Primary pulmonary myxoid sarcoma with EWSR1‐CREB1 fusion, resembling extraskeletal myxoid chondrosarcoma: Case report with a review of Literature

Reported herein is an extremely rare case of primary pulmonary myxoid sarcoma (PPMS). A 31‐year‐old man presented with a 2.7 cm‐sized pulmonary tumor surrounded by capsule‐like fibrosis. The patient has been free of disease for 5.8 years after surgery. This tumor focally showed endobronchial feature...

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Bibliographic Details
Published in:Pathology international 2012-12, Vol.62 (12), p.817-822
Main Authors: Matsukuma, Susumu, Hisaoka, Masanori, Obara, Kiyohaya, Kono, Takako, Takeo, Hiroaki, Sato, Kimiya, Hata, Yoshinobu
Format: Article
Language:English
Subjects:
Adult
Biomarkers, Tumor - analysis
Chondrosarcoma - pathology
EWSR1‐CREB1
extraskeletal myxoid chondrosarcoma
Humans
Immunohistochemistry
lung
Lung Neoplasms - genetics
Lung Neoplasms - pathology
Male
myoepithelioma
Neoplasms, Connective and Soft Tissue - pathology
Oncogene Proteins, Fusion - genetics
primary pulmonary myxoid sarcoma
Reverse Transcriptase Polymerase Chain Reaction
Sarcoma - genetics
Sarcoma - pathology
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Summary:Reported herein is an extremely rare case of primary pulmonary myxoid sarcoma (PPMS). A 31‐year‐old man presented with a 2.7 cm‐sized pulmonary tumor surrounded by capsule‐like fibrosis. The patient has been free of disease for 5.8 years after surgery. This tumor focally showed endobronchial features, and consisted of reticular cords of oval, short spindle, or polygonal cells with swollen vesicular nuclei accompanied by an abundant myxoid stroma, closely resembling extraskeletal myxoid chondrosarcoma. Tumor cells were diffusely positive for vimentin and focally positive for epithelial membrane antigen, but were negative for cytokeratin, TTF‐1, Napsin A, S‐100 protein, CD34, desmin, smooth‐muscle actin, CD10, p63, calponin, h‐caldesmon, c‐kit, HMB‐45, synaptophysin, or glial fibrillary acid protein. Our reverse transcription‐polymerase chain reaction using the formalin‐fixed, paraffin‐embedded tumor tissues detected EWSR1‐CREB1 fusion transcript, but could not demonstrate EWSR1‐ATF1 fusion or EWSR1/TAF15/TFG‐NR4A3 fusion. These findings indicate that the current tumor is an additional case of PPMS with EESR1‐CREB1 fusion, recently reported by Thway et al. Some cases of PPMS can behave in an indolent manner.
ISSN:1320-5463
1440-1827
DOI:10.1111/pin.12014