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松果体实质中等分化肿瘤初始治疗10年后的脊髓播散:一例报告

Spinal Dissemination of Pineal Parenchymal Tumors of Intermediate Differentiation Over 10 Years After Initial Treatment: A Case Report.

作者信息

Miyazaki Airi, Makino Keishi, Shinojima Naoki, Yamashita Shinji, Mikami Yoshiki, Mukasa Akitake

机构信息

Neurosurgery, Kumamoto University Hospital, Kumamoto, JPN.

Neurosurgery, Kumamoto City Hospital, Japan, JPN.

出版信息

Cureus. 2024 Mar 28;16(3):e57147. doi: 10.7759/cureus.57147. eCollection 2024 Mar.

Abstract

Pineal parenchymal tumors (PPTs) are rare, accounting for less than 0.3% of all primary central nervous system (CNS) tumors. Pineal parenchymal tumors of intermediate differentiation (PPTID) (WHO grade 2 or 3) show an intermediate prognosis between pineocytoma and pineoblastoma. The clinical course is unknown, and the optimal treatment for PPTID, especially for recurrence, has not been determined. We report a case of PPTID with spinal dissemination over 10 years after treatment and survival for four years. A 56-year-old woman presented with headaches and diplopia. Computerized tomography (CT) and magnetic resonance imaging (MRI) revealed a pineal mass, but leptomeningeal dissemination was not identified on whole-spine MRI. Microsurgical gross total tumor resection (GTR) was performed, and the pathological diagnosis was PPTID (grade 3). In addition, a later study found it to harbor a mutation. She underwent whole-brain radiation therapy with a focal boost. The patient was unable to continue chemotherapy for severe myelosuppression after the first course of treatment. Eleven years after the surgery, she was unable to walk, and a whole-spine MRI revealed multiple masses at C3-4, T4, and cauda equina. Fluorodeoxyglucose-positron emission tomography (FDG-PET) revealed accumulations of the same lesions. No recurrence was observed in the brain. A biopsy of the caudal portion was performed, and the histopathological findings were the same as those of the initial surgery. Spinal dissemination was refractory to chemotherapy but responded to whole spine radiotherapy with focal boost, and she remained tumor-free for four years. We considered good local control with a combination of GTR and subsequent radiation therapy to contribute to long-term survival. The timing of spinal radiation administration is controversial because of the tendency for late cerebrospinal dissemination. The importance of long-term follow-up of the spine and head is emphasized. In PPTID cases with good local control, withholding spinal radiation until spinal dissemination occurs may become a long-term treatment plan.

摘要

松果体实质肿瘤(PPTs)较为罕见,占所有原发性中枢神经系统(CNS)肿瘤的比例不到0.3%。中间分化型松果体实质肿瘤(PPTID)(世界卫生组织2级或3级)的预后介于松果体细胞瘤和松果体母细胞瘤之间。其临床病程尚不清楚,且PPTID的最佳治疗方法,尤其是针对复发情况的治疗方法尚未确定。我们报告一例PPTID病例,该病例在治疗后10年出现脊髓播散,并存活了4年。一名56岁女性出现头痛和复视症状。计算机断层扫描(CT)和磁共振成像(MRI)显示松果体区有肿块,但全脊柱MRI未发现软脑膜播散。进行了显微手术全切肿瘤(GTR),病理诊断为PPTID(3级)。此外,后续研究发现其存在一种突变。她接受了全脑放疗并局部加量。患者在第一个疗程治疗后因严重骨髓抑制无法继续化疗。手术后11年,她无法行走,全脊柱MRI显示在C3 - 4、T4和马尾部位有多个肿块。氟脱氧葡萄糖正电子发射断层扫描(FDG - PET)显示相同病变部位有积聚。脑部未观察到复发。对尾部肿块进行了活检,组织病理学结果与初次手术相同。脊髓播散对化疗耐药,但对全脊柱放疗并局部加量有反应,她无瘤生存了4年。我们认为GTR联合后续放疗实现的良好局部控制有助于长期生存。由于存在晚期脑脊液播散的倾向,脊髓放疗的时机存在争议。强调了对脊柱和头部进行长期随访的重要性。在局部控制良好的PPTID病例中,在脊髓播散发生之前暂不进行脊髓放疗可能会成为一种长期治疗方案。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2816/11055966/61f4921d3934/cureus-0016-00000057147-i01.jpg

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