ABSTRACT
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.
Subject(s)
Humans , Male , Middle Aged , Neuroectodermal Tumors, Primitive/diagnosis , Neuroectodermal Tumors, Primitive/surgery , Nose Neoplasms/diagnosis , Nose Neoplasms/surgeryABSTRACT
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.
Subject(s)
Humans , Adolescent , Female , Neural Tube Defects/complications , Neural Tube Defects/diagnosis , Spinal Cord Neoplasms/surgery , Spinal Cord Neoplasms/complications , Neuroectodermal Tumors, Primitive/surgery , Neuroectodermal Tumors, Primitive/complications , Low Back Pain/etiology , Spinal Cord Neoplasms/diagnosis , Spinal Dysraphism , Neuroectodermal Tumors, Primitive/diagnosisSubject(s)
Antineoplastic Agents/therapeutic use , Humans , Kidney , Male , Neuroectodermal Tumors, Primitive/diagnosis , Neuroectodermal Tumors, Primitive/drug therapy , Neuroectodermal Tumors, Primitive/pathology , Neuroectodermal Tumors, Primitive/radiotherapy , Neuroectodermal Tumors, Primitive/surgery , Young AdultSubject(s)
Heart Atria/pathology , Adult , Female , Heart Atria/surgery , Humans , Kidney Neoplasms/complications , Kidney Neoplasms/pathology , Kidney Neoplasms/surgery , Neuroectodermal Tumors, Primitive/complications , Neuroectodermal Tumors, Primitive/pathology , Neuroectodermal Tumors, Primitive/surgery , Prognosis , Thrombosis/pathology , Thrombosis/surgery , Tomography, X-Ray ComputedABSTRACT
Primitive neuroectodermal tumor (PNET) is a small round cell malignant tumor of neuroectodermal origin. Most of the PNETs occur in the central nervous system (CNS). PNETs recognized outside of CNS are diagnosed as peripheral PNET (pPNET). This tumor which expresses MIC-2 gene (CD99) seems to be least aggressive after complete tumor resection. We describe a rare case of PNET in a young girl.
Subject(s)
Biopsy , Child , Diagnosis, Differential , Female , Humans , Neuroectodermal Tumors, Primitive/diagnosis , Neuroectodermal Tumors, Primitive/surgery , Ophthalmologic Surgical Procedures/methods , Orbital Neoplasms/diagnosis , Orbital Neoplasms/surgery , Tomography, X-Ray ComputedABSTRACT
Peripheral primitive neuroectodermal tumors are malignant small round cell tumors which occur rarely in children we retrospectively reviwed the clinical data and radiological studies of four such cases. the youngest child had a pelvic tumor and each of two children had a chest wall tumor. the fourth patient had a bone swelling tumors were all diagnosed by histological and immunhistochimical examinations most patients had combined treatment with chemotherapy surgery and radiation therapy. Best results were obtained in two cases and another died. in this report the clinical aspects of PNET in children are described reviewing the presentation of the disease in four patients and detailing the treatment strategies
Subject(s)
Humans , Male , Female , Brain Neoplasms/pathology , Magnetic Resonance Imaging , Brain Neoplasms/diagnostic imaging , Radiography, Thoracic , Neuroectodermal Tumors, Primitive/therapy , Radiotherapy , Immunohistochemistry , Neuroectodermal Tumors, Primitive/surgery , Carcinoma, Small CellABSTRACT
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