Treatment of primary pulmonary malignant fibrous histiocytoma: case report and literature Review

XU Song; Li Xiongfei; SHI Tao; REN Fan; YANG Fan; Liu Renwang; REN Dian; DONG Shangwen; FAN Haiyang; WEI Sen; CHEN Gang; CHEN Jun

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Treatment of primary pulmonary malignant fibrous histiocytoma: case report and literature Review

XU Song 1 *,Li Xiongfei 2,SHI Tao 2,REN Fan 2,YANG Fan 2,Liu Renwang 2,REN Dian 2,DONG Shangwen 2,FAN Haiyang 2,WEI Sen 2,CHEN Gang 2,CHEN Jun 2

1.Department of Lung Cancer Surgery, Tianjin Medical University General Hospital

2.Department of Lung Cancer Surgery, Tianjin Medical University General Hospital

*Correspondence author

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Funding: Specialized Research Fund for the Doctoral Program of Higher Education （No.20131202120004),National Natural Science Foundation of China）

Opened online:21 April 2017

Accepted by: none

Citation: XU Song,Li Xiongfei,SHI Tao.Treatment of primary pulmonary malignant fibrous histiocytoma: case report and literature Review[OL]. [21 April 2017] http://en.paper.edu.cn/en_releasepaper/content/4725565

Objective: Malignant fibrous histiocytoma (MFH) is an aggressive soft tissue sarcoma which can occur in various organs. However, primary MFH arising from lung is quite rare. Method: Herein we reported a case of 61-year-old male with primary pulmonary MFH and explore the underlying mechanisms by next generation sequencing. Results: Five gene mutations, including TSC2, ARID1B, CDK8, KDM5C and CASP8, were detected and the mTOR inhibitor might be an efficient treatment for this patient. In addition, we reviewed in the English literature approximately 36 case reports of primary pulmonary MFH since 1979 and summarized the clinical features and prognosis of this rare pulmonary malignant tumor.Conclusion:MFH is not sensitive to chemo- and radio-therapy. Once the diagnosis is confirmed, a complete surgical resection is necessary. Target therapy by mTOR inhibitors might be a promising treatment.

Keywords:malignant fibrous histiocytoma; sarcoma; surgery

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