[Multiple primary sarcomatoid carcinoma of the jejunum with postoperative lung and brain metastases: a case report and literature review]

Nan Fang Yi Ke Da Xue Xue Bao. 2017 Dec 20;37(12):1695-inside back cover. doi: 10.3969/j.issn.1673-4254.2017.12.23.
[Article in Chinese]

Abstract

We report a rare case of a multiple sarcomatoid carcinoma of the jejunum with postoperative lung and brain metastases. The patient underwent jejunum segmental resection for intussusception and gastrointestinal bleeding. Multiple metastasis ofbrain and lung occurred 4 months after the operation, and the patient died for multiple organ failure 8 months after the surgery. Primary sarcomatoid carcinoma was difficult to diagnose at an early stage, and the diagnosis relies on optical microscopic and immunohistochemical observations. Currently no guidelinesare established for treatment for sarcomatoid carcinoma of the jejunum, and surgical resection remains the optimal therapeutic approach. Previous reports documented a poor prognosis of the patients with a median survival time of 5.5 months (0.36-36months), and the primary causes of death were tumor recurrence and metastasis. Ki-67 as a potential prognostic marker and the value of PD-L1-targeted immunotherapy for the treatment await further investigation.

目的: 探讨空肠肉瘤样癌患者的临床表现、治疗及预后等特点,为临床诊治提供参考。

方法: 本文报道1例我院收治的空肠多发肉瘤样癌术后肺、脑转移患者,并分析14篇个案报道、共18例病例。

结果: 患者因肠套叠并消化道出血,行空肠部分切除术,术后4月出现脑、肺等多发转移,术后8月因多器官功能衰竭而死亡。空肠肉瘤样癌国内外命名不统一,诊断困难,尤其难以早期明确诊断,主要诊断依据为病理检查和免疫组化。目前无明确的治疗指南,包括肿瘤在内的广泛手术切除是主要有效治疗手段,但预后极差,中位生存期仅为5.5月(0.36~36月),多死于复发和转移。

结论: 空肠肉瘤样癌较罕见,本例空肠多发性发病并发脑等部位转移更加罕见。规范命名、尽早诊断、通过全身多部位扫描明确其他部位是否存在癌灶、充分评估病程以及尽早治疗均十分重要。手术是治疗的基础,可尝试运用各种肿瘤局部治疗手段。Ki-67作为预后判断和PD-L1靶向免疫治疗的潜在价值需进一步探究。

Publication types

  • Case Reports
  • Review

MeSH terms

  • Brain Neoplasms / secondary*
  • Fatal Outcome
  • Humans
  • Jejunal Neoplasms / pathology*
  • Jejunal Neoplasms / surgery
  • Lung Neoplasms / secondary*
  • Neoplasm Recurrence, Local
  • Neoplasms, Multiple Primary / pathology*
  • Neoplasms, Multiple Primary / surgery