Prieto-Granada Carlos N, Ganim Rose B, Zhang Lei, Antonescu Cristina, Mueller James
1 The University of Alabama at Birmingham, Birmingham, AL, USA.
2 Baystate Medical Center-University of Massachusetts Medical School Regional Campus, Springfield, MA, USA.
Int J Surg Pathol. 2017 Sep;25(6):518-525. doi: 10.1177/1066896917706413. Epub 2017 Apr 28.
Primary pulmonary myxoid sarcoma (PPMS) is a recently described, exceedingly rare low-grade lung sarcoma that tends to present in young females as an endobronchial mass and shows evidence of an EWSR1- CREB1 fusion. Herein, we present a case of PPMS with fluorescence in situ hybridization (FISH) analysis for EWSR1 and CREB1 rearrangements. An 80-year-old woman presented with an endobronchial, multinodular tumor exhibiting spindle, ovoid and epithelioid cells arranged in reticular/lattice-like and alveolar-like patterns in a myxoid background. The tumor showed focal epithelial membrane antigen immunoreactivity as well as an Alcian blue-positive stroma that was sensitive to digestion with hyaluronidase. EWSR1 and CREB1 rearrangements were detected by break-apart FISH probes. The patient showed persistence of disease 36 months after diagnosis and was discharged to hospice care. We contribute with a report of an additional case of this very unusual entity and perform a brief review of the literature published so far on the subject.
原发性肺黏液样肉瘤（PPMS）是一种最近才被描述的极为罕见的低级别肺肉瘤，多见于年轻女性，表现为支气管内肿物，且有EWSR1-CREB1融合的证据。在此，我们报告一例通过荧光原位杂交（FISH）分析EWSR1和CREB1重排的PPMS病例。一名80岁女性患者，其支气管内出现多结节肿瘤，肿瘤细胞呈梭形、卵圆形和上皮样，在黏液样背景中呈网状/格子状及肺泡样排列。肿瘤显示局灶性上皮膜抗原免疫反应性，以及对透明质酸酶消化敏感的阿利新蓝阳性间质。通过断裂分离FISH探针检测到EWSR1和CREB1重排。该患者在诊断后36个月疾病持续存在，随后出院接受临终关怀。我们报告了这一非常罕见实体的另一病例，并对目前已发表的相关文献进行了简要综述。
