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Survival and Prognosis of Patients with Pilocytic Astrocytoma: a Single-Center Study

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Survival and Prognosis of Patients with Pilocytic Astrocytoma: a Single-Center Study Brain Tumor Res Treat 2019;7(2):92-97 / pISSN 2288-2405 / eISSN 2288-2413 ORIGINAL ARTICLE https://doi.org/10.14791/btrt.2019.7.e36 Survival and Prognosis of Patients with Pilocytic Astrocytoma: A Single-Center Study Jae Hui Park1, Nani Jung1, Seok Jin Kang1, Heung Sik Kim1, El Kim2, Hee Jung Lee3, Hye Ra Jung4, Misun Choe4, Ye Jee Shim1 Departments of 1Pediatrics, 2Neurosurgery, 3Radiology, 4Pathology, Keimyung University School of Medicine, Keimyung University Dongsan Medical Center, Daegu, Korea Background Pilocytic astrocytoma (PA) is a brain tumor that is relatively more common in children and young adults. Methods We retrospectively reviewed the medical records of patients with PA treated at a sin- gle center between 1988 and 2018. Results We included 31 subjects with PA. The median age at diagnosis was 13.4 years, and the median follow-up duration was 9.9 years. The total PA group had a 10-year disease-specific survival (DSS) rate of 92.6% [95% confidence interval (CI), 82.6-100] and 10-year progression-free survival (PFS) rate of 52.8% (95% CI, 32.0-73.6). In patients aged <20 years, tumors were more likely to be lo- cated in sites in which gross total tumor resection (GTR) was impossible. No statistically significant dif- ference in 10-year DSS was found between the GTR (100%) and non-GTR (89.7%; 95% CI, 76.2-100; p=0.374) groups. However, a statistically significant difference in 10-year PFS was found between the Received March 18, 2019 GTR (100%) and non-GTR groups (30.7%; 95% CI, 8.6-52.8; p=0.012). In the non-GTR group, no Revised June 24, 2019 statistically significant difference in 10-year DSS was found between the patients who received immedi- Accepted July 12, 2019 ate additional chemotherapy and/or radiotherapy (Add-Tx group, 92.9%; 95% CI, 79.4-100) and the Correspondence non-Add-Tx group (83.3%; 95% CI, 53.5-100; p=0.577). No statistically significant difference in 10- Ye Jee Shim year PFS was found between the Add-Tx group (28.9%; 95% CI, 1.7-56.1) and non-Add-Tx group Department of Pediatrics, (33.3%; 95% CI, 0-70.9; p=0.706). Keimyung University School of Medicine, The PFS of the patients with PA in our study depended only on the degree of surgical 1095 Dalgubeol-daero, Dalseo-gu, Conclusion Daegu 42601, Korea excision associated with tumor location. This study is limited by its small number of patients and retro- Tel: +82-53-258-7824 spective nature. A multicenter and prospective study is necessary to confirm these findings. Fax: +82-53-258-4875 E-mail: [email protected] Key Words Glioma; Survivors; Child; Adolescent; Prognosis. INTRODUCTION ported. However, no data are available on survival and prog- nosis in patients with PA in the Yeungnam area. Thus, we Pilocytic astrocytoma (PA) is brain tumor that is relatively retrospectively reviewed the long-term survival and prognosis more common in children and young adults. PA is a World of patients with PA in a single center in the Yeungnam region. Health Organization grade I neoplasm with an expected be- nign course and a 10-year survival rate exceeding 95% [1,2]. In MATERIALS AND METHODS Korea, single-center data about the prognosis of PA in a met- ropolitan area [3] and in the Honam area [4] have been re- Subjects and ethics statement Patients who were diagnosed with PA and followed up at This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (https://creativecommons.org/licenses/by-nc/4.0) Keimyung University Dongsan Medical Center from January which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. 1988 to December 2018 were enrolled. We pathologically con- Copyright © 2019 The Korean Brain Tumor Society, The Korean Society for Neuro- firmed primary brain tumors through biopsy; thus, optic or Oncology, and The Korean Society for Pediatric Neuro-Oncology brainstem gliomas that were not available for biopsy were ex- 92 JH Park et al. cluded. This study was approved by the Institutional Review survival (DSS) is defined as the proportion of people who Board of Keimyung University Dongsan Medical Center (no. died of PA progression; patients who died of medical condi- 2017-11-075). Data from the patients’ medical records, includ- tions other than PA were not included in DSS. ing those pertaining to age at diagnosis, sex, initial symptoms or signs, tumor site, treatment modality, survival rate, and Statistical analyses complications, were collected and retrospectively reviewed. To compare the ratio of the variables between the 2 groups, the χ2 test was used. The 10-year probabilities of overall surviv- Definitions al (OS), DSS, and progression-free survival (PFS) were esti- Gross total tumor resection (GTR) was defined when no mated with the Kaplan-Meier method using the log-rank test. obvious visible tumor was found on brain imaging performed p values <0.05 indicated statistical significance. All statistical soon after the operation. If a portion of the tumor was still analyses were performed using SPSS v23.0 software (IBM found on a brain imaging scan after surgery, it was defined as Corp., Armonk, NY, USA). The 95% confidence intervals (CIs) a subtotal tumor resection (STR) when the residual tumor were calculated using the mean values and standard errors. was <50% and as partial tumor resection (PTR) when the re- sidual tumor was ≥50% according to the neurosurgeon’s RESULTS judgment [4]. After surgical resection and/or postoperative radiotherapy or chemotherapy, the development of new le- Subjects’ characteristics and clinical manifestations sions or the increase of tumor size from that of the original During the study period, 1,017 patients with pathologically residual tumor was defined as progression. Disease-specific confirmed primary tumors that originated from the central A Pilocytic astrocytoma Pilomyxoid astrocytoma Oligodendroglioma Glioblastoma Anaplastic astrocytoma Diffuse astrocytoma Ependymoma Ganglioglioma Oligoastrocytoma Oligosarcoma Non-glial tumors Age <20 years (n=96) Age ≥20 years (n=921) <20 yrs: 64.5% (20/31) B 12 11 10 9 8 6 5 4 Number of patients of Number 3 2 2 1 0 0 Age<10 10≤age<20 20≤age<30 30≤age<40 40≤age<50 50≤age<60 60≤age<70 Age group (years) Fig. 1. The ratio of glial tumors between age groups in a single medical center between1988 and 2018. A: A total of 1,017 patients had pathologically confirmed primary brain tumors; of them, 314 had glial tumors. Pilocytic astrocytoma occurred more frequently in patients aged <20 years (20.8%) than in those aged ≥20 years (1.2%; p<0.001). B: The ratio of pilocytic astrocytoma among the patients aged <20 years was 64.5%. 93 Pilocytic Astrocytoma: Survival and Prognosis nervous system were treated at our center. Among them, 314 Table 1. Baseline characteristics of patients with pilocytic astrocy- toma treated at a single center had glial brain tumors; 155, glioblastoma; 46, oligodendrogli- oma; 31, PA; 2, pilomyxoid astrocytoma; 24, ependymoma; Characteristic Value 15, ganglioglioma; 13, diffuse astrocytoma; 12, anaplastic as- Age at diagnosis (years), median (range) 13.4 (0.6–67.0) trocytoma; 9, oligoastrocytoma; and 7, gliosarcoma. The ratio Sex (male to female ratio) 15:16 Underlying anomaly (NF1 to non-NF1 ratio) 1:30 of PA to the total number of primary brain tumors was higher Follow-up duration (years), median (range) 9.9 (0.1–30.2) in the patients aged <20 years [20.8% (20/96)] than in those Symptoms or signs at diagnosis, n (%) aged ≥20 years [1.2% (11/921); p<0.001]. The ratios of the re- Headache 7 (22.6) spective disease groups by patient age are shown in Fig. 1A. Seizure 6 (19.4) Thirty-one subjects had PA (male-to-female ratio, 15:16). Vomiting 6 (19.4) Among them, one had neurofibromatosis 1 confirmed by ge- Mental change 6 (19.4) netic testing. The median age at diagnosis was 13.4 years Visual disturbance 6 (19.4) (range, 0.6–67.0 years). The median follow-up duration was Gait disturbance 6 (19.4) 9.9 years (range, 0.1–30.2 years). The PA distribution by age is Hemiparesis 5 (16.1) shown in Fig. 1B. The patients with PA had symptoms or signs Sensory change 3 (9.7) that included headache (7), seizure (6), vomiting (6), mental Facial palsy 3 (9.7) change (6), visual disturbance (6), gait disturbance (6) or Male precocious puberty 2 (6.5) hemiparesis (5). The tumor sites were the cerebrum (11), cere- Dysarthria 2 (6.5) bellum (8), optic chiasm and hypothalamus (7), brain stem (2), Nystagmus 1 (3.2) basal ganglia (2), and ventricle (1). The baseline characteristics Dysmetria 1 (3.2) of the subjects with PA are summarized in Table 1. Site, n (%) Supratentorial tumor Treatment modalities Cerebrum 11 (35.5) Among the 31 patients with PA, 10 underwent GTR; 14, Optic chiasm and hypothalamus 7 (22.6) STR; 3, PTR; and 4, biopsy. In the non-GTR group (n=21), Basal ganglia 2 (6.5) additional immediate chemotherapy and/or radiotherapy Ventricle 1 (3.1) were administered to 15 patients (Add-Tx group): chemo- Infratentorial tumor therapy was administered to 10 patients and radiotherapy Cerebellum 8 (25.8) was administered to 7 patients (2 patients received both). In Brain stem 2 (6.5) the patients aged <20 years, tumors were more likely to be NF1, neurofibromatosis 1 located in sites where GTR could not be performed, includ- ing the optic chiasm and hypothalamus (7), brain stem (2), (100%), STR (92.9%; 95% CI, 79.4–100), PTR (100%), and bi- and basal ganglia (2).
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