Fibrosarcomatous dermatofibrosarcoma protuberans from scar following trauma

Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue neoplasm of low metastatic potential notable for its progressive growth and high rate of local recurrence after surgical excision. Fibrosarcomatous transformation of DFSP (FS-DFSP) is a rare variant characterized by higher rates of local recurrence and metastasis. Trauma has been hypothesized as a potential risk factor for the development of DFSP, although clear evidence has been lacking. In this study, we report a case of FS-DFSP that was found arising from a previously stable scar following a traumatic injury. A 49-year-old male was diagnosed with keloid scars following a motor vehicle accident where he sustained trauma. 12 years later, a large tumor developed immediately after a second traumatic event to the primary scar. Pathology of the excisional biopsy specimen demonstrated FS-DFSP with focal areas consistent with keloid and hypertrophic scar. This observation demonstrates the development of DFSP from underlying scar following a clear history of trauma. Furthermore, it suggests trauma as a possible trigger for the fibrosarcomatous transformation of DFSP.

Dermatofibrosarcoma Protuberans: A Study of Clinical, Pathologic, Genetic, and Therapeutic Features in Korean Patients

PURPOSE: Dermatofibrosarcoma protuberans (DFSP) carries a translocation resulting in the collagen type I alpha 1 (COL1A1)-platelet-derived growth factor beta (PDGFB) fusion gene, which is responsible for PDGFB activation. The purpose of this study is to evaluate the clinicopathological, genetic, and therapeutic features of DFSP in Korean patients. MATERIALS AND METHODS: Clinicopathological features of 37 patients with DFSP were reviewed. Multiplex reverse transcriptase-polymerase chain reaction (PCR) was carried out in 16 patients using formalin-fixed, paraffin-embedded tissues and specific primers for COL1A1 and PDGFB. RESULTS: The mean age of 37 patients was 37.4 years old. The most common tumor location was the trunk. All patients were treated primarily with surgery: 34 (91.7%) cases with Mohs micrographic surgery (MMS) and 3 (8.3%) cases with wide local excision. The median follow-up time was 33.7 months. Two patients, one in each treatment group, demonstrated local recurrence during the follow-up period. The COL1A1-PDGFB fusion gene was expressed in 14 (87.5%) cases, demonstrated by reverse transcriptase PCR analysis. No association was found among the different COL1A1-PDGFB fusion transcripts, the various histological subtypes and clinical features. CONCLUSION: Our results support the effectiveness of MMS in treating DFSP. The COL1A1-PDGFB fusion transcript was observed in 87.5% of patients. Therefore, COL1A1-PDGFB is a useful and accurate tool in diagnosing DFSP in Koreans.

Dermatofibrosarcoma Protuberans: A Study of Clinical, Pathologic, Genetic, and Therapeutic Features in Korean Patients

PURPOSE: Dermatofibrosarcoma protuberans (DFSP) carries a translocation resulting in the collagen type I alpha 1 (COL1A1)-platelet-derived growth factor beta (PDGFB) fusion gene, which is responsible for PDGFB activation. The purpose of this study is to evaluate the clinicopathological, genetic, and therapeutic features of DFSP in Korean patients. MATERIALS AND METHODS: Clinicopathological features of 37 patients with DFSP were reviewed. Multiplex reverse transcriptase-polymerase chain reaction (PCR) was carried out in 16 patients using formalin-fixed, paraffin-embedded tissues and specific primers for COL1A1 and PDGFB. RESULTS: The mean age of 37 patients was 37.4 years old. The most common tumor location was the trunk. All patients were treated primarily with surgery: 34 (91.7%) cases with Mohs micrographic surgery (MMS) and 3 (8.3%) cases with wide local excision. The median follow-up time was 33.7 months. Two patients, one in each treatment group, demonstrated local recurrence during the follow-up period. The COL1A1-PDGFB fusion gene was expressed in 14 (87.5%) cases, demonstrated by reverse transcriptase PCR analysis. No association was found among the different COL1A1-PDGFB fusion transcripts, the various histological subtypes and clinical features. CONCLUSION: Our results support the effectiveness of MMS in treating DFSP. The COL1A1-PDGFB fusion transcript was observed in 87.5% of patients. Therefore, COL1A1-PDGFB is a useful and accurate tool in diagnosing DFSP in Koreans.

Mongolian spots.

Mongolian spots (MS) are birthmarks that are present at birth and their most common location is sacrococcygeal or lumbar area. Lesions may be single or multiple and usually involve < 5% total body surface area. They are macular and round, oval or irregular in shape. The color varies from blue to greenish, gray, black or a combination of any of the above. The size varies from few to more than 20 centimetres. Pigmentation is most intense at the age of one year and gradually fades thereafter. It is rarely seen after the age of 6 years. Aberrant MS over occiput, temple, mandibular area, shoulders and limbs may be confused with other dermal melanocytoses and bruises secondary to child abuse, thus necessitating documentation at birth. Although regarded as benign, recent data suggest that MS may be associated with inborn errors of metabolism and neurocristopathies. Mongolian spots usually resolve by early childhood and hence no treatment is generally needed if they are located in the sacral area. However, sometimes it may be required for extrasacral lesions for cosmesis.

Perfil epidemiológico dos cidadãos de Florianópolis quanto à exposição solar / Epidemiologic profile of sun exposure in Florianópolis citizens

Objetivo: descrever as características de exposição e medidas de proteção solar contra câncer da pele no município de Florianópolis, estado de Santa Catarina, Brasil. Metodologia: estudo transversal, do Inquérito Domiciliar sobre Comportamento de Risco e Morbidade Referida de Doenças e Agravos não Transmissíveis. Foram calculadas medidas de frequência e aplicado o teste qui-quadrado. Resultados: dentre os 851 entrevistados, 77,1 por cento declararam se expor ao Sol. A exposição solar foi mais freqüente entre homens, jovens, solteiros e fisicamente ativos. Entre as medidas de proteção solar adotadas pela população exposta ao Sol, a mais utilizada foi o filtro solar (36,7 por cento das mulheres e 10,5 por cento dos homens). A utilização de filtro solar foi positivamente associada à escolaridade. Conclusão: a frequência de exposição ao Sol sem nenhum tipo de proteção foi elevada. Estes dados visam a colaborar na implementação de políticas públicas específicas para o fator de risco prevenível na carcinogênese do câncer da pele: a exposição inadequada ao Sol.

Objective: to describe the characteristics of exposure and sun protection measures in Florianopolis/SC. Methodology: it is a cross-sectional study with sun exposure data from the city of Florianópolis househould survey Risk Behavior and Reported Morbity of Diseases and Harm non-transmissibles. Results: it was used simple frequencies and proportions with chi-squared test. For this module 851 people were interviewed, while 77.1 per cent are exposed to the sun, more frequent among men, young, single and physically active. Among the sun exposure protection factors usedby the population exposed to the sun, sunscreen was the most used one (36,7 per cent of women and 10,5 per cent of men make use). Conclusion: high scholarity level was associated with the use of sunscreen. The obtained results can colaborate on the implementation of specific public policies for the most preventable risk factor on skin cancer carcinogenesis: the inadequate exposure to sun.

Nevos melanocíticos numa comunidade de origem predominantemente holandesa no Brasil (1999-2007) / Melanocytic nevi in a Brazilian community of predominantly Dutch descent (1999-2007)

FUNDAMENTOS: Estudos epidemiológicos têm demonstrado relação significativa entre nevos melanocíticos e melanoma cutâneo. OBJETIVO: Acompanhar o desenvolvimento de nevos melanocíticos nos alunos de uma escola composta, majoritariamente, por descendentes de holandeses e a influência do meio ambiente sobre esses indivíduos com características fenotípicas semelhantes às de seus antepassados. MÉTODOS: Em 1999, iniciou-se estudo coorte para contagem de nevos melanocíticos nos 282 alunos entre três e 17 anos, sendo 53,9 por cento meninos. Após cinco anos, realizou-se novo exame em 148 alunos entre oito e 22 anos, dos quais 49,3 por cento eram meninos. Analisou-se a relação da idade, sexo, fotótipo, cor dos olhos, cor dos cabelos e etnia dos alunos e dos pais com a presença de nevos melanocíticos no início e no final do estudo. RESULTADOS: Houve aumento significativo de nevos melanocíticos e nevos displásicos no reexame. Os meninos tiveram mais nevos melanocíticos (áreas cobertas e expostas) do que as meninas. A análise de probabilidade para razão de risco revelou que os meninos têm mais chance de desenvolver nevos melanocíticos do que as meninas, assim como os de etnia não miscigenada e miscigenada e com cabelos claros têm mais que os alunos de outras etnias e com cabelos escuros. Os que apresentam fotótipo I são mais propensos a desenvolver nevos melanocíticos nas áreas cobertas do que os que têm fotótipos II e III. CONCLUSÕES: Os dados demonstram que os indivíduos de etnia holandesa tiveram maior probabilidade de desenvolver nevos melanocíticos do que os outros grupos étnicos.

BACKGROUNDS: Epidemiological studies have shown a significant relationship between melanocytic nevi and cutaneous melanoma. OBJECTIVE: To evaluate the development of melanocytic nevi in the pupils of a school composed primarily of children of Dutch descent and to assess the effect of environmental factors on these individuals whose phenotypic characteristics were similar to those of their ancestors. METHODS: In 1999 a cohort study was initiated to count the number of melanocytic nevi in 282 pupils of 3 to 17 years of age, 53.9 percent of whom were boys. Five years later a repeat exam was conducted in 148 students of 8 to 22 years of age, 49.3 percent of whom were males. The association between the age, skin phototype, eye color, hair color and ethnic group of the pupils and their parents and the presence of melanocytic nevi was analyzed at the beginning and at the end of the study. RESULTS: There was a significant increase in cases of melanocytic nevi and dysplastic nevi at the follow-up examination. The number of melanocytic nevi was greater in boys than in girls in both covered and exposed areas of skin. Likelihood analysis calculated using the odds ratio showed that boys were more likely to develop melanocytic nevi than girls and that the children of non-mixed and mixed race with lighter hair were more likely to develop melanocytic nevi than those of other ethnic groups and those with dark hair. Children with skin phototype I were more likely to develop melanocytic nevi in covered areas of skin compared to those with skin types II or III. CONCLUSIONS: These data show that individuals of Dutch descent were more likely to develop melanocytic nevi than individuals of other ethnic origins.

Prevalence of precancerous skin lesions and non-melanoma skin cancer in Japanese-Brazilians in Bauru, São Paulo State, Brazil / Prevalência de lesões pré-neoplásicas e tumores cutâneos não melanomas em nipo-brasileiros residentes na cidade de Bauru, São Paulo, Brasil

Precancerous lesions and skin cancer are infrequent in Asians, and have received little documentation in the literature. Brazil has the world’s largest contingent of Japanese immigrants andtheir descendants, and 70% live in the State of São Paulo. The prevalence of such skin lesions in Japanese-Brazilians is unknown. This study aimed to assess the prevalence of actinic keratosesand non-melanoma skin cancer in first and second-generation Japanese-Brazilians over 30 years of age, without miscegenation, living in the city of Bauru, São Paulo State, in 2006. Of the 567 Japanese-Brazilians that underwent dermatological examination, actinic keratosis was diagnosed in 76, with a mean age of 68.9 years, and a single case of basal cell carcinoma was detected in a 39-year-old female patient. In Japan, prevalence of actinic keratosis varies from 0.76% to 5%,and the incidence of non-melanoma skin cancer is 1.2 to 5.4/100 thousand. Japanese-Brazilians from Bauru showed a 13.4% prevalence of actinic keratoses and earlier age at onset. Proximity tothe Equator and a history of farming contribute to these higher rates. Presence of solar melanosis was associated with a 1.9-fold risk of developing actinic keratosis.

Manifestações cutâneas pré-neoplásicas e neoplásicas em asiáticos são infreqüentes e pouco documentadas.O Brasil possui o maior contingente de imigrantes japoneses e 70% deles residem no Estado de São Paulo. A prevalência dessas lesões em nipo-brasileiros é desconhecida. O presente estudo tem como objetivo avaliara prevalência de queratoses actínicas e tumores cutâneos não melanomas em nipo-brasileiros acima de trinta anos de 1a geração ou 2a geração, sem miscigenação, residentes na cidade de Bauru, no ano de 2006. Dos 567 nipo-brasileiros submetidos a exame dermatológico, diagnosticou-se queratose actínica em 76 pacientes, com média de idade de 68,9 anos, e únicocarcinoma basocelular em paciente do sexo feminino de 39 anos. No Japão, a prevalência de queratose actínica é de 0,76% a 5% e a incidência de tumores cutâneos não melanomas é de 1,2 a 5,4/100 mil. Os nipo-brasileiros de Bauru apresentaram prevalência de13,4% de queratoses actínicas e idade mais precoce de aparecimento. Proximidade com o Equador e atividadesrurais contribuem para esses achados. A presença de melanose solar demonstrou risco 1,9 vez maior de desenvolver queratose actínica.

Múltiplos melanomas malignos em área de cicatriz de queimadura: relato de caso e revisão da literatura / Multiple malignant melanomas at burn scar: case report and review of the literature'

Alterações neoplásicas malignas podem ocorrer em aproximadamente 2 por cento dos casos de cicatrizes de queimaduras. As lesões neoplásicas se apresentam mais freqüentemente como carcinoma espinocelular. Neste artigo descreve-se o caso de uma paciente do sexo feminino, na sexta década de vida, com melanomas malignos múltiplos que se desenvolveram em uma antiga área de cicatriz de queimadura no tronco. Melanomas malignos múltiplos que se desenvolvem em região de cicatriz de queimadura são extremamente raros, e apenas quatro casos foram descritos na literatura até o presente momento.

Malignant changes in burn scars can develop in approximately 2 percent of patients. The majority of these lesions are squamous cell carcinomas. In this article we present the case of a female patient, in the sixth decade, with multiple melanomas arising in the setting of an extensive old truncal burn scar. Multiple malignant melanomas arising in a burn scar are extremely uncommon and only four previous cases have been described in literature.