Aggressive multiple surgical interventions to pulmonary artery sarcoma

Abstract

We describe our experience with a patient who had metastasized pulmonary artery sarcoma, but survived 7 years after diagnosis. A 61-year-old man was diagnosed with pulmonary artery intimal sarcoma after resection of metastatic tumours to the bilateral lungs. The primary lesion in the pulmonary artery trunk extending into the bilateral branches was treated by tumour endoarterectomy followed by chemotherapy. He underwent resections of lung metastases two more times before detection of recurrent obstructive pulmonary artery sarcoma 4 years after the tumour endoarterectomy. En bloc resection of the tumour including the pulmonary artery trunk, valve and interventricular septum was performed, and the right ventricular out flow tract was reconstructed with a stentless pulmonary valve and equine pericardium. He died of the disease soon after an operation for metastatic brain tumour 3 years later. Pulmonary artery sarcoma has a dismal prognosis, but aggressively repeated surgical interventions may lengthen survival.

Surgical treatment of pulmonary artery sarcoma: a report of 17 cases

“…Other than chemo-and radiotherapy, a number of reports have found that repeated surgical interventions could also be associated with prolonged survival in patients with recurrence or metastatic PAS. 23,24 In our study, two patients noted to have recurrent disease without metastasis, especially one (patient 11), the oldest example for reoperation as we know, underwent secondary operation successfully at the age of 81.…”

Primary Intimal Sarcoma of the Pulmonary Artery in a 25-Year-Old Woman with Dyspnea and Palpitation: A Case Report

“…A review of the literature showed 23 cases, of which 15 cases (65%) developed lung metastasis. Other metastasized sites were the pleura, brain, bone, liver, and others with the percentages of 9, 17, 17, 9, and 17%, respectively [2, 6, 13, 14]. Such information, especially concerning the brain, might be useful for careful follow-up of patients with IS.…”

An intimal sarcoma of pulmonary artery mimicking pulmonary embolism: a case report and literature review

“…Intimal sarcoma is a totipotent‐intimal‐cell originated tumour. Pulmonary artery intimal sarcoma arises from the intimal layer of the right, left, or main pulmonary arteries, where pulmonary metastases are common . In extremely rare cases, it may extend in a retrograde manner to the pulmonary valve and right ventricle .…”

“…The impact of chemotherapy and radiotherapy remains unclear, although some studies have demonstrated their effectiveness . The use of radiation therapy and chemotherapy after surgical treatment has been found to prolong survival time compared with surgery alone .…”