RESUMEN
A 2-year-old amerasian male with anhidrotic ectodermal dysplasia (Christ-Siemens-Touraine Syndrome) was admitted for status epilepticus and Mycobacteria avium-intracellulare infection. A computed tomography scan of the head revealed a mass thought to be a subdural hematoma. The patient died following overwhelming Mycobacteria avium-intracellular and Pseudomonas aeruginosa sepsis. Autopsy revealed extensive extramedullary hematopoiesis of the dura forming a tumor-like thickening with focal subdural hemorrhage. To our knowledge, this is the first report of extramedullary hematopoiesis of the cranial dura associated with anhidrotic ectodermal dysplasia.
Asunto(s)
Agammaglobulinemia/genética , Duramadre/patología , Displasia Ectodérmica/genética , Hematopoyesis Extramedular/genética , Agammaglobulinemia/patología , Preescolar , Glándulas Ecrinas/patología , Displasia Ectodérmica/patología , Células Madre Hematopoyéticas/patología , Humanos , Masculino , Bazo/patologíaRESUMEN
A pigmented choroid plexus papilloma was found in the left lateral ventricle of a 15-year-old white boy with a 10-year history of seizures. Grossly the lesion appeared finely lobular, orangish brown, rubbery, gritty, and friable. Microscopic examination revealed delicate papillary formations reminiscent of normal choroid plexus, with simple cuboidal to columnar epithelium overlying fibrovascular cores. Light- and electron-microscopic studies demonstrated that the neoplastic cell pigment consisted of both neuromelanin and lipofuscin. To our knowledge, this is the first reported case of neuromelanin pigmentation within a choroid plexus papilloma.
