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CC BY-NC-ND 4.0 · Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery 2018; 37(03): 252-257
DOI: 10.1055/s-0038-1670695
Case Report | Relato de Caso
Thieme Revinter Publicações Ltda Rio de Janeiro, Brazil

Papillary Tumor of the Pineal Region: Case Report and Literature Review

Tumor papilífero da região pineal: relato de caso e revisão da literatura
Vitor Nagai Yamaki
1   Department of Neurosurgery, School of Medicine, Universidade de São Paulo, SP, Brazil
,
Felipe Romero Vera
2   Department of Neurosurgery, Hospital Barros Luco Trudeau, Santiago, Chile
,
Renan Ribeiro Ribeiro
3   Department of Pathology, School of Medicine, Universidade de São Paulo, SP, Brazil
,
Raphael Salles Scortegagna Medeiros
3   Department of Pathology, School of Medicine, Universidade de São Paulo, SP, Brazil
,
Manoel Jacobsen Teixeira
1   Department of Neurosurgery, School of Medicine, Universidade de São Paulo, SP, Brazil
,
Eberval Gadelha Figueiredo
1   Department of Neurosurgery, School of Medicine, Universidade de São Paulo, SP, Brazil
› Institutsangaben Funding Information The authors have none to declare.
Weitere Informationen

Publikationsverlauf

31. Januar 2018

27. Juni 2018

Publikationsdatum:
18. Oktober 2018 (online)

   Lizenzen und Reprints

Abstract

Papillary tumor of the pineal region (PTPR) is a neuroectodermal tumor thought to originate from cells of the subcommissural organ. Its oncologic properties are still under investigation, as well as the most suitable therapeutic measures for this type of neoplasm. We report the case of a 36-year-old woman with a 1-year history of headache and intermittent diplopia. The magnetic resonance imaging (MRI) scan showed a heterogeneously enhancing mass in the pineal region that caused an acute hydrocephalus, and an emergency shunt derivation was necessary. One week later, the patient was submitted to subtotal tumor resection, and remained asymptomatic in the post-operative period. In the follow-up, the patient remained asymptomatic; in the imaging control 3.5 years after the surgical resection, local recurrence was identified, and the patient was submitted to a local radiation protocol. Our literature review showed an early clinical onset due to intracranial hypertension signs. Definitive clinical onset might be reached only through a histopathological examination. Gross total resection followed by radiotherapy is the current standard of care. Local recurrence is often observed, with rare dissemination to the cerebral spinal fluid. The natural history of the PTPR remains unknown, as well as the best treatment strategy. Large case series with longer follow-ups are necessary for further conclusions.

Resumo

O tumor papilífero da região pineal (TPRP) tem origem neuroectodérmica nas células do órgão subcommissural. Suas propriedades oncológicas ainda estão sob investigação, assim como as medidas terapêuticas mais adequadas para este tipo de neoplasma. Relatamos o caso de uma paciente do sexo feminino, de 36 anos, com um histórico de um ano de cefaleia e diplopia intermitente. O exame de imagem de ressonância magnética (RM) evidenciou massa em topografia da glândula pineal com captação heterogênea ao contraste. Tal lesão tumoral provocou hidrocefalia aguda obstrutiva, com necessidade de derivação de emergência do ventrículo peritoneal. Após 1 semana, a paciente foi submetida a ressecção subtotal do tumor e permaneceu assintomática no período pós-operatório. Durante o acompanhamento, a paciente manteve-se assintomática, com recorrência local da lesão após 3,5 anos, e foi submetida a um protocolo local de radiação. Após revisão da literatura, grande parte dos tumores papilíferos da região pineal tiveram diagnóstico precoce, especialmente devido a sinais de hipertensão intracraniana. Os achados em exames de imagem não são específicos, e o diagnóstico definitivo só pode ser obtido por meio de avaliação histopatológica. Ressecção cirúrgica seguida de radioterapia tem se mostrado a melhor alternativa terapêutica. A recorrência local sido é observada de modo frequente em tumores tratados, mas raramente com disseminação a distância para o líquido cefalorraquiano. A história natural dos tumores papilíferos da região pineal ainda está sob investigação, assim como a melhor estratégia de tratamento. Séries de casos com amostras maiores e acompanhamento mais prolongado são necessárias para que se obtenham conclusões mais consistentes.

Keywords

pineal gland - brain tumor - surgical procedures - local recurrence

Palavras-chave

glândula pineal - tumor cerebral - tratamento cirúrgico - recorrência local

Ethical Statement

We confirm that this study is original, never before published, and it is not under consideration by any other journal. This research is in compliance with ethical standards, and informed consent was obtained from all individual participants included in the study.


 

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