known risk factor for cutaneous melanoma but recognition of the phenotype has been claimed to be problematic and to require specialist assessment. This study determined the ability of previously unskilled doctors and nurses in five countries to recognize the phenotype after brief training. The system used was the AMS scoring system. This incorporates melanocytic naevus counts, clinical atypia of naevi and distribution of naevi. The agreement in scoring between the dermatologist and trained personnel was determined in 986 patients; overall agreement in diagnosis was 94·5% (kappa 0·70, P < 0·0001). The kappa scores in different countries ranged from 0·65 to 0·77 for individual naevus characteristics, indicative of good agreement. Accurate diagnosis of the atypical mole syndrome phenotype is possible by non-specialists. This has implications for collaborative studies of naevi, for screening and for both primary and secondary prevention of melanoma.

Keyword
AMS scoring system, atypical mole syndrome, melanoma, risk factor, teaching
National Category
Medical and Health Sciences
Identifiers
urn:nbn:se:liu:diva-14135 (URN)10.1046/j.1365-2133.2000.03405.x (DOI)
Available from: 2008-11-14 Created: 2008-11-14 Last updated: 2009-08-19
2. Prevalence of pigmented naevi in a Swedish population living close to the Arctic Circle
Open this publication in new window or tab >>Prevalence of pigmented naevi in a Swedish population living close to the Arctic Circle
2000 (English)In: Acta Dermato-Venereologica, ISSN 0001-5555, Vol. 80, no 5, 335-339 p.Article in journal (Refereed) Published
Abstract [en]

The prevalence of common naevi and dysplastic naevi was investigated in a Swedish population with a low incidence of melanoma. A total of 201 subjects aged 30-50 years living in northern Sweden participated. The median number of common naevi per individual was 15, and 11% had dysplastic naevi. Higher numbers of common naevi were found in individuals with dysplastic naevi (median 68) and in those who had spent their childhood in southern Sweden (median 44). The prevalence of common naevi and dysplastic naevi was significantly lower than reported from a previously studied population in southern Sweden, with a melanoma incidence 4 times higher than in the north. The strong variability in naevus phenotype, and in melanoma incidence, between different regions of Sweden seems to be due to different levels of sun exposure rather than to differences in constitutional factors.

National Category
Medical and Health Sciences
Identifiers
urn:nbn:se:liu:diva-14136 (URN)10.1080/000155500459259 (DOI)
Available from: 2008-11-14 Created: 2008-11-14 Last updated: 2009-08-19
3. Increasing incidence of cutaneous malignant melanoma in children and adolescents 12–19 years of age in Sweden 1973–92
Open this publication in new window or tab >>Increasing incidence of cutaneous malignant melanoma in children and adolescents 12–19 years of age in Sweden 1973–92
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1998 (English)In: Acta Dermato-Venereologica, ISSN 0001-5555, Vol. 78, no 4, 289-292 p.Article in journal (Refereed) Published
Abstract [en]

One-hundred-and-seventy-seven cases of cutaneous malignant melanoma were reported to the Swedish National Cancer Registry in subjects below the age of 20 during the period 1973-92. One-hundred-and-fifty-four (87%) were re-examined histologically, and the original diagnosis of primary cutaneous malignant melanoma was verified in 88%. The age-specific mean annual incidence rate increased to 0.5/100,000 in 1983-92 from 0.2/100,000 in 1973-82. Cutaneous malignant melanoma remained extremely rare in children below the age of 12, where only two cases were found. In subjects aged 12-19, the incidence doubled to 93 cases in the second 10-year period compared to 41 in the first. In boys, most of the melanomas occurred on the trunk, and, in girls, on the legs. Sixty-three percent of the melanomas were of the superficial spreading type, which also was the most rapidly increasing type of melanoma. These results emphasize the importance of surveillance and intensified preventive measures in protecting children and adolescents from the harmful effects of excessive exposure to the sun

National Category
Medical and Health Sciences
Identifiers
urn:nbn:se:liu:diva-14137 (URN)10.1080/000155598441882 (DOI)
Available from: 2008-11-13 Created: 2008-11-13 Last updated: 2009-08-19
4. Cutaneous malignant melanoma in Swedish children and teenagers 1973–1992 clinicopathological: study of 130 cases
Open this publication in new window or tab >>Cutaneous malignant melanoma in Swedish children and teenagers 1973–1992 clinicopathological: study of 130 cases
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1999 (English)In: International Journal of Cancer, ISSN 0020-7136, Vol. 80, no 5, 646-651 p.Article in journal (Refereed) Published
Abstract [en]

To assess whether there has been a change in histological features and prognostic factors of primary cutaneous malignant melanoma (CMM) in young individuals in Sweden, an unselected, population-based study was undertaken; 177 cases of primary CMM in persons below 20 years of age were reported to the Swedish National Cancer Registry between 1973 and 1992. In 87% of the cases, original tumor tissue was available for histo-pathological review. The original diagnosis was verified in 88% (n = 126) of these cases. All tumors had histological features similar to adult CMM; 17% had an associated precursor lesion. Superficial spreading melanoma (SSM) was the most common sub-type, constituting 20/36 cases in the first decade and 59/90 in the second. Corresponding figures for nodular melanoma (NM) were 11/36 and 23/90. Only 5 melanomas in situ were diagnosed. In girls, the mean thickness of SSM decreased from 1.5 to 0.6 mm (p < 0.001). Overall mortality was 10%, 22% in the group with CMM diagnosed 0-15 years of age and 8% in individuals 15-19 years. Fatal CMM cases diagnosed below 15 years of age (n = 4) were NM >1.6 mm thick and in subjects 15-19 years (n = 9) 44% of fatal tumors were NM with a mean thickness of 2.2 mm. Breslow index was the single most important prognostic factor. However, when known prognostic factors were adjusted for in a Cox regression analysis, young age remained an independent risk factor, with a relative death rate of 0.21 for individuals aged 15-19 compared with children <15 years of age.

National Category
Medical and Health Sciences
Identifiers
urn:nbn:se:liu:diva-14138 (URN)10.1002/(SICI)1097-0215(19990301)80:5<646::AID-IJC2>3.0.CO;2-H (DOI)
Available from: 2008-11-14 Created: 2008-11-14 Last updated: 2009-08-20
5. Cutaneous malignant melanoma in children and adolescents in Sweden, 1993–2002: the increasing trend is broken
Open this publication in new window or tab >>Cutaneous malignant melanoma in children and adolescents in Sweden, 1993–2002: the increasing trend is broken
2007 (English)In: International Journal of Cancer, ISSN 0020-7136, E-ISSN 1097-0215, Vol. 121, no 2, 323-328 p.Article in journal (Refereed) Published
Abstract [en]

The incidence of cutaneous malignant melanoma rose rapidly in teenagers in Sweden during 1973-1992, while it remained low in younger children. To study the further trends and characteristics of melanoma in this young population, data on all cases in individuals under 20 years of age reported to the Swedish Cancer Registry during 1993-2002, and the corresponding pathology reports were examined. Seventy-nine cases were reported to the Registry. There were 24 males and 55 females. Most melanomas occurred on the trunk followed by the legs in both genders. The median tumor thickness was 0.8 mm. Children under age 15 had thicker melanomas than individuals aged 15-19. Superficial spreading melanoma was the most common histological subtype (43/78, 55%). The melanoma-specific 5-year survival rate was 90%. During 1993-2002, the age-standardized incidence fell to 3.6/million from 5.0/million in 1983-1992 (RR 0.74, 95% CI 0.58-0.92). The most pronounced decrease was for melanomas on the trunk in boys and on the legs in girls. The incidence for 15-19-year-old boys peaked for the cohort born between 1968 and 1972 and for girls between 1973 and 1977. The decrease in incidence may be a result of public health campaigns aiming at reducing sun exposure in childhood. A contributing effect from an increased immigration of individuals with darker complexions and at a lower melanoma risk is probable.

Keyword
childhood, adolescence, melanoma, population-based, epidemiology
National Category
Medical and Health Sciences
Identifiers
urn:nbn:se:liu:diva-14139 (URN)10.1002/ijc.22692 (DOI)
Available from: 2006-11-09 Created: 2006-11-09 Last updated: 2017-12-13

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