Os protuberans de Dermatofibrosarcoma (DFSP) são um tumor raro que afeta as camadas profundas da pele. Pode ocorrer em toda a idade, incluindo o período recém-nascido, mas é a mais freqüente entre as segundas e quintas décadas da vida. As mulheres têm uma incidência ligeira mais alta, e cresce mais rapidamente do que usual durante a gravidez. Nos neonatos, pode ser confundida por uma marca de nascença.
A incidência entre pretos é aproximadamente duas vezes a incidência que foi observada nos brancos. Contudo, a incidência no último grupo está aumentando, especialmente entre os residentes brancos de Havaí. Os factores de risco para este tumor devem ser identificada ainda, mas alguns casos foram notados para elevarar na pele que previamente foi queimada ou sofreu o traumatismo severo. A etiologia subjacente parece ser genética, com a translocação t17; 22 que estão sendo encontrados em 90% de todos os pacientes afetados. Esta mutação é encontrada geralmente como as peças levando de um cromossoma supranumerário do anel dos genes COL1A1 e PDGFB, do cromossoma 17 e 22 respectivamente, no formulário fundido.
Quando COL1A1 for responsável para a produção do tipo do colagénio – 1, fornecendo o apoio e a força estrutural a muitos tecidos, outro é um factor de crescimento poderoso que permita muitas pilhas de crescer, prolifera e diferencia-se. O gene fundido conduz à produção de grandes quantidades de PDGFB, que promove a proliferação do clone transformado das pilhas para formar um tumor de DFSP.
Características clínicas
A falta dos sintomas significa que a apresentação está atrasada na maioria de pacientes. Somente menos do que um quinto de todos os pacientes relatam com dor ou vermelhidão.
O primeiro sinal de DFSP é um delicado para firmar a correcção de programa da pele que muda pouco sobre os meses ou os anos na maioria dos casos. É cor de carne, avermelhado ou roxo, e um ou outro plano, ou aumentado, ou mesmo comprimido, em alguns pacientes. Pode assemelhar-se a uma espinha, a uma cicatriz ou a uma marca de nascença.
As margens são irregulares em muitos casos. Tem uma superfície um tanto brilhante, mas é associado mal nunca com a dor ou a ternura até as fases atrasadas. Tende a ser localmente agressivo, mas nao metastático. Após diversos anos, o tumor pode começar crescer mais agressivelmente, inchar ou tornar-se doloroso, ou sangrar. Os tumores adicionais podem aparecer à proximidade do original. A cor destes tumores pode mudar a enegrecido ou a acastanhado, e estes tumores novos crescem mais rapidamente do que original.
Nas fases iniciais, o tumor tem três formulários:
- A morphea-como o formulário que se assemelha pròxima a uma cicatriz ou a uma chapa
- Atrophoderma-como o formulário
- Angioma-como o formulário
Na fase protuberante, o nódulo aumentado é evidente, uns ou vários de acordo com a duração da circunstância.
O tamanho do tumor varia de 1-25 cm, embora é mais menos de 5 cm largamente. Ocorre o mais frequentemente sobre o torso (50-60%), as extremidades (35%), ou a região da cabeça e do pescoço (10-15%). Contudo, nenhuma parte da pele é isenta e DFSP foi relatado na vulva, no peito ou no escalpe também.
A lesão é geralmente primeira observada quando começa crescer ràpida, ou torna-se mais proeminente. Neste momento o tumor crescente levanta a pele superficial para formar uma correcção de programa firme ou elástico, sobre que a pele é esticada, pode se transformar proposta, se ulcerar, sangrar, ou sentir fixa ao tumor.
O tumor pode incorporar uma fase de crescimento rápido durante a gravidez.
Referências
- https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4863404/
- https://www.dermnetnz.org/topics/dermatofibrosarcoma-protuberans/
- https://ghr.nlm.nih.gov/condition/dermatofibrosarcoma-protuberans #
- https://www.aad.org/public/diseases/skin-cancer/dermatofibrosarcoma-protuberans#symptoms
- https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4706526/
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