原发性结肠平滑肌肉瘤伴同步肝转移

IF 0.9 Q4 ONCOLOGY Rare Tumors Pub Date : 2022-01-01 DOI:10.1177/20363613221080549
D. Massaras, E. Kontis, K. Stamatis, E. Zampeli, D. Myoteri, Elias Primetis, E. Pantiora, G. Fragulidis
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引用次数: 2

摘要

平滑肌肉瘤(LMS)是起源于平滑肌的间质肿瘤。大约20%的平滑肌肉瘤发生在胃肠道,占胃肠道恶性肿瘤的1-2%。在胃肠道内,小肠是最常见的发病部位,其次是结肠。通常是在腹痛调查中偶然发现的,通常出现在50岁左右。由于胃肠道平滑肌肉瘤与胃肠道间质瘤(gist)在显微镜下具有相同的外观,因此过去的诊断鉴别非常具有挑战性。目前,这些肿瘤通过免疫组织化学方法诊断,因为它们的平滑肌标志物肌动蛋白(SMA)和desmin呈阳性,GIST标志物如受体酪氨酸激酶(KIT)、CD34、DOG1呈阴性,神经鞘瘤标志物S100蛋白呈阴性。在目前的文献中,大多数肠道平滑肌肉瘤的病例局限于小肠,在原发肿瘤出现时没有同步肝转移的报道。在此,我们报告一位在我科住院的病人,诊断为原发性结肠平滑肌肉瘤并同步肝转移。
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Primary leiomyosarcoma of the colon with synchronous liver metastasis
Leiomyosarcomas (LMS) are mesenchymal tumors of smooth muscle origin. Approximately 20% of leiomyosarcomas are found in the GI tract, and account for 1–2% of GI malignancies. Within the gastrointestinal tract, the small intestine is the most common site of presentation followed by the colon. They are often diagnosed incidentally during abdominal pain investigation, and they usually present in the fifth decade of life. In the past, the diagnostic differentiation between leiomyosarcomas of gastrointestinal tract and gastrointestinal stromal tumors (GISTs) was very challenging as they share common microscopic appearance. Nowadays, these tumors are diagnosed by immunohistochemical methods as they are positive for smooth muscle markers actin (SMA) and desmin, negative for GIST markers such as receptor tyrosine kinase (KIT), CD34, DOG1, and negative for the schwannoma marker S100 protein. In the current literature, most cases of intestinal leiomyosarcomas are localized in the small intestine, and there are no reports of synchronous liver metastases at the time of presentation of the primary tumor. Herein, we present a patient who was admitted in our department, with the diagnosis of primary leiomyosarcoma of the colon and synchronous liver metastasis.
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Rare Tumors
Rare Tumors ONCOLOGY-
CiteScore
1.50
自引率
0.00%
发文量
15
审稿时长
15 weeks
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