Esophageal Gastrointestinal Stromal Tumor with Rare Intracranial Metastasis

Abstract

介绍。胃肠道间质瘤（GIST）是间充质肿瘤，构成最大的肿瘤组。但是，它们不代表超过1％的原发性消化肿瘤。它们通常转移到肝脏和腹膜，但脑转移非常罕见。Clinical Case。A 76-year-old woman with a diagnosis of esophageal GIST with liver and lung metastases for 13 years, medicated with imatinib, is presented. She was brought to the emergency department after falling and due to changes in behavior and vertigo with 24 hours of evolution. On physical examination, she presented changes in behavior, dysarthria, dysmetria on the right, gait imbalance, and no motor or sensory deficits. On brain computed tomography and posteriorly on magnetic resonance, 2 lesions were observed, left frontal and right cerebellar, compatible with metastatic lesions. After contribution of neurosurgery, histology was obtained that confirmed the lesions were GIST metastases. Imatinib was maintained, and whole brain radiotherapy was performed. After 6 months, she died.讨论。GIST脑转移的稀有性是值得注意的，因此，没有足够的经验来确定最佳治疗。我们的患者用伊马替尼患有优秀的疾病控制，但它不会越过血脑屏障的事实使其不能用于预防或治疗脑病变。新的酪氨酸激酶抑制剂可能越过血脑屏障可能是这些病例的答案。

1.介绍

Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors and constitute the largest group of nonepithelial digestive neoplasms; however, they do not represent more than 1% of primary digestive tumors [1]。They are more commonly found in the stomach, jejunum, and ileum and rarely arise from the esophagus [2]。

GISTs may present with malignant behavior, depending on the mitotic rate, primary site, size, and metastases, in up to 30%. When there are metastases, they commonly are found in the liver and peritoneum. Brain metastases are extremely rare [2]。

Imatinib, a tyrosine kinase inhibitor (TKI), used to treat GISTs with outstanding success, does not cross the blood-brain barrier and seems to have a limited role in treating patients with intracranial metastases [3,4]。

我们present a case of an esophageal GIST treated with imatinib with success, but that presented later with rare intracranial metastasis.

2.临床案例

A 76-year-old woman with a diagnosis of esophageal GIST with liver and lung metastases for 13 years, medicated with imatinib 400 mg per day, was brought to the emergency department after falling. She presented changes in behavior, dysarthria, dysmetria of the right limbs, and gait imbalance with 24 hours of evolution. She had no sensory or motor deficits.

On brain computed tomography (CT), 2 contrast capturing lesions with halo of edema were observed, left frontal and right cerebellar. The frontal lesion had an associated bleeding area of 3 cm in diameter. The findings were suggestive of metastatic lesions (Figure1）。

Thirteen years earlier, she was started on imatinib with an excellent result, with resolution of lung and liver metastases, maintaining only a stable esophageal thickening over the years.

随着GIST脑转移稀有并且能够更好地表征病变，获得了正电子发射断层扫描计算机断层扫描（PET-CT）和脑的磁共振成像（MRI）。PET-CT描述了已经已知的代谢活性食管病变，具有可能的相邻神经节转移（图2）。MRI证实了与脑转移相容的2个病变（图3）。

在神经外科的贡献之后，进行正面病变的切除活组织检查，获得组织学，显示主轴细胞肿瘤，其免疫组化研究表明CD117，DOG1和CD34的阳性，与GIST转移相容（图4）。

治疗选择是维持伊马替尼并进行全脑放射治疗，但没有改善。她的临床状态逐渐恶化，直到她在第一次入场后约6个月死亡。

3.讨论

GIST脑转移的罕见是值得注意的[2,5,6]。在文献中只描述了少数案例，因为这是缺乏经验和证据来确定最佳治疗方法[2]。我们的患者用伊马替尼患有优秀的疾病控制，但它不会越过血脑屏障的事实使其不能用于预防或治疗脑病变。

有一种情况[3[描述了使用Sunitinib的其他TKI的脑病变减少，渗透血脑屏障，但是在少数情况下，难以证明其与切除或放射疗法有关的优势。尽管如此，可能会跨越血脑屏障的TKI可以在未来应得的研究。

数据可用性

The data used to support the findings of this study are available from the corresponding author upon request.

利益冲突

The authors declare that there are no conflicts of interest regarding the publication of this paper.

参考资料

1. I. Judson and G. Demetri, “Advances in the treatment of gastrointestinal stromal tumours,”肿瘤学史，卷。18，不。10，pp。20-24,2007。查看在：Publisher Site|谷歌学术
2. M. Prablek, V. M. Srinivasan, A. Srivatsan et al., “Gastrointestinal stromal tumor with intracranial metastasis: case presentation and systematic review of literature,”BMC Cancer，卷。19，没有。1，p。1119,2019。查看在：Publisher Site|谷歌学术
3. H. Takeuchi，H.Koike，T. Fujita，H. Tsujino，以及Ywamoto，“Sunitinib治疗Jejunal胃肠间质瘤的多脑转移：案例报告”Neurologia Medico-Chirurgica，卷。54，没有。8，pp。664-669,2014。查看在：Publisher Site|谷歌学术
4. T. Tanaka，“颅内转移的胃肠道基质肿瘤：治疗战略和文献审查，”Brain Metastases from Primary Tumors, Academic Press, Cambridge, MA, USA, 2016.查看在：Publisher Site|谷歌学术
5. H. Naoe, E. Kaku, Y. Ido et al., “Brain metastasis from gastrointestinal stromal tumor: a case report and review of the literature,”Case Reports in Gastroenterology，卷。5，不。3，pp。583-589，2011。查看在：Publisher Site|谷歌学术
6. K. Sato, T. Tanaka, N. Kato, T. Ishii, T. Terao, and Y. Murayama, “Metastatic cerebellar gastrointestinal stromal tumor with obstructive hydrocephalus arising from the small intestine: a case report and review of the literature,”肿瘤医学案例报告，卷。2014年，第343178条，6页，2014年。查看在：Publisher Site|谷歌学术

版权

版权© 2020 José Carvalho et al. This is an open access article distributed under theCreative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.