dc.description.abstract | To better define outcome and prognostic factors in primary pineal tumors. Thirty-five consecutive patients from seven academic centers of the Rare Cancer Network diagnosed between 1988 and 2006 were included. Median age was 36 years. Surgical resection consisted of biopsy in 12 cases and resection in 21 (2 cases with unknown resection). All patients underwent radiotherapy and 12 patients received also chemotherapy. Histological subtypes were pineoblastoma (PNB) in 21 patients, pineocytoma (PC) in 8 patients and pineocytoma with intermediate differentiation in 6 patients. Six patients with PNB had evidence of spinal seeding. Fifteen patients relapsed (14 PNB and 1 PC) with PNB cases at higher risk (p = 0.031). Median survival time was not reached. Median disease-free survival was 82 months (CI 50 % 28-275). In univariate analysis, age younger than 36 years was an unfavorable prognostic factor (p = 0.003). Patients with metastases at diagnosis had poorer survival (p = 0.048). Late side effects related to radiotherapy were dementia, leukoencephalopathy or memory loss in seven cases, occipital ischemia in one, and grade 3 seizures in two cases. Side effects related to chemotherapy were grade 3-4 leucopenia in five cases, grade 4 thrombocytopenia in three cases, grade 2 anemia in two cases, grade 4 pancytopenia in one case, grade 4 vomiting in one case and renal failure in one case. Age and dissemination at diagnosis influenced survival in our series. The prevalence of chronic toxicity suggests that new adjuvant strategies are advisable. | en_US |
dc.department-temp | [Villa, Salvador] H Univ Germans Trias, Catalan Inst Oncol, Barcelona, Catalonia, Spain -- [Villa, Salvador -- Lucas, Anna] Catalan Inst Oncol, Lhospitalet De Llobregat, Catalonia, Spain -- [Miller, Robert C.] Mayo Clin, Rochester, MN USA -- [Krengli, Marco] Univ Hosp Maggiore Carita, Novara, Italy -- [Abusaris, Huda] Erasmus MC, Radiat Oncol, Rotterdam, Netherlands -- [Baumert, Brigitta G.] Maastricht Univ Med Ctr MUMC, GROW Sch Oncol & Dev Biol, Radiat Oncol MAASTRO, Maastricht, Netherlands -- [Servagi-Vernat, Stephanie] CHU Jean Minjoz, Besancon, France -- [Igdem, Sefik] Istanbul Bilim Univ, Istanbul, Turkey -- [Boluda, Susanna] HU Bellvitge, Pathol, Lhospitalet De Llobregat, Catalonia, Spain -- [Mirimanoff, Rene O.] CHU Vaudois, CH-1011 Lausanne, Switzerland | en_US |