RESUMEN
Amyloidosis results from deposition of fibrous and insoluble amyloid protein in extracellular spaces of organs and tissues. Amyloid deposition can be localized or systemic and either primary or secondary. We report a case of localized primary cutaneous nodular amyloidosis manifested by papular-nodular, reddish-brown lesions affecting the nasal area, without evidence of systemic involvement. Immunohistochemistry showed the presence of immunoglobulin kappa light chain.
Asunto(s)
Amiloidosis/patología , Enfermedades Cutáneas Metabólicas/patología , Enfermedades de la Piel/patología , Adulto , Humanos , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas , Inmunohistoquímica , MasculinoRESUMEN
Myxofibrosarcoma, previously known as malignant fibrous histiocytoma, myxoid variant, is a rare tumor of mesenchymal origin, composed of spindle cells and myxoid stroma. It mainly affects elderly people, involving the lower extremities and frequently extending to the dermis and subcutaneous tissue. The tumor presents high rates of recurrence, and a deep biopsy is required to obtain the correct diagnosis. We report a case of high-grade mixofibrossarcoma characterized by a rapidly growing tumor and the presence of marked cellular pleomorphism and an abundant myxoid matrix.
Asunto(s)
Rodilla/patología , Mixosarcoma/patología , Neoplasias Cutáneas/patología , Adulto , Humanos , MasculinoRESUMEN
A amiloidose resulta da deposição de proteína amiloide fibrosa e insolúvel em espaços extracelulares de órgãos e tecidos. O depósito da substância amiloide pode ser localizado ou sistêmico e pode ser de natureza primária ou secundária. Relataremos um caso de amiloidose localizada cutânea primária nodular, manifesta por lesões pápulo-nodulares, eritêmato-acastanhadas, acometendo a região nasal, sem evidência de acometimento sistêmico. O estudo imunoistoquímico demonstrou presença de imunoglobulinas de cadeia leve kappa.
Amyloidosis results from deposition of fibrous and insoluble amyloid protein in extracellular spaces of organs and tissues. Amyloid deposition can be localized or systemic and either primary or secondary. We report a case of localized primary cutaneous nodular amyloidosis manifested by papular-nodular, reddish-brown lesions affecting the nasal area, without evidence of systemic involvement. Immunohistochemistry showed the presence of immunoglobulin kappa light chain.
Asunto(s)
Adulto , Humanos , Masculino , Amiloidosis/patología , Enfermedades Cutáneas Metabólicas/patología , Enfermedades de la Piel/patología , InmunohistoquímicaRESUMEN
O mixofibrossarcoma, previamente conhecido como histiocitoma fibroso maligno, variante mixoide, é um tumor raro, de origem mesenquimal, composto por células fusiformes e estroma mixoide. Acomete mais idosos, envolvendo extremidades inferiores e estendendo-se, em sua maior parte, até a derme e o subcutâneo. Apresenta altas taxas de recorrência e para seu diagnóstico é fundamental a realização de uma biópsia profunda. Relataremos o caso de um mixofibrossarcoma de alto grau, caracterizado por lesão tumoral de crescimento rápido e pela presença de marcado pleomorfismo celular e componente mixoide em abundância.
Myxofibrosarcoma, previously known as malignant fibrous histiocytoma, myxoid variant, is a rare tumor of mesenchymal origin, composed of spindle cells and myxoid stroma. It mainly affects elderly people, involving the lower extremities and frequently extending to the dermis and subcutaneous tissue. The tumor presents high rates of recurrence, and a deep biopsy is required to obtain the correct diagnosis. We report a case of high-grade mixofibrossarcoma characterized by a rapidly growing tumor and the presence of marked cellular pleomorphism and an abundant myxoid matrix.
