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  • br Case report A year old man

    2018-10-29


    Case report A 43-year-old man presented with odynophagia, progressive swallowing difficulty, and weight loss of 4 kg in 3 months. The patient had been smoking one pack of cigarettes per day for more than two decades and had a peptic ulcer, which had been under medical control for 5 years. Esophagography disclosed a huge tumor with severe mucosal destruction about 10 cm in length in the upper esophagus (Fig. 1). Endoscopic examination of the esophagus showed a huge tumor with irregular mucosa with ulceration 15 cm from the incisors, and the endoscope could not pass through this region (Fig. 2). Bronchoscopy demonstrated external compression on the posterior wall in the upper to middle trachea with obvious narrowing of up to 50% of the lumen. A computed tomography (CT) scan of the chest and upper abdomen showed a huge mass in the upper third of the esophagus with luminal widening (Fig. 3), but no direct invasion of adjacent structures or regional lymphadenopathy. No evidence of metastasis was found from the position emission tomography (PET) and whole-body bone scans. The tumor markers α-fetoprotein (AFP), squamous cell carcinoma antigen (SCC), and carcinoembryonic antigen (CEA) were all within normal limits. Histopathological examination via endoscopic biopsy showed high-grade pleomorphic spindle cell proliferation with an intersecting fascicular growing pattern and increased mitotic activities. These tumor niclosamide Supplier showed no immunoreaction to AE1/AE3, EMA, desmin, CD117, S100, or CD34. A high-grade pleomorphic sarcoma was diagnosed. The patient first received neo-adjuvant chemotherapy with the MAID [mesna (700 mg), doxorubicin (15 mg/m2), ifosfamide (2.5 mg/m2), and dacarbazine (250 mg/m2)] regimen. After completion of six courses of chemotherapy, a CT scan of the chest demonstrated that the tumor had mildly shrunk in size. Although smaller, it still caused obvious symptoms such as odynophagia and dysphagia. In this period, the patient could tolerate a liquid diet. His body weight was within an acceptable range. The patient underwent radical surgery 1 year after diagnosis. Right-sided video-assisted minithoracotomy was carried out. The operation consisted of total esophagectomy, cardiectomy, and total laryngectomy with three-field radical lymph node dissection. Reconstruction was performed using a gastric tube transposed to the neck via the posterior mediastinal route. Pylomyotomy and feeding jejunostomy were also performed. During the operation, one huge tumor was noted in the upper esophagus just below the esophageal inlet. Enlarged lymph nodes were found in the para-esophgeal and parapancreatic areas. The surgical specimen contained a huge intraluminal tumor with only a thin transverse pedicle stalk 3 cm in length connecting it to the esophageal mucosa. The gross size of the tumor was 7 × 5 × 3 cm. It was located near the esophageal inlet (Fig. 4) and had a firm and highly vascular nature. The tumor had only invaded the submucosal layer, and there were adequate peripheral margins of uninvolved tissue in all planes. The second histopathological examination revealed the same tumor morphology as that of the initial examination, and no lymph node metastasis was found (Fig. 5). Immunohistochemical staining was AE1/AE3 (–), EMA (–), desmin (–), CD34 (–), CD-117 (–), S-100 (focal +), CD68 (focal +), CD163 (focal +), CD35 (–), and actin-M851 (focal +). The immunohistochemical staining showed complete absence of reactions with any lineage-selective markers. The diagnosis of high-grade undifferentiated pleomorphic sarcoma was confirmed.
    Discussion Primary sarcomas of the esophagus are rare neoplasms. In a previous study, sarcomas and carcinosarcomas accounted for approximately 0.1–1.5% of all esophageal tumors and comprised only 5% of all gastrointestinal sarcomas. Leiomyosarcoma is the most common cell type of the esophageal sarcomas. The rarity of esophageal sarcoma is illustrated by the small number of previous reports, consisting of single or small groups of patients. The clinical symptoms of esophageal sarcomas are nonspecific and different from those of esophageal carcinoma, and include progressive dysphagia, loss of weight, regurgitation, retrosternal pain, respiratory distress, odynophagia, sensation of a lump in the throat, hemorrhage, anemia, sudden death (due to asphyxia), vomiting (food or tumor fragments), fever, and cough. Our patient presented with odynophagia and dysphagia initially, which was consistent with symptoms in previously reported cases.