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儿童松果体区肿瘤。30年经验总结。

Pineal region tumours in childhood. A 30-year experience.

作者信息

Drummond K J, Rosenfeld J V

机构信息

Department of Surgery, The Royal Melbourne Hospital, Parkville, Victoria, Australia.

出版信息

Childs Nerv Syst. 1999 Mar;15(2-3):119-26; discussion 127. doi: 10.1007/s003810050347.

DOI:10.1007/s003810050347
PMID:10230668
Abstract

Patients aged under 16 years presenting to the Royal Children's Hospital between 1967 and 1997 with pineal region tumours were retrospectively reviewed. Thirty-seven patients were identified, with 13 germinomas, 7 nongerminomatous germ cell tumours, 6 pineoblastomas, 2 pineocytomas, and 3 astrocytomas, while in 6 patients no histopathological diagnosis was obtained. The most common presentation was with symptoms of raised intracranial pressure due to hydrocephalus. Thirty-two of the 37 patients required a shunt. Thirteen had a biopsy as a separate procedure, 3 of which were stereotactic. Tumour excision was performed in 21 patients and was complete in 4 and subtotal in 17. There were 2 perioperative deaths and 6 patients who were neurologically worse after surgery. Twenty-six patients had radiotherapy and 16 chemotherapy, with significant complications of radiotherapy in half of the patients who received it. The 5-year survival of patients with benign tumours was 75%, 5-year survival with germinomas 62% and with other malignant tumours 14%. This series demonstrates significant improvements in management of pineal region tumours in the last 30 years and highlights some of the current controversies. A collaborative research approach is necessary to determine optimal management of the varied tumour types occurring in the pineal region in childhood.

摘要

对1967年至1997年间在皇家儿童医院就诊的16岁以下松果体区肿瘤患者进行回顾性研究。共确定了37例患者,其中13例为生殖细胞瘤,7例为非生殖细胞性生殖细胞瘤,6例为松果体母细胞瘤,2例为松果体细胞瘤,3例为星形细胞瘤,另有6例未获得组织病理学诊断。最常见的表现是因脑积水导致颅内压升高的症状。37例患者中有32例需要进行分流术。13例患者单独进行了活检,其中3例为立体定向活检。21例患者进行了肿瘤切除术,4例完全切除,17例次全切除。围手术期死亡2例,6例患者术后神经功能恶化。26例患者接受了放疗,16例接受了化疗,接受放疗的患者中有一半出现了严重并发症。良性肿瘤患者的5年生存率为75%,生殖细胞瘤患者为62%,其他恶性肿瘤患者为14%。该系列研究表明,过去30年中松果体区肿瘤的治疗有了显著改善,并突出了一些当前的争议问题。需要采取合作研究方法来确定儿童松果体区各种肿瘤类型的最佳治疗方案。

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