Clinical features and surgical outcomes of pulmonary artery sarcoma

Abstract

Although pulmonary artery sarcoma has a very poor prognosis, surgical treatment offers a chance for symptom relief and better long-term outcome. Aggressive postoperative adjuvant treatment may be necessary to improve survival.

Surgical treatment of primary pulmonary artery sarcoma

“…In 1923, Mandelstamm reported the first PAS via autopsy, and since then, approximately 400 cases have been described, mostly as case reports [8]. The incidence of PAS was reported as 0.001-0.03% based on autopsy [21], but this incidence might be underestimated because of the insidious onset of PAS and misdiagnoses as any cause of obstructive pulmonary hypertension, such as pulmonary embolism (PE) and CTEPH, primary lung cancer and extra thoracic tumor emboli extending into the PA [13].…”

“…We retrospectively reviewed their clinical records to evaluate the patient characteristics, perioperative findings, and postoperative outcomes. There were 14 articles, which reported at least 6 each, that identified 201 patients diagnosed with PAS, and 158 patients had detailed treatment and follow-up data [7][8][9][10][11][12][13][14][15][16][17][18][19][20].…”

“…However, DHCA could damage our normal physiological process. Yin and colleagues [8] performed distal embolectomy routinely via the aid of CPB, but DHCA was not performed in their series. In Deng's study [7], 13 patients underwent PTE; among these patients, DHCA was applied in 3 patients, deep hypothermic low flow was used in 6 patients, and the other four patients received mild or moderate CPB.…”

A primary pulmonary artery sarcoma associated with multiple lesions

“…The most effective therapeutic option for prolonging survival in patients with a PPAS diagnosis seems to be complete tumor resection. [17] In thi s case, unfortunately, PPAS determination was possible only at the most advanced stage, and so radical resection was almost impossible. Radiotherapy and chemotherapy can be applied for patients who have unresectable or recurrent PPAS to prevent distal tumor micro-embolization and prolong survival.…”

From Pulmonary Artery Repair to Right Ventricular Outflow Tract Replacement

“…3 Pulmonary artery sarcoma (PAS) first was described in 1923, and approximately 400 cases have been reported since then. 10 It often is misdiagnosed as PE or CTEPH. 4 As a result, patients receive unnecessary anticoagulant and thrombolytic administrations and delays in oncologic management.…”