Tumor neuronal multinodular y vacuolizante cerebral, una entidad rara. Descripción de un nuevo caso y revisión sistemática de la literatura

Translated title of the contribution: Multinodular and vacuolating neuronal tumor of the cerebrum. A rare entity. New case and review of the literature

Lain Hermes Gonzalez-Quarante, Fernando Ruiz-Juretschke, Emma Sola Vendrell, Oscar Lucas Gil de Sagredo del Corral, Vijay Agarwal, Roberto Garcia-Leal

Research output: Contribution to journalShort survey

8 Citations (Scopus)

Abstract

Background Multinodular and vacuolating neuronal tumor has been recently described and included in the World Health Organization Classification of Tumors of The Central Nervous System, even though its consideration as a true tumor is controversial. Patients with these lesions usually present with refractory seizures and inconclusive imaging findings that may be confused with other more common diagnoses such as dysembryoplastic neuroepithelial tumors or low-grade gliomas. Therefore, surgical resection is warranted to reach a pathologic diagnosis and seizure control. To the best of our knowledge, only 16 cases have been published in the English literature. Case description We present the case of a 52-year-old male who presented at our institution with a 2-year-history of absence of seizures. Brain MRI showed a T2-hyperintense lesion with no contrast enhancement affecting his temporal lobe. Temporal craniotomy and microsurgical resection was scheduled. The procedure was uneventful and a grayish, gluey mass was sent for pathologic analysis. The tumor was formed by immature neuronal cells organized in nodules with a vacuolated matrix. A thorough immunohistochemical analysis showed positivity for: Protein Gene Product 9.5. ATRX. OLIG2. SOX10. p16. Nestin. Synaptophysin. The findings were consistent with multinodular and vacuolating neuronal tumor. The patient has been seizure-free after surgery and with no signs of tumor progression. Conclusion We present a thorough review addressing this uncommon tumor along with a description of the 17th reported case of MVNT, a tumor that was described for the first time in 2013. Further studies and case studies are necessary to establish a well-defined morphological and immunohistochemical profile along with knowledge about its natural history.

Original languageSpanish
Pages (from-to)44-55
Number of pages12
JournalNeurocirugia
Volume29
Issue number1
DOIs
StatePublished - Jan 2018
Externally publishedYes

Fingerprint

Cerebrum
Neoplasms
Seizures
Neuroepithelial Neoplasms
Literature
Absence Epilepsy
Nestin
Central Nervous System Neoplasms
Synaptophysin
Craniotomy
Temporal Lobe
Natural History
Glioma
Brain
Proteins

Keywords

  • Brain tumor
  • Epilepsy
  • Low grade glioma
  • Multinodular and vacuolating neuronal tumor
  • Seizures

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

Cite this

Gonzalez-Quarante, L. H., Ruiz-Juretschke, F., Sola Vendrell, E., Gil de Sagredo del Corral, O. L., Agarwal, V., & Garcia-Leal, R. (2018). Tumor neuronal multinodular y vacuolizante cerebral, una entidad rara. Descripción de un nuevo caso y revisión sistemática de la literatura. Neurocirugia, 29(1), 44-55. https://doi.org/10.1016/j.neucir.2017.08.003

Tumor neuronal multinodular y vacuolizante cerebral, una entidad rara. Descripción de un nuevo caso y revisión sistemática de la literatura. / Gonzalez-Quarante, Lain Hermes; Ruiz-Juretschke, Fernando; Sola Vendrell, Emma; Gil de Sagredo del Corral, Oscar Lucas; Agarwal, Vijay; Garcia-Leal, Roberto.

In: Neurocirugia, Vol. 29, No. 1, 01.2018, p. 44-55.

Research output: Contribution to journalShort survey

Gonzalez-Quarante, LH, Ruiz-Juretschke, F, Sola Vendrell, E, Gil de Sagredo del Corral, OL, Agarwal, V & Garcia-Leal, R 2018, 'Tumor neuronal multinodular y vacuolizante cerebral, una entidad rara. Descripción de un nuevo caso y revisión sistemática de la literatura', Neurocirugia, vol. 29, no. 1, pp. 44-55. https://doi.org/10.1016/j.neucir.2017.08.003
Gonzalez-Quarante, Lain Hermes ; Ruiz-Juretschke, Fernando ; Sola Vendrell, Emma ; Gil de Sagredo del Corral, Oscar Lucas ; Agarwal, Vijay ; Garcia-Leal, Roberto. / Tumor neuronal multinodular y vacuolizante cerebral, una entidad rara. Descripción de un nuevo caso y revisión sistemática de la literatura. In: Neurocirugia. 2018 ; Vol. 29, No. 1. pp. 44-55.
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abstract = "Background Multinodular and vacuolating neuronal tumor has been recently described and included in the World Health Organization Classification of Tumors of The Central Nervous System, even though its consideration as a true tumor is controversial. Patients with these lesions usually present with refractory seizures and inconclusive imaging findings that may be confused with other more common diagnoses such as dysembryoplastic neuroepithelial tumors or low-grade gliomas. Therefore, surgical resection is warranted to reach a pathologic diagnosis and seizure control. To the best of our knowledge, only 16 cases have been published in the English literature. Case description We present the case of a 52-year-old male who presented at our institution with a 2-year-history of absence of seizures. Brain MRI showed a T2-hyperintense lesion with no contrast enhancement affecting his temporal lobe. Temporal craniotomy and microsurgical resection was scheduled. The procedure was uneventful and a grayish, gluey mass was sent for pathologic analysis. The tumor was formed by immature neuronal cells organized in nodules with a vacuolated matrix. A thorough immunohistochemical analysis showed positivity for: Protein Gene Product 9.5. ATRX. OLIG2. SOX10. p16. Nestin. Synaptophysin. The findings were consistent with multinodular and vacuolating neuronal tumor. The patient has been seizure-free after surgery and with no signs of tumor progression. Conclusion We present a thorough review addressing this uncommon tumor along with a description of the 17th reported case of MVNT, a tumor that was described for the first time in 2013. Further studies and case studies are necessary to establish a well-defined morphological and immunohistochemical profile along with knowledge about its natural history.",
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N2 - Background Multinodular and vacuolating neuronal tumor has been recently described and included in the World Health Organization Classification of Tumors of The Central Nervous System, even though its consideration as a true tumor is controversial. Patients with these lesions usually present with refractory seizures and inconclusive imaging findings that may be confused with other more common diagnoses such as dysembryoplastic neuroepithelial tumors or low-grade gliomas. Therefore, surgical resection is warranted to reach a pathologic diagnosis and seizure control. To the best of our knowledge, only 16 cases have been published in the English literature. Case description We present the case of a 52-year-old male who presented at our institution with a 2-year-history of absence of seizures. Brain MRI showed a T2-hyperintense lesion with no contrast enhancement affecting his temporal lobe. Temporal craniotomy and microsurgical resection was scheduled. The procedure was uneventful and a grayish, gluey mass was sent for pathologic analysis. The tumor was formed by immature neuronal cells organized in nodules with a vacuolated matrix. A thorough immunohistochemical analysis showed positivity for: Protein Gene Product 9.5. ATRX. OLIG2. SOX10. p16. Nestin. Synaptophysin. The findings were consistent with multinodular and vacuolating neuronal tumor. The patient has been seizure-free after surgery and with no signs of tumor progression. Conclusion We present a thorough review addressing this uncommon tumor along with a description of the 17th reported case of MVNT, a tumor that was described for the first time in 2013. Further studies and case studies are necessary to establish a well-defined morphological and immunohistochemical profile along with knowledge about its natural history.

AB - Background Multinodular and vacuolating neuronal tumor has been recently described and included in the World Health Organization Classification of Tumors of The Central Nervous System, even though its consideration as a true tumor is controversial. Patients with these lesions usually present with refractory seizures and inconclusive imaging findings that may be confused with other more common diagnoses such as dysembryoplastic neuroepithelial tumors or low-grade gliomas. Therefore, surgical resection is warranted to reach a pathologic diagnosis and seizure control. To the best of our knowledge, only 16 cases have been published in the English literature. Case description We present the case of a 52-year-old male who presented at our institution with a 2-year-history of absence of seizures. Brain MRI showed a T2-hyperintense lesion with no contrast enhancement affecting his temporal lobe. Temporal craniotomy and microsurgical resection was scheduled. The procedure was uneventful and a grayish, gluey mass was sent for pathologic analysis. The tumor was formed by immature neuronal cells organized in nodules with a vacuolated matrix. A thorough immunohistochemical analysis showed positivity for: Protein Gene Product 9.5. ATRX. OLIG2. SOX10. p16. Nestin. Synaptophysin. The findings were consistent with multinodular and vacuolating neuronal tumor. The patient has been seizure-free after surgery and with no signs of tumor progression. Conclusion We present a thorough review addressing this uncommon tumor along with a description of the 17th reported case of MVNT, a tumor that was described for the first time in 2013. Further studies and case studies are necessary to establish a well-defined morphological and immunohistochemical profile along with knowledge about its natural history.

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