Tumor neuroectodérmico primitivo de cavidad nasal / Primitive neuroectodermal tumor nasal cavity

Los tumores neuroectodérmicos primitivos son tumores muy infrecuentes. Derivan de células embrionarias de la cresta neural, y comparten características con los sarcoma de Ewing extraóseos. Se presentan habitualmente en niños y adultos jóvenes, su ubicación más frecuente es en la región toracopulmonar, abdomen y extremidades. Son tumores que presentan una agresividad importante, lo que condiciona un pronóstico sombrío. La ubicación en fosas nasales-cavidades perinasales es anecdótica, existiendo el reporte de tan sólo un caso en la literatura mundial de ubicación en cavidad nasal.

Primitive neuroectodermal tumors are rare malignancies arising from embryonic neural crest cells. They share characteristics with extraosseous Ewing sarcoma. They usually occur in children and young adults and the most common location is the thoracopulmonary region, abdomen and extremities. These tumors have significant aggressiveness, which determines their poor prognosis. The location in nasal fossa-paranasal cavities is anecdotal. Until now only one previous case has been reported in the located in the nasal fossa.

Asociación entre diastematomielia y meduloepitelioma: reporte de un caso y revisión de la literatura / Association between diastematomyelia and medullo epithelioma: case report and literature review

Aim: Case report of association between diastematomyelia and medulloepithelioma. Method: 14-year-old patient with lower back pain and recent neurological deficit in extremities. CT and MRI scans of the thoracolumbar spine revealed a diastematomyelia. Intraoperative examination confirmed the presence of a spinal dysraphism and associated tumor, which was almost completely resected. Result: Histopathological and immunohistochemical findings were consistent with medulloepithelioma. Her postoperative course was uneventful. Conclusion: Diastematomyelia may manifest during adolescence as lower back pain and neurological deficit. The association of this malformation with a neoplasia is extremely rare; the present case describes concomitance with medulloepithelioma.

Reporte de un caso de asociación de diastematomielia y meduloepitelioma. Método: Paciente de 14 años con dolor lumbar y déficit neurológico en extremidades de reciente instalación. En TAC y RNM de columna dorsolumbar se pesquisa una diastematomielia. La exploración intraoperatoria, comprueba la presencia de una disrafia espinal y un tumor asociado al defecto, que se reseca casi en su totalidad. Resultado: Los hallazgos histopatológicos e inmunohistoquímicos son concordante con meduloepitelioma. La paciente tuvo un postoperatorio satisfactorio. Conclusión: La diastematomielia se puede manifestar durante la adolescencia como dolor lumbar y déficit neurológico. La asociación de esta malformación, con una neoplasia es muy poco frecuente, el presente caso describe la concomitancia con un meduloepitelioma.

[Intraocular tumor diagnosed two years after perforating trauma]. / Tumor intraocular diagnosticado dos años después de un trauma perforante.

CASE REPORT: We describe the unusual diagnosis of a ciliary body medulloepithelioma by histopathology of a subretinal membrane obtained from vitreoretinal surgery of a 10-year-old boy. The patient had a history of perforating trauma OS 2 years earlier, and both fundus exam and B-scan ultrasound revealed only a retinal detachment with a subretinal membrane. No detectable mass was present. DISCUSSION: The membrane removed from underneath the peripheral retina revealed a blue cell tumor confirmed by histopathology and immunohistochemistry to be a primitive neuroectodermal tumor. Currently, the patient has been followed for 5 years with no signs of recurrence.

Primitive neuroectodermal peripheral tumour of the retroperitoneum.

Retroperitoneal primitive neuroectodermal tumour is an uncommon disease. The main treatment consists of surgical resection, which can require resection of great vessels, depending on the location. We present a single case of a 19-year-old male with a large tumour. To achieve R0 resection we needed to remove the vena cava. Venous flow reconstruction was performed with a cava prosthesis.

Tumor neuroectodérmico primitivo en mandíbula: reporte de un caso / Primary neuroectodermal tumor of the mandible: report of a case

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

Prognostic factors in children with supratentorial (nonpineal) primitive neuroectodermal tumors. A neurosurgical perspective from the Children's Cancer Group.

Supratentorial primitive neuroectodermal tumors (S-PNETs), which have also been called cerebral neuroblastomas, have been considered to be the hemispheric equivalent of posterior fossa medulloblastomas. Twenty-seven children with S-PNETs (excluding pineoblastomas) which were confirmed by central pathology review were treated on the CCG-921 protocol from 1986 to 1992. After operation, all patients were staged with CSF cytology and spinal myelography or magnetic resonance scans and were treated with craniospinal irradiation and chemotherapy. Data from these 27 patients have been reviewed to evaluate neurosurgical treatment, survival, and prognostic variables that correlate with survival. Overall survival at 5 years was 34% (SE 20%) and progression-free survival (PFS) was 31% (SE 18%), which is lower than the survival of patients with posterior fossa PNETs (medulloblastomas). PFS was significantly worse in children 1.5-3 years of age at diagnosis and in those with evidence of tumor dissemination at the time of diagnosis. Large preoperative tumors were more likely to be associated with greater than 1.5 cm2 residual tumor postoperatively. Neurosurgeons estimated that less than 1.5 cm2 of residual tumor was present in 52% of the cases; postoperative scans confirmed that in 58%. For children with less than 1.5 cm2 residual tumor, postoperative survival at 4.0 years was 40% (SE 22%); for those with greater than 1.5 cm2 residual tumor, survival was 13% (SE 8%). The difference did not reach statistical significance, due to small numbers in this series, though a trend did exist (p = 0.19). Large series will be required to clarify the effects of extent of resection on survival.