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Case Report
DOI: 10.1016/j.neucir.2018.03.003
Papillary tumor of the pineal region: Case report and review of the literature
Tumor papilar de la región pineal, caso clínico y revisión de la literatura
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María de los Ángeles Cañizares Méndeza,
Corresponding author
angeles.ca.me@gmail.com

Corresponding author.
, Manuel Amosa Delgadoa, Juan Antonio Álvarez Salgadoa, Jorge Javier Villaseñor Ledezmaa, Elena Capilla Cabezuelob, Francisco Díaz Crespoc
a Department of Neurosurgery, Complejo Hospitalario de Toledo (CHT), Spain
b Department of Radiology, Complejo Hospitalario de Toledo (CHT), Spain
c Department of Pathology, Complejo Hospitalario de Toledo (CHT), Spain
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Received 31 October 2017, Accepted 15 March 2018
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Table 1. Table including the 23 patients reported in the literature that were treated by GTR, without complementary treatment and with some follow up.
Abstract

Papillary tumor of the pineal region is a rare neuroepithelial tumor characterized by papillary architecture and epithelial cytology, immunopositivity for cytokeratin and ependymal differentiation. It is considered grade II–III by the World Health Organization and was first described by Jouvet in 2003. We present a 34-year-old male with headaches, blurred vision and normal examination. Radiological study showed a nodulocystic lesion in the pineal region compatible with pineocytoma. Surgery was performed using an infratentorial supracerebellar approach, finding a cystic tumor in the quadrigeminal cistern which was completely resected. Histopathology reported a papillary tumor of the pineal region. The patient made good progress without adjuvant therapy, and after 57 months of follow-up he remained asymptomatic and free of recurrence. A review of the literature was performed to collect all the cases published with gross total resection and no complementary treatment. In conclusion, there is still much to be learned about the pathogenesis, prognosis and management of this tumor.

Keywords:
Hydrocephalus
Pineal gland
Pineal gland tumor
Immunohistochemistry
Abbreviations:
PTPR
WHO
CT
MRI
CBV
CSF
CK
GFAP
EMA
GCT
AFP
β-hCG
GTR
Resumen

El tumor papilar de la región pineal es un infrecuente tumor neuroepitelial con arquitectura y citología epitelial, citoqueratina positiva y signos de diferenciación ependimaria. Descrito por Jouvet en 2003 es considerado grado II-III de la Organización Mundial de la Salud. Presentamos el caso de un varón de 34 años con cefalea, visión borrosa y exploración normal. El estudio radiológico muestra una lesión noduloquística en región pineal compatible con pineocitoma. Es intervenido mediante abordaje supracerebeloso infratentorial apreciándose lesión de aspecto quístico en cisterna cuadrigémina que es resecada por completo. El resultado histopatológico es tumor papilar de la región pineal. El paciente presenta buena evolución sin tratamiento adyuvante y tras 57 meses de seguimiento permanece asintomático y sin recidiva. Realizamos una revisión sistemática sobre la evolución de los casos publicados tratados mediante resección completa y sin tratamiento complementario, concluyendo que continuamos necesitando aprender sobre la patogénesis, pronóstico y manejo de este tumor.

Palabras clave:
Hidrocefalia
Glándula pineal
Tumor de la glándula pineal
Inmunohistoquímica

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