A primitive neuroectodermal tumor in an adult

A primitive neuroectodermal tumor in an adult AbstractRationale:Central nervous system primitive neuroectodermal tumors (CNS PNETs) mostly occur in children and present as cerebellar medulloblastoma. A few cases of PNETs occur in the cerebral hemisphere. The presence of a PNET in ventricles is extremely rare. The prognosis of CNS PNET is extremely poor, and the 5-year survival rate does not exceed 35%. In the present study, we describe the first case of a PNET in the ventricles with good prognosis.Patient concerns:The case of a 36-year-old man is reported, who presented with a progressively worsening headache for 2 months.Diagnoses:Magnetic resonance imaging (MRI) revealed multiple tubercula on the walls of the lateral and third ventricles. Histopathologic analysis revealed a hypercellular tumor with small round cells containing hyperchromatic nuclei and a high nucleus:cytoplasm ratio. The analysis was consistent with PNET.Interventions:Radiation therapy covering the entire craniospinal axis was administered, with Temozolomide for synchronous auxiliary treatment.Outcomes:The patient was follow-up for a year and showed no signs of recurrence.Lessons:We present the first CNS PNET located in the ventricles with good prognosis. In this case, radiotherapy with Temozolomide auxiliary treatment presented good efficacy and safety to treat PNET. Additional studies on biomarkers may be useful in predicting personalized therapeutic response. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Medicine Wolters Kluwer Health

A primitive neuroectodermal tumor in an adult

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Publisher
Wolters Kluwer Health
Copyright
Copyright © 2018 the Author(s). Published by Wolters Kluwer Health, Inc.
ISSN
0025-7974
eISSN
1536-5964
D.O.I.
10.1097/MD.0000000000009933
Publisher site
See Article on Publisher Site

Abstract

AbstractRationale:Central nervous system primitive neuroectodermal tumors (CNS PNETs) mostly occur in children and present as cerebellar medulloblastoma. A few cases of PNETs occur in the cerebral hemisphere. The presence of a PNET in ventricles is extremely rare. The prognosis of CNS PNET is extremely poor, and the 5-year survival rate does not exceed 35%. In the present study, we describe the first case of a PNET in the ventricles with good prognosis.Patient concerns:The case of a 36-year-old man is reported, who presented with a progressively worsening headache for 2 months.Diagnoses:Magnetic resonance imaging (MRI) revealed multiple tubercula on the walls of the lateral and third ventricles. Histopathologic analysis revealed a hypercellular tumor with small round cells containing hyperchromatic nuclei and a high nucleus:cytoplasm ratio. The analysis was consistent with PNET.Interventions:Radiation therapy covering the entire craniospinal axis was administered, with Temozolomide for synchronous auxiliary treatment.Outcomes:The patient was follow-up for a year and showed no signs of recurrence.Lessons:We present the first CNS PNET located in the ventricles with good prognosis. In this case, radiotherapy with Temozolomide auxiliary treatment presented good efficacy and safety to treat PNET. Additional studies on biomarkers may be useful in predicting personalized therapeutic response.

Journal

MedicineWolters Kluwer Health

Published: Feb 1, 2018

References

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