ABSTRACT
The prognosis for many pediatric and young adult patients with solid tumors that have metastasized at the time of diagnosis or have relapsed after therapy remains very poor. The steep dose-response curve of many of these tumors to alkylating agents makes myeloablative chemotherapy followed by autologous stem cell transplantation (ASCT) an attractive potential therapy. The role of ASCT for these high-risk patients is yet to be conclusively determined. We have transplanted 36 patients on two consecutive protocols with a variety of histological diagnoses. Overall survival (OS) was 63% (95% CI: 47-79%) at 1 year and 33% (95% CI: 16-50%) at 3 years. Patients with a diagnosis of Ewing's sarcoma (ES) or desmoplastic small round cell tumor (DSRCT) had significantly better survival than those with other diagnoses with estimated 3-year OS of 54% (95% CI: 29-79%) for this group of patients (P = 0.03). There were two transplant-related deaths both attributable to hepatic veno-occlusive disease. Median follow-up among survivors is 3.5 years (range: 0.6-7.9 years). These data justify continued investigation of ASCT as a consolidation therapy in patients with metastatic or relapsed ES and DSRCT.
Subject(s)
Bone Neoplasms/mortality , Bone Neoplasms/therapy , Neoplasm Recurrence, Local/mortality , Neoplasm Recurrence, Local/therapy , Sarcoma, Ewing/mortality , Sarcoma, Ewing/therapy , Adolescent , Adult , Bone Neoplasms/complications , Bone Neoplasms/pathology , Child , Child, Preschool , Disease-Free Survival , Female , Fibroma, Desmoplastic/complications , Fibroma, Desmoplastic/mortality , Fibroma, Desmoplastic/pathology , Fibroma, Desmoplastic/therapy , Follow-Up Studies , Hepatic Veno-Occlusive Disease/etiology , Hepatic Veno-Occlusive Disease/mortality , Humans , Male , Neoplasm Recurrence, Local/complications , Neoplasm Recurrence, Local/pathology , Risk Factors , Sarcoma, Ewing/complications , Sarcoma, Ewing/pathology , Stem Cell Transplantation/methods , Stem Cell Transplantation/mortality , Survival Rate , Transplantation, AutologousABSTRACT
El tumor desmoplásico de células redondas pequeñas (TDCRP) es una neoplasia maligna que se presenta típicamente como una masa abdominopélvica en un paciente varón durante la segunda o tercera década de la vida. Presentamos dos casos de esta rara patología, describiendo los hallazgos en la tomografía computarizada (TC) y en la anatomía patológica. Realizamos una breve revisión de la bibliografía, haciendo hincapié en las manifestaciones radiológicas de dicha neoplasia (AU)
