Asunto(s)
Braquiterapia , Cuerpo Ciliar/patología , Radioisótopos de Yodo/uso terapéutico , Tumores Neuroectodérmicos Primitivos/terapia , Procedimientos Quirúrgicos Oftalmológicos , Radioterapia Adyuvante , Neoplasias de la Úvea/terapia , Biomarcadores de Tumor/análisis , Preescolar , Terapia Combinada , Femenino , Humanos , Tumores Neuroectodérmicos Primitivos/patología , Tumores Neuroectodérmicos Primitivos/radioterapia , Tumores Neuroectodérmicos Primitivos/cirugía , Neoplasias de la Úvea/patología , Neoplasias de la Úvea/radioterapia , Neoplasias de la Úvea/cirugíaRESUMEN
We have evaluated the response rate and survival utilizing intensified chemotherapy followed by myeloablative chemotherapy with autologous hematopoietic cell rescue (AuHCR) and adjuvant radiation therapy in six young children with newly diagnosed brainstem primitive neuroectodermal tumors (bstPNET). Following maximum surgical resection of the tumor, patients received high dose induction chemotherapy including vincristine, cisplatin, cyclophosphamide, and etoposide. Eligible patients received a single cycle of myeloablative chemotherapy followed by AuHCR. Two patients survive at least 32 months with stable disease. This approach provides an alternative for young patients with bstPNET who in prior reports have had a uniformly fatal prognosis.