Weiler Eric J, Murickan Thomas, Drayer Crystal N, Samat Sajjaad H, Kia Michael A
Sparrow Hospital, 1200 E, Michigan Ave, #255, Lansing, MI, 48912, USA.
University of Nebraska Medical Center, 983285 Nebraska Medical Center, Omaha, NE, 68198, USA.
Surg Case Rep. 2023 Jul 3;9(1):123. doi: 10.1186/s40792-023-01662-3.
Low-grade fibromyxoid sarcoma (LGFMS) is an uncommon neoplasm generally affecting muscle tissue. It presents rarely in abdominal viscera and even more rarely occurs in the pancreas. All types of pancreatic sarcomas are uncommon, and LGFMS is a rarer still. We present the case of an LGFMS in the pancreas. Because of its rarity, there are no guidelines for appropriate treatment or summations of the natural course of this illness.
We present the case of a 49-year-old female who presented with epigastric pain. She had a prior history of three episodes of acute pancreatitis many years earlier. A CT revealed a pancreatic body mass, which was biopsied. Pathology returned LGFMS. The patient underwent a distal pancreatectomy and splenectomy. She did well after the case and did not require further intervention.
Though it is exceedingly rare, cases of pancreatic LGFMS should be reported in order to guide clinical decisions. LGFMS has been shown to have high malignant potential in other tissues, and there is no reason to think pancreatic masses will be different. By building a body of evidence about these rare tumors, patient care will benefit.
低度恶性纤维黏液样肉瘤（LGFMS）是一种罕见的肿瘤，通常累及肌肉组织。它很少出现在腹部脏器，在胰腺中更为罕见。所有类型的胰腺肉瘤都不常见，而LGFMS更是罕见。我们报告一例胰腺LGFMS病例。由于其罕见性，目前尚无关于适当治疗的指南或该疾病自然病程的总结。
我们报告一例49岁女性，因上腹部疼痛就诊。她多年前有过三次急性胰腺炎发作史。CT显示胰体有肿块，遂进行活检。病理结果为LGFMS。患者接受了胰体尾切除术和脾切除术。术后恢复良好，无需进一步干预。
尽管胰腺LGFMS极为罕见，但仍应报告此类病例以指导临床决策。LGFMS在其他组织中已显示出高恶性潜能，没有理由认为胰腺肿块会有所不同。通过积累关于这些罕见肿瘤的证据，将有利于患者的治疗。
