Leiomyosarcoma of stomach extending to gastroesophageal junction and distal esophagus as a rare cause of dysphagia -A case report

Lilamani Rajthala, Sagar Gyawali, Sabin Banmala, Surendra Shah
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Abstract

Gastric leiomyosarcoma is a rare malignant tumor among the primary gastric carcinomas. Among the different common presentations, dysphagia is an uncommon presentation of gastric leiomyosarcoma. A 29-year-old female presented with complaints of progressive dysphagia for one year associated with vomiting, significant weight loss, and anorexia for six months. On blood investigations, she had anemia, hypokalemia, pre-renal acute kidney injury, and unconjugated hyperbilirubinemia. Upper gastrointestinal endoscopy and contrast-enhanced computed tomography (CECT) were initially suggestive of carcinoma of stomach. Immunohistochemistry was diagnostic of leiomyosarcoma of stomach extending to the gastroesophageal junction and distal esophagus. She underwent total gastrectomy with distal esophagectomy with lateral segmentectomy of liver (non-anatomical) with Roux-en-Y esophago-jejunal anastomosis (end-to-side and retro-colic)) through thoracoabdominal approach. After six weeks, she received four cycles of doxorubicin therapy. Follow-up at 18 months after surgery revealed no recurrence of malignancy. Leiomyosarcoma, a rare malignant tumor arising from stomach involves commonly gastric body followed by antrum and fundus. Imaging including CECT and tissue diagnosis including immunohistochemistry [positive for α-SMA, desmin, calponin, h-caldesmon, or smoothelin] have been mainstay for definitive diagnosis. The standard treatment for leiomyosarcoma of stomach is complete surgical resection of tumor because it has malignant potential and does not respond to targeted treatment with a tyrosine kinase inhibitor. The type of surgery depends on the size and localization of the tumor. Early diagnosis with proper imaging, immunohistochemistry, and biopsy play important role in differentiating gastric leiomyosarcoma from GIST. Surgical resection is the mainstay of treatment.
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延伸至胃食管交界处和食管远端的胃雷肌肉瘤是吞咽困难的罕见病因--病例报告
胃癌是原发性胃癌中一种罕见的恶性肿瘤。在胃癌的各种常见表现中,吞咽困难是一种不常见的表现。 一名 29 岁的女性主诉吞咽困难进展一年,伴有呕吐、体重明显下降和厌食六个月。经血液检查,她患有贫血、低钾血症、肾前性急性肾损伤和未结合高胆红素血症。上消化道内窥镜检查和对比增强计算机断层扫描(CECT)初步提示胃癌。免疫组织化学检查诊断为延伸至胃食管交界处和食管远端的胃癌。她接受了全胃切除术、食管远端切除术和肝脏侧段切除术(非解剖性),并通过胸腹腔途径进行了Roux-en-Y食管空肠吻合术(端对端和后结肠)。六周后,她接受了四个周期的多柔比星治疗。术后18个月的随访显示,恶性肿瘤没有复发。 胃癌是一种罕见的恶性肿瘤,通常累及胃体,其次是胃窦和胃底。影像学检查(包括 CECT)和组织诊断(包括免疫组化[α-SMA、desmin、calponin、h-caldesmon 或 smoothelin 阳性])是明确诊断的主要方法。胃亮肌肉瘤的标准治疗方法是彻底手术切除肿瘤,因为它具有恶性潜能,且对酪氨酸激酶抑制剂的靶向治疗无效。手术方式取决于肿瘤的大小和位置。 通过适当的影像学检查、免疫组化和活组织检查进行早期诊断,对于区分胃癌和胃腺体肉瘤具有重要作用。手术切除是治疗的主要方法。
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