- 期刊
Medulloblastoma: The Treatment Results and Literature Review
神經管胚細胞瘤：治療結果及文獻回顧
摘要
自1982年元月到1991年12月，共有24例（男：女=13:11）年齡介於10個月到13歲（中值年齡是6歲）經組織切片證實之神經管胚細胞瘤病患於馬偕醫院接受治療；全部病患均接受手術切除腫瘤及輔助性腦脊髓軸放射治療和後顱窩加強治療。頭痛，嘔吐及步態不穩為本文病患最主要的臨床表現；疾病之程度則依照Chang氏分級系統加以區分；其全體5年生存率為58%；此外女性的5年生存率比男性佳（73%及46%, P=0.03），早期患者（T1及T2）比晚期（T3及T4）有較好的5年生存率（71%及40%, P =0.016） ，後顱窩之劑量至50 Gy以上的有較高的5年生存率（73%及33%, P=0.05）；而手術切除的完整性並不影響5年生存率(P=0.13) ，且年紀大於5歲者雖然5年生存率較高但P值為0.066（71%及40%）。本報告之復發率達50%(12/24)，其中只有3個病例於追蹤中尚存活。 由文獻之回顧及本文中分析可知由於後顱窩超過50 Gy的照射及診斷技術改進，全體生存率已有很大之進步，因而晚期的後遺症已成為重要主題；然而，在晚期或復發病例其預後仍然不佳。故訂定適當要件以降低長期副作用及進一步改善治療結果是必須的。
並列摘要
Twenty-four children (M:F=13:11) whose ages ranged from 10 months to 13 years (median age 6 years) with histologically confirmed medulloblastomas were treated at Mackay Memorial Hospital from January 1982 to December 1991. All 24 patients were to undergo extensive resection and then to receive a primary course of adjuvant craniospinal axis irradiation with a local posterior fossa boost. The anatomical extent of the disease was classified according to the Chang staging system. Headache, vomiting and an unsteady gait were the major presentations in this series. The overall 5-year survival rate was 58% respectively. In addition, the overall 5-year survival rate was statistically higher in females than in males (73% vs 46%, P=0.03), in early-stage tumors (T1 & T2) than in late-stage tumors (T3 & T4) (71% vs 40%, P=0.066), and in patients who received a dose of 50 Gy or more to the posterior fossa than in those who received a lower dose (73% vs 33%, P=0.05). The extent of surgery had no impact on the survival rate (P=0.13). Patients older than 5-years of age had a higher 5-year survival rate but it was not statistically significant (71% vs 40%, P=0.066). The overall relapse rate was 50% in our series with only 3 eases survived in the follow-up. From the literature review and the results of this series, we can conclude that by more than 50 Gy posterior fossa irradiation, the overall survival rate has much improved, so the late sequellae have become an important subject. However, there is still poor outcome in advanced disease and recurrent disease. The optimal selection criteria are urgently required to reduce long-term complications and to increase survival in poor outcome groups.
延伸閱讀
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