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立体定向放射治疗后出现软骨样化生的间变性室管膜瘤:病例说明

An anaplastic ependymoma with chondroid metaplasia after stereotactic radiosurgery: illustrative case.

作者信息

Ishikawa Takayuki, Kishida Yugo, Kato Takenori, Iwata Hiromitsu, Hasegawa Toshinori, Nagatani Tetsuya, Seki Yukio

机构信息

Department of Neurosurgery, Japanese Red Cross Aichi Medical Center Nagoya Daini Hospital, Nagoya, Aichi Prefecture, Japan.

Department of Neurosurgery, Tokyo D Tower Hospital, Tokyo, Tokyo Prefecture, Japan.

出版信息

J Neurosurg Case Lessons. 2025 Aug 4;10(5). doi: 10.3171/CASE25308.

Abstract

BACKGROUND

Anaplastic ependymomas are malignant CNS tumors with poor prognosis. Chondroid metaplasia in ependymomas is extremely rare, and its clinical significance remains unclear.

OBSERVATIONS

A 21-year-old male underwent gross-total resection and adjuvant radiotherapy for an anaplastic ependymoma. Over the following 6 years, he experienced multiple recurrences treated with endoscopic resections, Gamma Knife radiosurgery, temozolomide, and bevacizumab. Seven years after initial surgery, a previous irradiated cerebellar lesion showed aggressive regrowth and was resected. Histopathology revealed chondroid metaplasia within the recurrent tumor. Although temporary control of the tumor within the irradiated field with proton therapy was achieved, the rapid progression of disseminated lesions proved fatal.

LESSONS

This is the fifth reported case of chondroid metaplasia in an ependymoma following radiotherapy. Once metaplasia occurs, the prognosis is highly unfavorable, particularly in anaplastic cases. Although proton therapy was administered under suboptimal conditions, the initial tumor control suggests it may be more effective if applied earlier. In the absence of effective chemotherapy, repeated minimally invasive surgeries and radiosurgeries may help preserve cognitive function and control disease progression. https://thejns.org/doi/10.3171/CASE25308.

摘要

背景

间变性室管膜瘤是预后较差的恶性中枢神经系统肿瘤。室管膜瘤中的软骨化生极为罕见,其临床意义尚不清楚。

观察结果

一名21岁男性因间变性室管膜瘤接受了全切手术及辅助放疗。在接下来的6年里,他经历了多次复发,接受了内镜下切除术、伽玛刀放射外科手术、替莫唑胺和贝伐单抗治疗。初次手术后7年,先前接受过放疗的小脑病灶出现侵袭性再生长并被切除。组织病理学显示复发性肿瘤内有软骨化生。尽管质子治疗暂时控制了放疗区域内的肿瘤,但播散性病灶的快速进展被证明是致命的。

经验教训

这是放疗后室管膜瘤中第五例报告的软骨化生病例。一旦发生化生,预后非常不利,尤其是在间变性病例中。尽管质子治疗是在不太理想的条件下进行的,但最初的肿瘤控制表明,如果更早应用可能会更有效。在缺乏有效化疗的情况下,重复的微创手术和放射外科手术可能有助于保留认知功能并控制疾病进展。https://thejns.org/doi/10.3171/CASE25308

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/30fa/12320730/94a6c4646c20/CASE25308_figure_1.jpg

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