在最新一期國際著名期刊《神經(jīng)腫瘤期刊》上，北京三博腦科醫(yī)院神經(jīng)外科主任石祥恩及其博士研究生高樹梓,，報(bào)告了全球首例自發(fā)惡性變的顱咽管瘤病例,。該發(fā)現(xiàn)改變了以往顱咽管瘤良性組織病理特征、無惡性變的觀點(diǎn),，為該疾病的治療和研究提供了新的依據(jù),。
據(jù)介紹，這是一名37歲的女性患者,，7年前被確診為顱咽管瘤,，并分別于2004年8月和2008年11月因視力障礙和頭痛發(fā)病2次行顱咽管瘤手術(shù)治療，此后未進(jìn)行任何形式的放療和化療,。直至2009年8月該患者因進(jìn)行性視力障礙就診于北京三博腦科醫(yī)院神經(jīng)外科,，在行復(fù)發(fā)顱咽管瘤手術(shù)并進(jìn)行腫瘤病理檢查研究時(shí)發(fā)現(xiàn)，腫瘤成釉細(xì)胞基底膜破壞,，細(xì)胞異型性明顯,，過度染色，核分裂活躍,，提示腫瘤癌變,。
據(jù)悉，傳統(tǒng)觀點(diǎn)認(rèn)為顱咽管瘤一種常見的先天性顱內(nèi)良性腫瘤,，約占顱內(nèi)腫瘤發(fā)病率的1.2%~4.6%,，人群中發(fā)病率約為0.5/百萬~2.5/百萬。由于既往認(rèn)為該疾病無惡變傾向,，因此手術(shù)全切除后,，便可達(dá)到治愈的效果。但由于腫瘤發(fā)生在垂體前葉,，向鞍上生長累及下丘腦,，手術(shù)全切除困難，術(shù)后復(fù)發(fā)率較高,。
石祥恩教授指出,，此次發(fā)現(xiàn)提示，對于顱咽管瘤患者應(yīng)提高其手術(shù)全切除率,，避免腫瘤復(fù)發(fā),。同時(shí)，應(yīng)重視此類患者的復(fù)查,、隨診,，并進(jìn)一步對該疾病的發(fā)病機(jī)制、惡變誘因以臨床治療方法進(jìn)行研究和更新,，盡快提高防治效果,。
據(jù)悉,，石祥恩教授從1995年開始從事手術(shù)切除顱咽管瘤及其下丘腦功能保護(hù)研究，手術(shù)切除顱咽管瘤550余例,，腫瘤全切除率95%以上,。2011年5月《美國神經(jīng)外科雜志》114卷第5期，總結(jié)全球7位報(bào)告手術(shù)切除顱咽管瘤100例以上學(xué)者中,，石祥恩教授被評為手術(shù)例數(shù)最多,，全切除率最高，效果最好的醫(yī)生,。此次顱咽管瘤惡變病例是其550余例患者中的一例,。(生物谷 Bioon.com)
生物谷推薦原文出處：
Journal of Neuro-Oncology doi： 10.1007/s11060-010-0407-2
Case ReportMalignant transformation of craniopharyngioma: case report and review of the literature
Shuzi Gao, Xiangen Shi, Yanxia Wang, Hai Qian and Chengyin Liu
Craniopharyngioma is considered to be a benign intracranial tumor. Malignant transformation of craniopharyngioma has rarelybeen described. In this article, we report a case of ameloblastic carcinoma arising from a previously benign craniopharyngiomain a 42-year-old woman. The patient was diagnosed with craniopharyngioma in August 2004 and underwent surgical resection ofa typical craniopharyngioma, the pathological result was craniopharyngioma, papillary and adamantinomatous types. During thesubsequent 5 years, this patient experienced two recurrences, for which surgical resections were performed without radiotherapy.The last two pathologic diagnoses were malignant craniopharyngiomas and there was more apparent sign of malignancy in thethird pathologic section. The exact pathogenesis and the biological behavior of malignant change in craniopharyngioma arenot yet clear. We review the relevant literature of malignant craniopharyngioma. Histopathologic, clinical and imaging featuresof malignant craniopharyngioma and the possible effect of radiotherapy on the carcinogenesis are discussed.
