Kim Lucia, Yoon Yong Han, Choi Suk-Jin, Han Jee Young, Park In Suh, Kim Joon Mee, Chu Young Chae, Kim Ye Ji
Department of Pathology, Inha Research Institute for Medical Science, Inha University Hospital, 7-206 3rd Street, Shinheung-dong, Jung-gu, Incheon 400-711, Korea.
Pathol Int. 2007 Mar;57(3):153-7. doi: 10.1111/j.1440-1827.2006.02073.x.
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.
伴有巨大玫瑰花结的透明变性梭形细胞肿瘤(HSCT)是一种非常罕见的间叶性肿瘤,其形态学和生物学特征与低度纤维黏液样肉瘤(LGFMS)相似。HSCT累及肺部极为罕见,英文文献中仅报道过1例。本文报告1例原发性肺HSCT病例,该病例具有FUS-CREB3L2融合转录本,这是HSCT和LGFMS特征性染色体异常t(7;16)(q33;p11)的产物。患者为一名50岁女性,肺部有一个巨大的孤立性肿块。组织学上,它由细胞密度不一的温和梭形细胞组成,沉积于密集透明变性的间质中,并与黏液样区域交替存在。特征性的胶原玫瑰花结散在于细胞区域。使用福尔马林固定、石蜡包埋组织进行的逆转录-聚合酶链反应(RT-PCR)检测到FUS-CREB3L2融合转录本。尽管其形态温和,但它被认为是低度肉瘤,在肺部识别该肿瘤有助于对这种罕见肿瘤进行准确诊断和恰当处理。检测FUS-CREB3L2融合转录本的RT-PCR是鉴别原发性肺HSCT的一种有用方法。