ABSTRACT
Los linfomas testiculares primarios representan aproximadamente el 5% de todas las neoplasias testiculares malignas, el 1% de los linfomas no Hodgkin y el 4% de los linfomas no Hodgkin extranodales. Se trata por tanto de neoplasias infrecuentes aunque muy agresivas en la mayoría de los casos ya que entre el 80% y el 90% de los linfomas testiculares pertenecen al subtipo no Hodgkin B difuso de células grandes. Esta neoplasia constituye el tumor maligno testicular más frecuente en ancianos. La presentación clínica típica es una masa testicular unilateral acompañada a veces de hidrocele y dolor agudo escrotal. A pesar de que existe buena tasa de respuestas a la quimioterapia, sobre todo en estadios tempranos, el porcentaje de recaídas es alto y el pronóstico muy pobre. Presentamos aquí 3 casos y estudiamos la histología y la inmunohistoquímica, y revisamos la literatura en este sentido repasando las nuevas perspectivas terapéuticas (AU)
Primary testicular lymphoma accounts for approximately 5% of malignant tumours of the testis, 1% of all non-Hodgkin lymphomas and 4% of extra-nodal non-Hodgkin's lymphoma. 80-90% of testicular lymphomas belong to the diffuse large B-cell lymphoma group which is clinically very aggressive. Although infrequent, it is the most common malignant testicular tumour in the elderly. The typical presentation is a unilateral testicular mass sometimes accompanied by acute scrotal pain or a hydrocele. Although the initial response to chemotherapy may be favorable, relapses are frequent and the overall prognosis of this neoplasm is very poor. We present three cases with histopathological and immunohistochemical studies, a review the literature and new therapeutic perspectives (AU)
Subject(s)
Humans , Male , Middle Aged , Aged , Lymphoma, Large B-Cell, Diffuse/pathology , Testicular Neoplasms/pathology , Immunohistochemistry/methods , Lymphoma, Non-Hodgkin/pathology , Orchiectomy , BiopsyABSTRACT
Darier disease is a genodermatosis with a pattern of autosomal dominant inheritance, which occasionally presents by following a linear or segmental pattern. We present the case of a 22-year-old woman who had presented for three years with small, linearly distributed, yellowish-brown papules on the right lower extremity, with no other skin, mucous membrane or appendage involvement. She had no personal or family history of any interest. Histologically, suprabasal acantholysis and abundant dyskeratotic cells were seen. All of this data led to the diagnosis of linear Darier disease.
Subject(s)
Darier Disease/pathology , Adult , Calcium-Transporting ATPases/deficiency , Calcium-Transporting ATPases/genetics , Chromosomes, Human, Pair 12/genetics , Darier Disease/diagnosis , Darier Disease/genetics , Female , Humans , Leg , Mosaicism , Sarcoplasmic Reticulum Calcium-Transporting ATPasesABSTRACT
La enfermedad de Darier es una genodermatosis con un patrón de herencia autosómico dominante que en ocasiones se presenta siguiendo un patrón lineal. Presentamos el caso de mujer de 22 años que presentaba desde hacía 3 años unas pequeñas pápulas marrones-amarillentas de distribución lineal en la extremidad inferior derecha sin otra afectación ni cutánea, ni mucosa ni de anejos. No presentaba antecedentes personales ni familiares de interés. Histológicamente se apreciaba una acantolisis suprabasal y abundantes células disqueratósicas. Con todos estos datos se llegó al diagnóstico de enfermedad de Darier lineal
Darier disease is a genodermatosis with a pattern of autosomal dominant inheritance, which occasionally presents by following a linear or segmental pattern. We present the case of a 22-year-old woman who had presented for three years with small, linearly distributed, yellowish-brown papules on the right lower extremity, with no other skin, mucous membrane or appendage involvement. She had no personal or family history of any interest. Histologically, suprabasal acantholysis and abundant dyskeratotic cells were seen. All of this data led to the diagnosis of linear Darier disease
