Gao Jidi, Chow Erika, Aloma Aishatu, Gupta Payal
Tufts Medical Center, 800 Washington St, Boston, MA 02111, USA.
Tufts University School of Medicine, 145 Harrison Ave, Boston, MA 02111, USA.
Radiol Case Rep. 2018 Feb 5;13(2):392-396. doi: 10.1016/j.radcr.2018.01.003. eCollection 2018 Apr.
Primitive neuroectodermal tumor is a high-grade malignant tumor originating from the neural crest and neuroectoderm, which can be subdivided into central and peripheral categories. Peripheral primitive neuroectodermal tumor is thought to be identical to Ewing's sarcoma, and falls under a broader category of Ewing's sarcoma family of tumors. Very rarely, it may present without osseous involvement, known as extraosseous Ewing's sarcoma. Here we present a case of a 38-year-old woman, who presented with several-month history of a slow-growing chest wall mass, initially thought to be a breast mass. The mass was diagnosed as extraosseous Ewing's sarcoma upon tissue biopsy. The patient was started on a dose-intensified neoadjuvant therapy, based on protocol from pediatric population given rarity of this tumor in the adult population. While the patient was initially planned for surgical resection, the tumor showed excellent response to chemotherapy on follow-up imaging, and radiation therapy was elected in lieu of resection.
原始神经外胚层肿瘤是一种起源于神经嵴和神经外胚层的高级别恶性肿瘤，可分为中枢型和外周型。外周原始神经外胚层肿瘤被认为与尤因肉瘤相同，属于更广泛的尤因肉瘤肿瘤家族。极少数情况下，它可能不累及骨骼，称为骨外尤因肉瘤。在此，我们报告一例38岁女性患者，她有几个月缓慢生长的胸壁肿块病史，最初被认为是乳腺肿块。经组织活检，该肿块被诊断为骨外尤因肉瘤。鉴于该肿瘤在成人中罕见，根据儿科治疗方案，患者开始接受剂量强化新辅助治疗。虽然最初计划对患者进行手术切除，但随访影像学显示肿瘤对化疗反应良好，因此选择放疗代替手术切除。
