Bustinza-Linares Ernesto, Socola Francisco, Ernani Vinicius, Miller Shelly A, Trent Jonathan C
Division of Hematology and Oncology, Department of Medicine, Sylvester Comprehensive Cancer Center, University of Miami, Miami, FL 33136, USA.
Case Rep Oncol Med. 2012;2012:621025. doi: 10.1155/2012/621025. Epub 2012 Nov 18.
A 28-year-old female with history of chest wall extraskeletal myxoid chondrosarcoma (EMC) presented to the emergency department complaining of two weeks of lightheadedness and fatigue. Laboratories showed hemoglobin of 7.6 g/dL and a positive hemoccult test. Upper and lower endoscopies were unremarkable, and the patient was discharged after blood transfusion. The next day she returned to the ED with left-sided weakness and perioral numbness. Brain CT scan revealed a 6 cm right frontal mass with midline shift and edema that required urgent craniotomy with resection of a hemorrhagic tumor. The patient continued dropping her hemoglobin, and CT scans showed a rounded 3 cm small bowel mass in the mid ileum. Repeat upper endoscopy revealed a 2 × 2 cm ulcerated mass in the fourth portion of the duodenum. The patient was taken to the operating room and was found to have two lesions; one in the distal duodenum and a second one in the mid ileum causing small bowel intussusception. Pathology was consistent with metastatic EMC grade 2/3, involving the bowel and mesenteric fat. Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft-tissue sarcoma with unique features that distinguishes, it from other sarcomas. It has been often described as a low-grade sarcoma although there are certain characteristics like high mitotic activity and the presence of focal regions of Ki67 staining above 25% that correlate with aggressive behavior of the tumor. This is the first case of EMC metastatic to the small bowel to be reported to the medical community.
一名28岁女性，有胸壁骨外黏液样软骨肉瘤（EMC）病史，因两周来头晕和疲劳就诊于急诊科。实验室检查显示血红蛋白为7.6 g/dL，潜血试验阳性。上消化道和下消化道内镜检查均无异常，患者输血后出院。第二天，她因左侧肢体无力和口周麻木再次返回急诊科。脑部CT扫描显示右额叶有一个6 cm的肿块，伴有中线移位和水肿，需要紧急开颅切除出血性肿瘤。患者血红蛋白持续下降，CT扫描显示回肠中部有一个3 cm的圆形小肠肿块。再次进行上消化道内镜检查发现十二指肠第四段有一个2×2 cm的溃疡性肿块。患者被送往手术室，发现有两个病灶；一个在十二指肠远端，另一个在回肠中部，导致小肠套叠。病理结果与2/3级转移性EMC一致，累及肠道和肠系膜脂肪。骨外黏液样软骨肉瘤（EMC）是一种罕见的软组织肉瘤，具有独特的特征，使其有别于其他肉瘤。尽管它通常被描述为低级别肉瘤，但它具有某些特征，如高有丝分裂活性以及Ki67染色局部区域高于25%，这些特征与肿瘤的侵袭性行为相关。这是首例向医学界报道的EMC转移至小肠的病例。
