Published online Mar 6, 2020. doi: 10.12998/wjcc.v8.i5.986
Peer-review started: December 10, 2019
First decision: December 23, 2019
Revised: January 16, 2020
Accepted: January 24, 2020
Article in press: January 24, 2020
Published online: March 6, 2020
Processing time: 86 Days and 23.2 Hours
Primary intimal sarcoma of the pulmonary artery is a rare malignant tumor originating from the pulmonary artery, which has a low incidence rate and is easily misdiagnosed as pulmonary embolism. There is no standard protocol for the treatment of primary intimal sarcoma of the pulmonary artery.
This study reports a patient with primary intimal sarcoma of the pulmonary artery who was admitted to our hospital in 2017. The clinical characteristics, diagnosis, treatment and outcome of the patient were retrospectively analyzed. The patient was a Chinese Han male aged 44 years. He had three consecutive episodes of syncope, and was thus admitted to a local hospital. Computed tomography pulmonary angiography showed multiple lesions with abnormal densities in the pulmonary trunk, left pulmonary artery, mediastinum and pericardium, which were consistent with recurrence after tumor resection. He underwent surgery, and was pathologically diagnosed with intimal sarcoma of the pulmonary artery. He relapsed 3 mo after surgery, and apatinib was administered. His condition was stable after 4 mo, with tolerable and controllable adverse reactions. He subsequently died 19 mo after surgery.
Primary intimal sarcoma of the pulmonary artery has no specific clinical or imaging manifestations. The diagnosis of this disease depends on histopathology and immunohistochemistry, and has a poor clinical prognosis. Surgical treatment is currently a favorable option for primary intimal sarcoma of the pulmonary artery, and targeted therapy may provide new insights for the development of effective treatment methods.
Core tip: Primary intimal sarcoma of the pulmonary artery is a rare malignant tumor. Herein, we present the case of a 44-year-old man who showed multiple lesions with abnormal densities in the pulmonary trunk, left pulmonary artery, mediastinum and pericardium on computed tomography pulmonary angiography, which were consistent with disease recurrence. Three months after surgery, disease relapse was reported, and the patient was given apatinib. The patient’s condition was stable after 4 mo, with tolerable and controllable adverse reactions. The patient subsequently died 19 mo after surgery.