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松果体肿瘤:10年48例的经验

Pineal tumors: experience with 48 cases over 10 years.

作者信息

Cho B K, Wang K C, Nam D H, Kim D G, Jung H W, Kim H J, Han D H, Choi K S

机构信息

Department of Neurosurgery, Seoul National University Children's Hospital, Korea.

出版信息

Childs Nerv Syst. 1998 Jan-Feb;14(1-2):53-8. doi: 10.1007/s003810050175.

Abstract

The authors retrospectively reviewed 48 patients treated at Seoul National University Hospital (SNUH) between 1986 and 1995. There were 35 children and 13 adults, accounting for 10.1% of 345 pediatric and 0.68% of 1914 adult brain tumors in SNUH during the same period. The 48 cases consisted of 33 cases of germ cell tumor (69%, GCT); 6 of pineoblastoma (PB, 12.5%); 3 of pineocytoma (PC, 6.3%); 3 of anaplastic astrocytoma (6.3%); 1 of astrocytoma; 1 of glioblastoma; and 1 of ependymoma. The median age was 13 years (range 1-59) and the male-to-female ratio was 3.36:1. The most frequent presenting symptom was due to increased intracranial pressure (90%), followed by Parinaud syndrome or diplopia (50%). Patients with a benign tumor, such as teratoma (TE), astrocytoma, or ependymoma, underwent surgery by the occipital transtentorial approach (OTT) for attempted radical resection without adjuvant therapy, while patients with immature teratoma (imTE), PC, and anaplastic astrocytoma underwent regional radiotherapy (RT) after debulking via OTT. Seven patients with nongerminomatous malignant GCT (NG-MGCT) and 3 with germinoma (GE) underwent craniospinal radiation only, 6 with GE, a NG-MGCT, and 2 with GE+TE received craniospinal radiotherapy (CSRT) after debulking via OTT. Three patients with GE, 4 with NG-MGCT, and 3 with PB underwent radiochemotherapy after debulking via OTT. Forty-four patients were followed up after treatment. The median follow-up period was 36 months. All patients with GE were alive after RT at 36 months (median) of follow-up (range 7-70 months). All with GE+TE and TE were alive. Three patients with PC or astrocytoma were also alive with stable or no evidence of disease. In 1 of the 3 cases of imTE there was a recurrence. However, 4 patients with NG-MGCT died, all of whom had undergone CSRT only; 2 PB patients were alive (12, 19 months), 1 in a moribund status (36 months), and 2 were dead (6, 60 months). The overall mean survival time with pineal tumors was 66 months and the 3-year survival rate was 84% with minimal posttreatment complications. It is concluded that pineal region tumors have male and childhood predominances, and the most common tumor is GCT. The majority of pineal region tumors are malignant. Pineal region tumors can be approached safely and effectively and the surgical complications are mostly transient. Their prognosis is dependent on the pathologies and treatment modalities.

摘要

作者回顾性分析了1986年至1995年间在首尔国立大学医院(SNUH)接受治疗的48例患者。其中有35名儿童和13名成人,分别占同期SNUH 345例儿童脑肿瘤的10.1%和1914例成人大脑肿瘤的0.68%。这48例病例包括33例生殖细胞瘤(69%,GCT);6例松果体母细胞瘤(PB,12.5%);3例松果体细胞瘤(PC,6.3%);3例间变性星形细胞瘤(6.3%);1例星形细胞瘤;1例胶质母细胞瘤;以及1例室管膜瘤。中位年龄为13岁(范围1 - 59岁),男女比例为3.36:1。最常见的首发症状是颅内压升高(90%),其次是帕里诺德综合征或复视(50%)。患有良性肿瘤如畸胎瘤(TE)、星形细胞瘤或室管膜瘤的患者,采用枕下经小脑幕入路(OTT)进行手术,试图根治性切除,无需辅助治疗;而患有未成熟畸胎瘤(imTE)、PC和间变性星形细胞瘤的患者在通过OTT进行肿瘤大部分切除后接受局部放疗(RT)。7例非生殖细胞性恶性GCT(NG - MGCT)患者和3例生殖细胞瘤(GE)患者仅接受全脑全脊髓放疗;6例GE、1例NG - MGCT以及2例GE + TE患者在通过OTT进行肿瘤大部分切除后接受全脑全脊髓放疗(CSRT)。3例GE、4例NG - MGCT和3例PB患者在通过OTT进行肿瘤大部分切除后接受放化疗。44例患者在治疗后进行了随访。中位随访期为36个月。所有GE患者在随访36个月(中位)时(范围7 - 70个月)放疗后存活。所有GE + TE和TE患者均存活。3例PC或星形细胞瘤患者也存活,病情稳定或无疾病证据。3例imTE病例中有1例复发。然而,4例NG - MGCT患者死亡,他们均仅接受了CSRT;2例PB患者存活(12、19个月),1例处于濒死状态(36个月),2例死亡(6、60个月)。松果体区肿瘤的总体平均生存时间为66个月,3年生存率为84%,治疗后并发症极少。结论是松果体区肿瘤以男性和儿童为主,最常见的肿瘤是GCT。大多数松果体区肿瘤是恶性的。松果体区肿瘤可以安全有效地进行手术,手术并发症大多是短暂的。其预后取决于病理类型和治疗方式。

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