RESUMEN
Dermatofibrosarcoma protuberans is a malignant and locally invasive tumor. It generally affects young adults. However, in rare but not exceptional cases, children can suffer from this disease. In the literature, there are only few studies on this pathology occurring in children. The main treatment is surgical excision with large margins. We studied in our series 15 cases of dermatofibrosarcoma in children between 1995 and 2008. The data were retrospectively collected. The aim of our study is to underline the best support for this pathology in children. The mean age at the moment of diagnosis was 13 years old. None of our patients underwent surgery for dermatofibrosarcoma removal before. The diagnosis was confirmed by biopsy and immunology by FISH method. In 60% of the cases, the lesion, which is very polymorphic, was on the trunk. In three cases, a skin trauma was noted in the patient's history. Surgical management consisted in tumor's excision with a 3 cm margin laterally associated with the removal of the first unaffected anatomical layer in depth. Reconstruction was adapted to the defect's width, depth and topography. The mean follow-up was 7.8 years. There were no relapses reported in a 3-year period at least. Complications were two scars dehiscences which needed skin graft for repair and a scarring alopecia reconstructed secondarily by a skin expansion protocol. No functional sequelae were reported in our series. As dermatofibrosarcoma in children presents the same clinical aspect and evolution as in adults, we treated it the same way. This diagnosis should not be ignored in children, and should be made early to give the patient the most effective support.
Asunto(s)
Dermatofibrosarcoma , Neoplasias Cutáneas , Adolescente , Niño , Preescolar , Dermatofibrosarcoma/patología , Dermatofibrosarcoma/cirugía , Femenino , Humanos , Lactante , Masculino , Estudios Retrospectivos , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/cirugíaRESUMEN
56 cases of granular cell tumour and 2 cases of granular cell congenital epulis have been studied morphologically, histochemically and immunohistochemically. The subject of this study is to evaluate the diagnosis interest of the PAS stain, determine the positivity of 4 antibodies (vimentin, NSE, PS100, NK1/C3) and confirm or not the neuroectodermic origin. The study showed that the microscopic findings are often sufficient. When the diagnosis is less evident, the PAS stain is not discriminant, but we can use the immunohistochemistry study: we confirmed the positivity of the 4 antibodies, but PS100 and NK1/C3 are the most constant. Only the 2 cases of granular cell congenital epulis always show negative immunoreactivity with S100 protein. About the histogenesis, our study confirm the neurogenic origin, but the hypothesis of a non neoplastic nature is raised.