Hyalinizing spindle cell tumor with giant rosettes arising in the lung: report of a case with FUS-CREB3L2 fusion transcripts.
Hyalinizing spindle cell tumor with giant rosettes (HSCT) is a very uncommon mesenchymal tumor that has similar morphological and biological features to the low-grade fibromyxoid sarcoma (LGFMS). Lung involvement of HSCT is extremely rare, and only one case has been reported in the English-language literature. Reported herein is a case of primary pulmonary HSCT that had FUS-CREB3L2 fusion transcripts, a product of characteristic chromosomal abnormality t(7;16)(q33;p11) of HSCT and LGFMS. The patient was a 50-year-old woman who had a large solitary mass in the lung. Histologically, it was composed of bland spindle cells with variable cellularity deposited in a densely hyalinized stroma alternating with myxoid areas. Characteristic collagen rosettes were scattered in the cellular areas. Reverse transcription-polymerase chain reaction (RT-PCR) assay using formalin-fixed, paraffin-embedded tissue detected FUS-CREB3L2 fusion transcripts. Despite its bland morphology, it is known as low-grade sarcoma and its recognition in the lung would be helpful for accurate diagnosis and proper management of this rare tumor. RT-PCR for detection of FUS-CREB3L2 fusion transcripts is a useful method for differential diagnosis of primary pulmonary HSCT.
Kim
Lucia
L
Department of Pathology, Inha Research Institute for Medical Science, Inha University Hospital, 7-206 3rd Street, Shinheung-dong, Jung-gu, Incheon 400-711, Korea.
Yoon
Yong Han
YH
Choi
Suk-Jin
SJ
Han
Jee Young
JY
Park
In Suh
IS
Kim
Joon Mee
JM
Chu
Young Chae
YC
Kim
Ye Ji
YJ
D014408:Biomarkers, Tumor / Q000378:metabolism; D017362:Cyclic AMP Response Element-Binding Protein / Q000235:genetics / Q000378:metabolism*; D005260:Female; D005354:Fibrosarcoma / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D006801:Humans; D006818:Hyalin / Q000378:metabolism; D008175:Lung Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000378:metabolism*; D015514:Oncogene Proteins, Fusion / Q000235:genetics / Q000378:metabolism*; D020133:Reverse Transcriptase Polymerase Chain Reaction; D014157:Transcription Factors; D016896:Treatment Outcome
D014408:Biomarkers, Tumor; C485618:CREB3L1 protein, human; D017362:Cyclic AMP Response Element-Binding Protein; D009419:Nerve Tissue Proteins; D015514:Oncogene Proteins, Fusion; D014157:Transcription Factors