We describe two new cases of Rosai-Dorfman disease with intrascrotal involvement. Also known as sinus histiocytosis with massive lymphadenopathy, this is a rare entity, usually presenting with massive bilateral cervical lymphadenopathy. It is associated with extranodal involvement in up to 40% of cases, commonly in the face and neck area. Isolated intrascrotal involvement with no lymphadenopathies is extremely rare, and its definitive diagnosis is histopathological. Although they are the most frequent type, not all intrascrotal lesions are neoplastic; therefore, it is important to know other possible etiologies and their radiogical features that might help in the diagnosis. We also review other cases of intrascrotal involvement reported in the literature, as well as a review clinical features, treatment and prognosis of this entity, and a differential diagnosis of solid intrascrotal lesions.
INTRODUCCIÓNLa Hiperplasia Suprarrenal Congénita (HSC) está causada por un defecto enzimático autosómico recesivo, que en más del 90% de los casos es por el déficit de 21 hidroxilasa. Cuando esta enzima falla, los niveles de dos hormonas esenciales como el cortisol y la aldosterona disminuyen y sus precursores aumentan (17-hidroxiprogesterona, androstenodiona y testosterona). La disminución de cortisol en sangre provoca la elevación de la hormona adrenocorticotropa (ACTH) y como consecuencia se produce hiperplasia suprarrenal. Existen varias formas de HSC por déficit de 21-hidroxilasa: la clásica (neonatal) con ó sin pérdida de sal, la forma tardía (con ó sin virilización) y la asintomática.
MRI is considered an excellent diagnostic test for the management of patients with testicular diseases of difficult diagnosis on ultrasound, contributing to define the lesion, a Leydig cell testicular tumor in this case.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.