ABSTRACT
El tumor neuroectodérmico es una neoplasia maligna de células redondas originada en la cresta neural. La ocurrencia de este tumor en cabeza y cuello es rara. Comunicamos una pacientes mexicana de 5 meses de edad con un tumor neuroectodérmico primitivo en estadio IV, en la mandíbula
Subject(s)
Humans , Female , Infant, Newborn , Mandibular Neoplasms , Neuroectodermal Tumors, Primitive/surgery , Neuroectodermal Tumors, Primitive/diagnosis , Neuroectodermal Tumors, Primitive/epidemiology , Biopsy , Drug Therapy , Mexico , Neoplasm Metastasis , Tomography, X-Ray ComputedABSTRACT
Proposta: a proposta desse estudo é a análise retrospectiva dos pacientes com diagnóstico de tumor neuroectodérmico primitivo (PNET), admitidos no Departamento de Pediatria do Hospital do Câncer no periodo de 1989 a 1996. Pacientes e métodos: Ttdos os 13 pacientes portadores de PNET foram analisados retrospectivamente utilizando uma ficha para obtenção dos dados epidemiológicos clínicos, terapêuticos e seguimento de cada paciente. Resultados: a cirurgia foi a primeira abordagem terapêutica em 2 pacientes. Quimioterapia foi administrada em 12 pacientes e radioterapia em 2. Dos 13 pacientes 5 estão vivos em seguimento médio de 48 meses após o término do tratamento. Conclusão: a análise dos 13 pacientes com PNET e revisão da literatura demonstram a agressividade desse tumor. Devido a raridade dessa patologia, estudos multiinstitucionais, podem ter papel importante para análise de fatores prognósticos e consequente abordagem terapêutica.
Subject(s)
Male , Female , Child, Preschool , Child , Adolescent , Humans , Sarcoma, Ewing , Soft Tissue Neoplasms , Neuroectodermal Tumors, Primitive/diagnosis , Neuroectodermal Tumors, Primitive/epidemiology , Neuroectodermal Tumors, Primitive/pathology , Neuroectodermal Tumors, Primitive/therapy , Follow-Up StudiesABSTRACT
Primitive neuroectodermal or neuroepithelial tumors are names used to describe neoplasias composed of undifferentiated cells resembling germinal cells of the embryonic neural tube. These tumors are small round cell malignancies of the neural crest origin arising outside the central and sympathetic nervous system. They are described as peripheral and central neuroectodermal tumors related to the original malignant cell. A great number of tumors are described under this classification in spite of the fact that there is no an universal acceptance that these small-cell neoplasms, regardless of their primary site, are derived from immature neuroectoderm tissue. Because one tumor resembles others in terms of its phenotypic expression, multiple specific studies such as clinical profile, ultrastructural, immunocytochemical, and cytogenetic features should be studied, since no single clinical or laboratory marker is by itself diagnostic. However, there is a chromosomal reciprocal translocation, t(11;22)(q24;q12), which is unique to Primitive Neuroectodermal Tumor (PNET)