Las neoplasias primarias múltiples (NPM) se definen como dos o más tumores malignos primarios de diferente origen en un mismo individuo. La causa de las NPM no se ha identificado en todos los casos, sin embargo puede deberse a factores ambientales, estilos de vida y/o factores genéticos. Se presenta el caso de una paciente que ha tenido ocho neoplasias (cinco malignas y tres benignas) que han sido tratadas con éxito y que actualmente se encuentra en controles, sin evidencia de recurrencia de enfermedad o alguna otra neoplasia de novo.Palabras clave: Tumores primarios múltiples. Síndrome de cáncer hereditario. Neoplasias metacrónicas.
La neoplasia quística mucinosa (NQM) primaria de hígado es una neoplasia poco usual de tipo epitelial que se caracteriza por la formación de quistes y que típicamente no tienen comunicación con los ductos biliares. Representa menos del 5% de todas las lesiones quísticas del hígado y existen solo 250 casos en la literatura mundial. Presentamos el caso de una paciente mujer de 23 años con una lesión de 13,5 x 10,2 cm, hipodensa, lobulada, con múltiples tabiques de hasta 2,5 mm de espesor y pequeñas imágenes quísticas en su interior, que condiciona dilatación de la vía biliar intrahepática a predominio izquierdo y del colé a tumoración correspondía a una neoplasia quística mucinosa de hígado.
Background: Clear cell sarcoma (CCS) of soft tissue is a rare disease with a high risk of metastasis to regional lymph nodes and distant organs and a poor survival rate. The aim of this study is to determine the rate of lymph node involvement, the effectiveness of treatment, the risk of recurrence and progression after surgery. Methods: We collected data from twenty patients diagnosed with CCS and treated in our institute, between 1998 and 2018. Subsequently, survival rates were determined according to local, regional and distant involvement, as well as the prognostic factors. Results: Twenty patients with CCS were included. The 2-year survival rate was 20%, and the 5-year survival rate was 5%. Patients with CCS with local stage and with tumor size < 5.0 cm were more likely to have a good survival rate. Conclusion: The initial management is crucial for the prognosis of the disease, with surgery being the mainstay of treatment. This study revealed a high rate of lymph node metastasis, so regional lymph node dissection should be done. Finally, the role of chemotherapy and/or radiotherapy for survival is still unclear. Keywords: Sarcoma; clear cell sarcoma; soft tissue tumors
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