Assuntos
Antineoplásicos , Carcinoma Basocelular , Neoplasias Cutâneas , Humanos , Dermatologistas , Carcinoma Basocelular/terapia , Carcinoma Basocelular/tratamento farmacológico , Neoplasias Cutâneas/terapia , Neoplasias Cutâneas/tratamento farmacológico , Antineoplásicos/uso terapêutico , Anilidas/uso terapêutico , Proteínas HedgehogRESUMO
Basal cell carcinoma (BCC) is the most common human cancer, with approximately 3.6 million cases diagnosed each year. About 2000 deaths annually in the United States are attributed to basal and squamous cell skin cancers. There is a direct link between ultraviolet exposure and the development of BCC, as UV exposure damages DNA and induces mutations in tumor suppressor genes. Aberrations in the hedgehog pathway can also result in BCC, highlighted by the fact that most cases of sporadic BCCs have been found to have mutations in different genes involved in the hedgehog pathway. There are several genetic syndromes that are associated with BCCs, including basal cell nevus syndrome, xeroderma pigmentosum, Bazex-Dupré-Christol syndrome, Rombo syndrome, and Oley syndrome. Other risk factors include age, male gender, occupational hazards, radiation, and immunosuppression. BCCs are not typically staged but are instead stratified by their risk of recurring or metastasizing. Locally advanced BCCs are those tumors that are not amenable to surgery or radiation therapy.
Assuntos
Carcinoma Basocelular , Neoplasias Cutâneas , Carcinoma Basocelular/diagnóstico , Carcinoma Basocelular/epidemiologia , Carcinoma Basocelular/genética , Dermatologistas , Proteínas Hedgehog/metabolismo , Humanos , Masculino , Recidiva Local de Neoplasia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/genéticaRESUMO
ABSTRACT: S100-negative CD1a-positive cutaneous histiocytosis is an exceedingly rare histiocytosis that is defined histopathologically by a dense dermal infiltrate of ovoid mononuclear cells with grooved nuclei and ample cytoplasm with variable nuclear atypia and mitoses that are immunohistochemically positive for CD1a and negative for S100 and CD207 (langerin). The histogenesis of S100-negative CD1a-positive histiocytosis is unclear, and its precursor cell has yet to be characterized. Although all cases thus far have been described as benign and occasionally self-resolving, the clinical course and outcome of this disease are not fully understood. This case expands the spectrum of disease associated with S100-negative CD1a-positive histiocytosis given its malignant course.
