中华神经外科杂志
中華神經外科雜誌
중화신경외과잡지
Chinese Journal of Neurosurgery
2009年
2期
146-148
,共3页
周涛%张远征%桂秋萍%姜金利%孟祥辉%朱儒远%魏少波
週濤%張遠徵%桂鞦萍%薑金利%孟祥輝%硃儒遠%魏少波
주도%장원정%계추평%강금리%맹상휘%주유원%위소파
神经外胚瘤,原始%病理状态,体征和症状%复发
神經外胚瘤,原始%病理狀態,體徵和癥狀%複髮
신경외배류,원시%병리상태,체정화증상%복발
Neuroectodermal tumors,primitive%Pathological conditions,signs and symptoms%Recurrence
目的 分析原始性神经外胚瘤(PNET)在全身各系统特别是在中枢神经系统的临床表现及其治疗.方法 结合有关文献,回顾解放军总医院近10年来通过手术治疗的89例PNET,对不同系统发生的PNET进行比较,并总结了同期90例髓母细胞瘤的病例作为对比.结果 89例PNET中,中枢神经系统21例占24%(大脑半球9例,椎管内8例,脑干4例);其他系统68例(76%).21例发生于中枢神经系统的PNET病人共接受手术27次,肿瘤全切18次,次全切9次.15例成功随访的病人有12例复发,占80%;转移4例,占27%;随访病人中已死亡10例.中枢神经系统的PNET平均发病年龄22.0岁,其他系统的PNET平均发病年龄29.2岁,髓母细胞瘤平均发病年龄13.9岁.结论 发生在全身各系统的PNET临床表现相似,病理形态和免疫标记物相同,复发率和转移率很高,手术全切结合化疗有一定疗效.中枢神经系统的PNET与其他系统的PNET虽为同一种疾病,但也有其自身特点.
目的 分析原始性神經外胚瘤(PNET)在全身各繫統特彆是在中樞神經繫統的臨床錶現及其治療.方法 結閤有關文獻,迴顧解放軍總醫院近10年來通過手術治療的89例PNET,對不同繫統髮生的PNET進行比較,併總結瞭同期90例髓母細胞瘤的病例作為對比.結果 89例PNET中,中樞神經繫統21例佔24%(大腦半毬9例,椎管內8例,腦榦4例);其他繫統68例(76%).21例髮生于中樞神經繫統的PNET病人共接受手術27次,腫瘤全切18次,次全切9次.15例成功隨訪的病人有12例複髮,佔80%;轉移4例,佔27%;隨訪病人中已死亡10例.中樞神經繫統的PNET平均髮病年齡22.0歲,其他繫統的PNET平均髮病年齡29.2歲,髓母細胞瘤平均髮病年齡13.9歲.結論 髮生在全身各繫統的PNET臨床錶現相似,病理形態和免疫標記物相同,複髮率和轉移率很高,手術全切結閤化療有一定療效.中樞神經繫統的PNET與其他繫統的PNET雖為同一種疾病,但也有其自身特點.
목적 분석원시성신경외배류(PNET)재전신각계통특별시재중추신경계통적림상표현급기치료.방법 결합유관문헌,회고해방군총의원근10년래통과수술치료적89례PNET,대불동계통발생적PNET진행비교,병총결료동기90례수모세포류적병례작위대비.결과 89례PNET중,중추신경계통21례점24%(대뇌반구9례,추관내8례,뇌간4례);기타계통68례(76%).21례발생우중추신경계통적PNET병인공접수수술27차,종류전절18차,차전절9차.15례성공수방적병인유12례복발,점80%;전이4례,점27%;수방병인중이사망10례.중추신경계통적PNET평균발병년령22.0세,기타계통적PNET평균발병년령29.2세,수모세포류평균발병년령13.9세.결론 발생재전신각계통적PNET림상표현상사,병리형태화면역표기물상동,복발솔화전이솔흔고,수술전절결합화료유일정료효.중추신경계통적PNET여기타계통적PNET수위동일충질병,단야유기자신특점.
Objective To elucidate the clinical features and treatment of Primitive neuroectodermal tumor (PNET) in every part of the body, especially in CNS. Methods Clinical records of 89 cases with PNET of every system treated at 301 Hospital between 1996 and 2006 were carefully assembled. A review of relevant literatures was also done. The duration of follow-up was from 4 to 25 months (mean 13 months). Results Among 89 patients of HPC, 21eases (24%) occurred in CNS (9 in cerebral hemisphere; 8 in spinal canal; 4 in brain stem). The rest 68 cases (76%) occurred in other systems. All the 21 cases PNET in CNS underwent tumorectomy. Among all the 25 operations, total tumor removal was achieved in 18 cases and subtotal removal in 7 cases. Local recurrence occurred 12 times (80%) in 15 patients with successful visit. 4 patients (27%) presented one or more distant metastasis, and 10 patients died. Conehlsious PNET in CNS and PNET in other systems displayed similar clinical characteristics and pathological morphous. It is a great challenge to neurosurgeons as well as other surgeons to cure the disease. PNET has an unfavorable prognosis because its high rate of recurrence and metastasis. PNET in CNS as well as in other parts of the body should be recognized, as one disease as a whole. At the same time, PNET in CNS has its distinct features.
