Title: | A case of radio-insensitive SMARCA4-deficient thoracic undifferentiated carcinoma with severe right heart failure |
Authors: | Ito, Shotaro Browse this author |
Asahina, Hajime Browse this author |
Yamaguchi, Naoko Browse this author |
Tomaru, Utano Browse this author |
Hasegawa, Tadashi Browse this author |
Hatanaka, Yutaka Browse this author |
Hatanaka, Kanako C. Browse this author |
Taguchi, Hiroshi Browse this author |
Harada, Taisuke Browse this author |
Ohira, Hiroshi Browse this author |
Ikeda, Daisuke Browse this author |
Mizugaki, Hidenori Browse this author |
Kikuchi, Eiki Browse this author |
Kikuchi, Junko Browse this author |
Sakakibara-Konishi, Jun Browse this author |
Shinagawa, Naofumi Browse this author |
Konno, Satoshi Browse this author |
Keywords: | SMARCA4-Deficient thoracic undifferentiated carcinoma |
Radiation therapy |
Right heart failure |
SMARCA4 |
Sarcomatoid tumor |
Radio-insensitive |
Issue Date: | 2021 |
Publisher: | Elsevier |
Journal Title: | Respiratory Medicine Case Reports |
Volume: | 32 |
Start Page: | 101364 |
Publisher DOI: | 10.1016/j.rmcr.2021.101364 |
Abstract: | SMARCA4-deficient thoracic sarcomatoid tumors were characterized by inactivating mutations of SMARCA4 and often found in the chest of young and middle-aged males with a smoking history. Recently, SMARCA4-deficient thoracic sarcomatoid tumors were reported to represent primarily smoking-associated undifferentiated/dedifferentiated carcinomas rather than primary thoracic sarcomas. The main complication of this tumor is compression of the respiratory tract and/or blood vessels. A 39-year-old man presented with a 2-month history of fever and dyspnea. Computed tomography revealed a mediastinal tumor invading the right and left pulmonary arteries. Because of severe right heart failure, we considered him ineligible for bronchoscopy. We scheduled palliative irradiation with 40 Gy/20 Fr to improve hemodynamics and perform endobronchial ultrasound transbronchial needle aspiration later. However, irradiation was ineffective, and his general condition deteriorated quickly and he died after a 7-week hospitalization. An autopsy revealed that the diagnosis was SMARCA4-deficient thoracic undifferentiated carcinoma. It has been reported that this tumor is insensitive to radiotherapy and there were some cases which responded to an immune checkpoint inhibitor. Therefore, when caring for patients with mediastinal tumors that invade and compress the trachea and large vessels, it is important to consider this tumor as a differential diagnosis and try to make a pathological diagnosis as soon as possible. |
Rights: | http://creativecommons.org/licenses/by-nc-nd/4.0/ |
Type: | article |
URI: | http://hdl.handle.net/2115/81160 |
Appears in Collections: | 北海道大学病院 (Hokkaido University Hospital) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)
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