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中枢神经系统胰腺母细胞瘤转移1例罕见病例:病例展示

A rare case of central nervous system pancreatoblastoma metastasis: illustrative case.

作者信息

Mastropasqua Vincenzo, Obersnel Marco, Stifano Vito, Gessi Marco, Cristiano Corsi Domenico, Doglietto Francesco

机构信息

Departments of Neurosurgery, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy.

Departments of Pathology, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy.

出版信息

J Neurosurg Case Lessons. 2024 Sep 16;8(12). doi: 10.3171/CASE23764.

Abstract

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.

摘要

背景

胰腺母细胞瘤是胰腺的一种恶性肿瘤,通常发生于儿童,成人罕见。治疗包括手术及多种辅助治疗的联合应用。肝转移常见,而脑转移极为罕见。

观察

作者报告了一例42岁男性,患有转移性胰腺母细胞瘤16年,此前接受过手术、化疗和放疗,显示部分缓解。他出现头痛和头晕,脑部磁共振成像(MRI)显示小脑有病变。进行了开颅手术并完整切除肿瘤,术后过程顺利。脑部MRI显示病变已大体全切,患者出院时术前症状有所改善。组织病理学分析证实为胰腺母细胞瘤转移。患者接受了辅助立体定向放疗,在最后一次随访时临床症状进一步改善。

经验教训

胰腺母细胞瘤脑转移极为罕见,文献中对此描述甚少。对于这种情况没有标准治疗方法;因此,患者通常接受与其他中枢神经系统转移瘤相似的治疗。所有已描述的患者临床结局良好,但随访时间短;因此,需要进一步研究以更好地了解针对这种情况的最佳治疗方法。https://thejns.org/doi/10.3171/CASE23764

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b003/11418033/b52f8a7e226a/CASE23764_figure_1.jpg

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