Asunto(s)
Neoplasias de la Mama Masculina/complicaciones , Neurofibromatosis 1/complicaciones , Enfermedad de Paget Mamaria/complicaciones , Anciano , Axila , Biopsia , Neoplasias de la Mama Masculina/patología , Neoplasias de la Mama Masculina/cirugía , Diagnóstico Diferencial , Humanos , Escisión del Ganglio Linfático , Masculino , Mastectomía Radical , Enfermedad de Paget Mamaria/patología , Enfermedad de Paget Mamaria/cirugíaRESUMEN
Multiple endocrine neoplasia type 2B/3 is characterized by multiple mucosal neuromas, a marfanoid appearance, medullary thyroid carcinoma, pheochromocytoma, gastrointestinal ganglioneuromatosis, and thickened corneal nerves. This rare syndrome is inherited in an autosomal dominant pattern. Early recognition followed by appropriate screening and treatment can be life-saving.
Asunto(s)
Neoplasia Endocrina Múltiple Tipo 2b/genética , Neoplasias de las Glándulas Suprarrenales/genética , Neoplasias de las Glándulas Suprarrenales/patología , Carcinoma/genética , Carcinoma/patología , Córnea/inervación , Ganglioneuroma/genética , Ganglioneuroma/patología , Neoplasias Gastrointestinales/genética , Neoplasias Gastrointestinales/patología , Humanos , Síndrome de Marfan/patología , Neoplasia Endocrina Múltiple Tipo 2b/diagnóstico , Neoplasia Endocrina Múltiple Tipo 2b/patología , Neuroma/genética , Neuroma/patología , Feocromocitoma/genética , Feocromocitoma/patología , Neoplasias de la Tiroides/genética , Neoplasias de la Tiroides/patologíaRESUMEN
Mycosis fungoides and Sézary syndrome, collectively referred to as cutaneous T-cell lymphoma, are non-Hodgkin's lymphomas that initially appear in the skin. Early-stage disease, limited to the skin, is best treated with sequential topical therapies such as topical nitrogen mustard, psoralen phototherapy (PUVA), or total-skin electron beam therapy. Photopheresis is the first line of therapy for the patient with erythroderma. Systemic therapy is generally reserved for patients with refractory disease and patients who initially present with extracutaneous involvement. Although there are several treatment options for cutaneous T-cell lymphoma, there have been few randomized comparative trials.