PMID- 28449608 OWN - NLM STAT- MEDLINE DCOM- 20180509 LR - 20180509 IS - 1940-2465 (Electronic) IS - 1066-8969 (Linking) VI - 25 IP - 6 DP - 2017 Sep TI - Primary Pulmonary Myxoid Sarcoma: A Newly Described Entity-Report of a Case and Review of the Literature. PG - 518-525 LID - 10.1177/1066896917706413 [doi] AB - 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. FAU - Prieto-Granada, Carlos N AU - Prieto-Granada CN AD - 1 The University of Alabama at Birmingham, Birmingham, AL, USA. FAU - Ganim, Rose B AU - Ganim RB AD - 2 Baystate Medical Center-University of Massachusetts Medical School Regional Campus, Springfield, MA, USA. FAU - Zhang, Lei AU - Zhang L AD - 3 Memorial Sloan Kettering Cancer Center, New York, NY, USA. FAU - Antonescu, Cristina AU - Antonescu C AD - 3 Memorial Sloan Kettering Cancer Center, New York, NY, USA. FAU - Mueller, James AU - Mueller J AD - 2 Baystate Medical Center-University of Massachusetts Medical School Regional Campus, Springfield, MA, USA. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20170428 PL - United States TA - Int J Surg Pathol JT - International journal of surgical pathology JID - 9314927 RN - 0 (CREB1 protein, human) RN - 0 (Cyclic AMP Response Element-Binding Protein) RN - 0 (EWSR1 protein, human) RN - 0 (RNA-Binding Protein EWS) SB - IM MH - Aged, 80 and over MH - Cyclic AMP Response Element-Binding Protein/genetics MH - Female MH - Gene Rearrangement MH - Humans MH - In Situ Hybridization, Fluorescence MH - Lung Neoplasms/genetics/*pathology MH - RNA-Binding Protein EWS/genetics MH - Sarcoma/genetics/*pathology OTO - NOTNLM OT - EWSR1-CREB1 OT - fluorescence in situ hybridization OT - lung sarcoma OT - primary pulmonary myxoid sarcoma EDAT- 2017/04/30 06:00 MHDA- 2018/05/10 06:00 CRDT- 2017/04/29 06:00 PHST- 2017/04/30 06:00 [pubmed] PHST- 2018/05/10 06:00 [medline] PHST- 2017/04/29 06:00 [entrez] AID - 10.1177/1066896917706413 [doi] PST - ppublish SO - Int J Surg Pathol. 2017 Sep;25(6):518-525. doi: 10.1177/1066896917706413. Epub 2017 Apr 28.