一例罕见的中枢神经系统胰母细胞瘤转移病例:示例。

Vincenzo Mastropasqua, Marco Obersnel, Vito Stifano, Marco Gessi, Domenico Cristiano Corsi, Francesco Doglietto
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引用次数: 0

摘要

背景:胰母细胞瘤是一种胰腺恶性肿瘤:胰母细胞瘤是一种胰腺恶性肿瘤,通常发生在儿童身上,成人很少见。治疗方法包括手术和多种辅助疗法。肝转移很常见,而脑扩散则异常罕见:作者报告了一例 42 岁男性患者的病例,该患者有 16 年的转移性胰腺母细胞瘤病史,曾接受过手术、化疗和放疗,并取得了部分疗效。他出现头痛和头晕,脑磁共振成像(MRI)显示小脑病变。他接受了开颅手术,肿瘤被完全切除,术后恢复顺利。脑部核磁共振成像显示病灶已被完全切除,患者术前症状有所改善后出院。组织病理分析确诊为胰母细胞瘤转移。患者接受了辅助立体定向放射治疗,最后一次随访时临床症状进一步改善:启示:胰母细胞瘤脑转移异常罕见,文献中的描述也很少。目前还没有针对这种情况的标准疗法;因此,患者通常会接受与其他中枢神经系统转移瘤类似的治疗。https://thejns.org/doi/10.3171/CASE23764。
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A rare case of central nervous system pancreatoblastoma metastasis: illustrative case.

Background: Pancreatoblastoma is a malignant neoplasm of the pancreas, occurring usually in children and rarely in adults. Treatment consists of surgery with a variable combination of adjuvant therapies. Liver metastases are common, whereas brain diffusion is exceptionally rare.

Observations: The authors report the case of a 42-year-old man with a 16-year history of metastatic pancreatoblastoma, previously treated with surgery, chemotherapy, and radiotherapy, demonstrating a partial response. He presented with headache and dizziness, and brain magnetic resonance imaging (MRI) showed a cerebellar lesion. A craniotomy was performed with complete tumor removal, and the postoperative course was uneventful. Brain MRI showed gross-total resection of the lesion, and the patient was discharged with an improvement of the preoperative symptoms. Histopathological analysis confirmed the diagnosis of metastasis from pancreatoblastoma. The patient received adjuvant stereotactic radiotherapy and showed further clinical improvement at the last follow-up.

Lessons: Brain metastases from pancreatoblastoma are exceptionally rare and poorly described in the literature. There is no standard therapy for this condition; hence, patients usually undergo treatments similar to those for other central nervous system metastases. All the described patients have had good clinical outcomes yet short-term follow-ups; therefore, further investigations are needed to better understand the best treatments for this condition. https://thejns.org/doi/10.3171/CASE23764.

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