Abstract
BackgroundPrimary pulmonary myxoid sarcoma (PPMS) is an extremely rare lung sarcoma that is characterized in most cases by recurrent balanced chromosomal translocation t(2;22)(q33;q12) leading to the oncogenic fusion gene EWSR1-CREB1.Case presentationWe report a case of PPMS with molecular confirmation using fluorescence in situ hybridization (FISH) and DNA sequencing in a 45-year-old female patient. Computer tomography (CT) scanning revealed a peripheral circumscribed solid mass of 2.1 × 2 cm in the right lung superior lobe. Histologically, the tumor cells ranged from stellate, polygonal to chondrocyte-like or physaliferous-like, forming reticular network of delicate lace-like cellular strands and cords in abundant myxoid stroma. The tumor cell immunophenotype was positive for vimentin, EMA and negative for CK-pan, TTF-1, CAM5.2, S-100, calponin, SMA, desmin, ALK, CD31 and CD34. Molecular analysis demonstrated EWSR1-CREB1 gene fusion in this tumor. During 38 months of follow-up, the patient was alive with no clinical or radiological evidence of recurrence or metastasis.ConclusionPPMS is a rare low-grade sarcoma with distinct histological and genetic features. We add another case to the literature of this rare tumor and report for the first time occurrence of chondrocyte-like and physaliferous-like tumor cells in this tumor, thus enriching its morphologic and cytologic spectrum.
Highlights
Primary pulmonary myxoid sarcoma (PPMS) is an extremely rare lung sarcoma that is characterized in most cases by recurrent balanced chromosomal translocation t(2;22)(q33;q12) leading to the oncogenic fusion gene EWSR1-CREB1.Case presentation: We report a case of PPMS with molecular confirmation using fluorescence in situ hybridization (FISH) and DNA sequencing in a 45-year-old female patient
PPMS is a rare low-grade sarcoma with distinct histological and genetic features. We add another case to the literature of this rare tumor and report for the first time occurrence of chondrocyte-like and physaliferous-like tumor cells in this tumor, enriching its morphologic and cytologic spectrum
We report another case of PPMS harboring the EWSR1-CREB1 gene fusion confirmed by molecular method, with review of the literature
Summary
PPMS is a rare low-grade sarcoma that occurs mostly in middle age women with most harboring the characteristic EWSR1-CREB1 fusion gene. Reticular network with delicate lacelike strands and cords of cells within a prominent myxoid stroma, tumor cells showed moderate to marked atypia. Reticular network with NR delicate lacelike strands and cords of cells within a prominent myxoid stroma, tumor cells showed mild to moderate atypia. Multinodular, reticular network with delicate lacelike strands and cords of cells within prominent myxoid stroma, tumor cells showed mild to moderate atypia. 19(7) 54 F EBC+ 4.5 Multinodular, reticular network with delicate lacelike strands and cords of cells within prominent myxoid stroma, tumor cells showed moderate atypia. 26 45 F EBC- 2.1 Well circumscribed, multinodular, reticular network of (our delicate lace-like cellular strands and cords in abundant case) myxoid stroma, chondrocyte or physaliferous-like tumor cells with mild atypia.
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