Five patients had RT at the time of progression, and 5 received no RT whatever. Initial therapy consisted of surgical resection and chemotherapy in all patients and up-front radiotherapy (RT) in 5 patients. Patients and Methods: Fifteen children aged <18 years with non-pineal sPNETs diagnosed between 19 were identified. Purpose: To review a historical cohort of pediatric patients with supratentorial primitive neuroectodermal tumors (sPNET), to clarify the role of radiation in the treatment of these tumors. Radiation Is an Important Component of Multimodality Therapy for Pediatric Non-Pineal Supratentorial Primitive Neuroectodermal Tumors Conclusions According to current knowledge, primitive neuroectodermal tumors belong to the Ewing's sarcoma family, and the improvement of treatment outcome in our patient was due to dose-intensive neoadjuvant chemotherapy, surgery and consolidation chemotherapy in accordance with the protocol for bony Ewing's sarcoma. There was no trace of the tumor after four years of follow-up. Our patient underwent radical hysterectomy. Her clinical stage IB2 tumor was treated successfully with chemotherapy. Case presentation A 45-year-old Iranian woman presented to our facility with a primitive neuroectodermal tumor of the cervix uteri. Between 19, there were only nine cases reported in the English literature, with considerably different management policies.
Introduction Peripheral primitive neuroectodermal tumor of the cervix uteri is extremely rare. Primitive neuroectodermal tumor of the cervix: a case report From a practical, histologic point of view, these tumors are often indistinguishable from one another and are best thought of as primitive neuroectodermal tumors with or without differentiating features. Under the designation primitive neuroectodermal tumors are included medulloblastomas and tumors that may differentiate in other directions, such as medulloepithelioma, neuroblastoma, polar spongioblastoma, pineoblastoma, ependymoblastoma, retinoblastoma, and olfactory neuroblastoma. They occur primarily in children and young adults. Primitive neuroectodermal tumors are morphologically similar malignant tumors arising in intracranial and peripheral sites of the nervous system, showing varying degrees of cellular differentiation with a tendency to disseminate along cerebrospinal fluid pathways. Primitive neuroectodermal tumors of the central nervous system. Tipifarnib in Treating Young Patients With Recurrent or Progressive High-Grade Glioma, Medulloblastoma, Primitive Neuroectodermal Tumor, or Brain Stem GliomaĬhildhood High-grade Cerebral Astrocytoma Childhood Oligodendroglioma Recurrent Childhood Brain Stem Glioma Recurrent Childhood Cerebellar Astrocytoma Recurrent Childhood Cerebral Astrocytoma Recurrent Childhood Medulloblastoma Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumor Recurrent Childhood Visual Pathway and Hypothalamic Glioma Only one child is still in remission 86 months after diagnosis. Despite (high-dose) systemic chemotherapy and intraventricular mafosfamide, he died 21 months after diagnosis due to tumor although remission could be achieved. One child had a diffuse meningeal relapse 12 months after diagnosis. Two of the 4 children with complete tumor resection had local relapses 8 months after diagnosis and died after 14 and 18 months. The two children with incomplete resection died due to tumor progression after 7 and 10 months.
All had craniospinal irradiation and chemotherapy according to the HIT-91 protocol. In four of the six children radical resection could be achieved.
We saw within three years six children with stPNETs. Supratentorial primitive neuroectodermal tumors (stPNETs) are malignant tumors. Schmid, I Stachel, D Graubner, U B Elsner, R Schulze, S Pöllinger, B Goetz, C Haas, R J
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