• Vitor Nagai Yamaki, Solla Davi Jorge Fontoura DJF Department of Neurosurgery, School of Medicine, Universidade de Sao Paulo, Sao Paulo, Brazil., Renan Ribeiro Ribeiro, da Silva Saul Almeida SA Department of Neurosurgery, School of Medicine, Universidade de Sao Paulo, Sao Paulo, Brazil., Manoel Jacobsen Teixeira, and Eberval Gadelha Figueiredo.
• Department of Neurosurgery, School of Medicine, Universidade de Sao Paulo, Sao Paulo, Brazil.
• Neurosurgery. 2019 Sep 1; 85 (3): E420-E429.

BackgroundClinical outcomes and biological behavior of papillary tumors of the pineal region (PTPR) are still under investigation. The best therapeutic strategy has not been defined.ObjectiveTo perform a comprehensive patient-level analysis of all PTPR cases and identify their clinical features, treatment options, and prognostic factors.MethodsA search of the medical databases for case series and reports on PTPRs from January 2003 to June 2017 was performed. Data addressing PTPR's clinical presentation, imaging, treatment, and histological features were. Variables associated with the primary outcome of 36-mo survival were identified through Cox regression models.ResultsThe initial search yielded 1164 studies, of which 71 were included (60 case reports and 11 case series), containing 177 patients (mean age 33.0 ± 15.3 yr and 53.2% male). Intracranial hypertension and hydrocephalus prevailed as the clinical picture. Surgery was performed on 82.0% and gross total resection (GTR) was achieved on 71.4%. A total of 56.8% recurred after a median 29 mo (quartiles 10.5-45.5). The 36-mo survival rate was 83.5% (95% confidence interval [CI] 76.2-89.2%). Good functional outcomes (Glasgow Outcome Scale 4/5) were observed in 60.0%. The variables of interest were inconsistently reported and the multivariable analysis final sample was 133 patients. After adjustment for age, tumor size (each additional centimeter, hazard ratio [HR] 1.99, 95% CI 1.12-3.53, P = .019) and surgical treatment (HR 0.16, 95% CI 0.05-0.45, P = .001) were associated with 36-mo survival.ConclusionTumor size and surgery are associated with improvement in 36-mo survival. We did not observe any significant benefits from GTR or adjuvant treatments.Copyright © 2019 by the Congress of Neurological Surgeons.

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