Mirzasadeghi Anahita, Jalaeefar Amirmohsen, Jahanbin Behnaz, Avanaki Foroogh Alborzi, Sharifi Amirsina
Department of Surgical Oncology, Cancer Institute, Tehran University of Medical Science, Tehran, Iran.
Cancer Institute, Pathology Department, Imam Khomeini Hospital Complex, Tehran University of Medical Science, Tehran, Iran.
Int J Surg Case Rep. 2023 Aug;109:108578. doi: 10.1016/j.ijscr.2023.108578. Epub 2023 Jul 28.
Esophageal sarcoma is a rare neoplasm of the gastrointestinal tract. The majority of the esophageal neoplasms have an epithelial origin. In this report, we present a case of a middle-aged man with an enormous spindle cell sarcoma whose symptoms initiated only a few weeks before diagnosis.
A 41-year-old man with an unremarkable past medical history and physical examination presented with recent aggravation of cough and severe, progressive dysphagia to solid foods resulting in a 25-kilogram weight loss without any prior symptoms. He had no history of cigarette smoking and alcohol consumption. The CT scan showed a huge soft tissue mass with heterogeneous enhancement from the proximal esophagus to 4 cm above the gastro-esophageal junction, causing luminal bulging. Trans-hiatal esophagectomy and gastric pull-up were performed. Pathology report confirmed the diagnosis of sarcoma. Further pathological evaluation using immune-histochemical studies, confirmed the tumor as spindle cell sarcoma. The postoperative period was uneventful, and there were no signs and symptoms related to tumor recurrence one year after surgery.
The most challenging aspect of diagnosing sarcomas is differentiating them from other pathologies, such as gastrointestinal stromal tumors, synovial sarcomas, sarcomatoid carcinomas, melanomas, and solitary fibrous tumors. Immunohistochemical studies play a vital role in this differentiation. Additionally, cytokeratin AE1/AE3 has been introduced as a marker of epithelial differentiation and can verify the presence of the epithelial component in tumors, such as in carcinosarcomas.
Considering the potential for an unusual size, sarcoma should be considered in a differential diagnosis for huge esophageal masses.
食管肉瘤是胃肠道的一种罕见肿瘤。大多数食管肿瘤起源于上皮组织。在本报告中，我们呈现了一例中年男性巨大梭形细胞肉瘤病例，其症状在诊断前仅数周才出现。
一名41岁男性，既往病史和体格检查均无异常，近期出现咳嗽加重以及严重的、进行性固体食物吞咽困难，体重减轻25千克，此前无任何症状。他无吸烟和饮酒史。CT扫描显示一个巨大的软组织肿块，从食管近端至胃食管交界处上方4厘米处有不均匀强化，导致管腔膨出。行经裂孔食管切除术及胃上提术。病理报告确诊为肉瘤。进一步通过免疫组织化学研究进行病理评估，证实肿瘤为梭形细胞肉瘤。术后恢复顺利，术后一年无肿瘤复发的迹象和症状。
诊断肉瘤最具挑战性的方面是将其与其他病理类型区分开来，如胃肠道间质瘤、滑膜肉瘤、肉瘤样癌、黑色素瘤和孤立性纤维瘤。免疫组织化学研究在这种鉴别中起着至关重要的作用。此外，细胞角蛋白AE1/AE3已被引入作为上皮分化的标志物，可验证肿瘤中上皮成分的存在，如在癌肉瘤中。
考虑到肉瘤可能具有不寻常的大小，对于巨大食管肿块的鉴别诊断应考虑肉瘤。
