성인 다형성 황색 성상세포종의 임상결과

Abstract

Introduction: PXA is known for clinically indolent tumor, however clinical behavior of PXA is not uniform. The predicting factors of aggressive behaviors of PXA have not been fully elucidated. The purpose of this report is to demonstrate the experiences and clinical outcomes of PXA in our institution, to find prognostic factors of PXA. Method: Our institutional database was searched for patients aged 18 years or older who underwent surgery and were diagnosed with PXA between 2002 and 2016. Total of 25 patients were identified and analyzed. Result: There were 8 (32%) male and 17 (68%) female patients. Mean age was 29.9. Most common presenting symptoms was seizure. Most common location of PXA was temporal lobe (11, 44%). Mean tumor size was 33.6 mm (range: 10 to 70 mm). 21 (84%) patients were diagnosed as PXA, WHO grade II and 4 (16%) patients were diagnosed anaplastic PXA, WHO grade III. The perilesional edema was seen on 13 patients (52%). Twenty-one (84%) patients underwent GTR, 4 (16%) of patients underwent STR. Seven (28%) patients received adjuvant radiation therapy. No patient received adjuvant chemotherapy. Recurrence occurred in 11 (44%) patients. High grade transformation was observed in 4 patients (36.4%). Six (24%) patients were died during follow up period. The OS rate of PXA in 1,2,3,5,7 and 10 years were 100%, 89.5%, 89.5%, 89.5%, 61.4% and 40.9% and OS rate of anaplastic PXA in 1,2,3,5,7,10 years were 100%, 100%,100%, 0%, 0% and 0%. The PFS rate of PXA in 6 months, 1,2,3,5 and 7 years were 90.5%, 81%, 70.5%, 65.1%, 65.1% and 52% and PFS rate of anaplastic PXA in 6 months, 1,2,3,5 and 7 years were 100%, 75%, 50%, 50%, 0% and 0%. The differences of OS and PFS between PXA and anaplastic PXA were not statistically significant. (p value= 0.379 and 0.213, respectively. We did comparison the advanced MR imaging (DWI, PWI) and PET finding between PXA and anaplastic PXA. This result failed to show statistical significance. We analyzed the impact of advanced MR findings and PET findings to recurrence rate of PXA grade II. In recurrence group, There were no differences of DWI, PWI and PET findings between recurrence and non-recurrence group. Tumor size larger than 40mm, solid with mixed cystic and hemorrhagic tumor, presence of perilesional edema were poor prognostic factors for PFS (HR=4.394, 11.846, 15.239, p value=0.036, 0.013, 0.01 respectively) were poor prognostic factors in univariate analysis. In multivariate analysis, only perilesional edema was significant poor prognostic factor. (HR=20.523, p value=0.009) We did comparison characteristics between post-treatment silent PXA grade II group and recurrent PXA (grade II) group. In recurrent PXA grade II group, the rate of presence of peritumoral edema was 87.5%, while in silent PXA grade II group, the rate of presence of peritumoral edema was 23.1%. This difference showed statistically significance. (p value= 0.008). A PXA grade II grading system was constructed using the 3 variable, size, peritumoral edema and tumor type. Score 1 to 2 were classified low risk and score 3 to 4 were high risk. Tumor progression and recurrence was predicted according to PXA grade II group, 5-year progression-free survival was 80.2% in low risk group and 20% in high risk group. (p value= 0.025) Conclusion:. To our limited knowledge, tumor size, solid plus cyst and hemorrhage tumor type and peritumoral edema of PXA is associated with poorer progression free survival in univariate analysis. DWI, PWI and PET findings of PXA can’t expect clinical behavior and grade of the PXA. PXA show high recurrence rate, thus close follow up is needed. In the future, multicenter larger size prospective study should be needed