Epithelioid inflammatory myofibroblastic sarcoma arising in the pleural cavity.
A 57-year-old Japanese man presented with massive right pleural effusion, and a huge tumor arising in the pleural cavity was detected by chest computed tomography. A thoracoscopic tumor biopsy revealed that the tumor protruded extensively into the pleural cavity, and its gross appearance was cystic and glossy. Microscopically, the tumor cells were rounded and epithelioid in shape. Prominent and abundant myxoid stroma was also present together with an inflammatory infiltrate, and the tumor was anaplastic lymphoma kinase (ALK)-immunopositive. Fluorescence in situ hybridization revealed that the Ran-binding protein 2-ALK fusion gene was present. Taken together, these findings supported the diagnosis of epithelioid inflammatory myofibroblastic sarcoma (EIMS), which is a variant of an inflammatory myofibrobrastic tumor. This is the first reported case of an EIMS arising in the pleural cavity.
Division of Thoracic Surgery, Shizuoka Cancer Center, Shimonagakubo 1007, Nagaizumi, Shizuoka, 411-8777, Japan, y.kozu@scchr.jp.
Yoshiki
Y
Kozu
Mitsuhiro
M
Isaka
Yasuhisa
Y
Ohde
Kengo
K
Takeuchi
Takashi
T
Nakajima
D000328:Adult; D000077548:Anaplastic Lymphoma Kinase; D002648:Child; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D007223:Infant; D008297:Male; D008875:Middle Aged; D018832:Molecular Chaperones / Q000235:genetics; D028861:Nuclear Pore Complex Proteins / Q000235:genetics; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D035422:Pleural Cavity; D010997:Pleural Neoplasms / Q000235:genetics* / Q000473:pathology* / Q000601:surgery; D020794:Receptor Protein-Tyrosine Kinases / Q000235:genetics; D012509:Sarcoma / Q000235:genetics* / Q000473:pathology* / Q000601:surgery
D018832:Molecular Chaperones; D028861:Nuclear Pore Complex Proteins; D015514:Oncogene Proteins, Fusion; C094493:ran-binding protein 2; C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D020794:Receptor Protein-Tyrosine Kinases