一例因脊髓液中胎盘碱性磷酸酶阴性而分化出双灶性病灶的中间分化型松果体实质肿瘤。

IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY
Child's Nervous System Pub Date : 2024-09-01 Epub Date: 2024-05-07 DOI:10.1007/s00381-024-06429-1
Koki Ito, Yasuo Aihara, Kentaro Chiba, Yuichi Oda, Takakazu Kawamata
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引用次数: 0

摘要

脑脊液中的胎盘碱性磷酸酶(PLAP)有助于诊断颅内生殖细胞瘤。有报道称,松果体和垂体区域的双灶病变也是颅内胚芽细胞瘤的特征性发现。我们报告了一例罕见的病例,一名 15 岁男孩患有中间分化型松果体实质肿瘤(PPTID),其双灶病变对 PLAP 阴性。脑部磁共振成像显示,松果体和小脑上部有双灶性肿块病变,并伴有非交流性脑积水。根据成像结果,我们最初怀疑是生殖细胞瘤,但脊髓液中包括 PLAP 在内的所有肿瘤标记物均为阴性。基于这些结果,我们认为生殖细胞瘤的可能性较小,于是进行了内镜下第三脑室造口术和内镜下肿瘤活检以确诊。松果体和鞍上标本的组织病理学诊断均为 PPTID,相当于世界卫生组织的 3 级。为切除肿瘤进行了开颅手术,结果是肿瘤全部切除。众所周知,PLAP 对生殖细胞瘤具有很高的灵敏度和极高的阴性预测值。虽然双灶病变高度提示生殖细胞瘤,但也有例外,本病例就是如此。本病例表明,PLAP 测量有助于进行鉴别,从而制定适当的治疗策略。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

A case of a pineal parenchymal tumor of intermediate differentiation with bifocal lesions differentiated by negative placental alkaline phosphatase in the spinal fluid.

A case of a pineal parenchymal tumor of intermediate differentiation with bifocal lesions differentiated by negative placental alkaline phosphatase in the spinal fluid.

Placental alkaline phosphatase (PLAP) in the spinal fluid is helpful for the diagnosis of intracranial germinomas. Bifocal lesions involving the pineal and pituitary regions have also been reported as characteristic findings of intracranial germinomas. We present a rare case of a 15-year-old boy with a pineal parenchymal tumor of intermediate differentiation (PPTID) with bifocal lesions negative for PLAP. Magnetic resonance imaging of the brain revealed bifocal mass lesions in the pineal and suprasellar regions and non-communicating hydrocephalus. We initially suspected a germinoma based on imaging findings, but all tumor markers, including PLAP, in the spinal fluid were negative. Based on these results, germinoma was considered less likely, and an endoscopic third ventriculostomy and endoscopic tumor biopsy were performed for diagnosis. The histopathological diagnosis was PPTID, corresponding to World Health Organization grade 3, in both pineal and suprasellar specimens. A craniotomy for tumor removal was performed, resulting in total resection. PLAP is known to have high sensitivity and extremely high negative predictive value for germinomas. Although bifocal lesions highly suggest germ cell tumors, there are exceptions, as in the present case. This case suggests that PLAP measurements are useful for differentiation, leading to appropriate treatment strategies.

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来源期刊
Child's Nervous System
Child's Nervous System 医学-临床神经学
CiteScore
3.00
自引率
7.10%
发文量
322
审稿时长
3 months
期刊介绍: The journal has been expanded to encompass all aspects of pediatric neurosciences concerning the developmental and acquired abnormalities of the nervous system and its coverings, functional disorders, epilepsy, spasticity, basic and clinical neuro-oncology, rehabilitation and trauma. Global pediatric neurosurgery is an additional field of interest that will be considered for publication in the journal.
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