Advertisement

Unexpected Diagnosis of Pseudoaneurysmal Pulmonary Artery Sarcoma in a Patient with Acute Chest Pain

      A 64-year-old man without medical history of cardiovascular disease was admitted to our emergency department with acute chest pain. The initial chest radiograph showed a prominent enlargement of the pulmonary trunk. Troponin I and d-dimer levels were within normal limits. Electrocardiograph showed atrial fibrillation. Echocardiography revealed a moderately reduced left ventricular function and aneurysmatic protrusion of the pulmonary trunk. Subsequent electrocardiograph-gated dual-source computed tomography demonstrated an irregular shaped aneurysm with a free floating soft-tissue attenuating mass within the vascular lumen (FIGURE 1, FIGURE 2). The suspected diagnosis was an aneurysm of the pulmonary trunk with questionable perforation, and the patient was referred for urgent cardiac surgery. Intraoperative findings revealed a tumor of the pulmonary trunk with a free floating tumor mass. The patient underwent a valve conserving surgical resection with an allograft replacement of the pulmonary trunk. Histopathology finally showed a poorly differentiated intimal leiomyosarcoma of the pulmonary artery (pT2b, pN0, pMx, R1).
      Figure thumbnail gr1
      FIGURE 1Electrocardiograph-gated dual-source computed tomography scan showing formation of a pseudoaneurysm of the pulmonary trunk floating hypodense masses representing sarcoma.
      Figure thumbnail gr2
      FIGURE 23D volume-rendering of the dual source CT showing the pseudoaneurysmatic pulmonary artery sarcoma of the pulmonary trunk.
      Primary pulmonary artery sarcoma is a rare vascular malignancy. Even after radical surgery resection, the prognosis is poor because of the tendency of embolic metastasis.
      • Anderson MB
      • Kriett JM
      • Kapelanski DP
      • Tarazi R
      • Jamieson SW
      Primary pulmonary artery sarcoma: a report of six cases.
      • Kruger I
      • Borowski A
      • Horst M
      • de Vivie ER
      • Theissen P
      • Gross-Fengels W
      Symptoms, diagnosis, and therapy of primary sarcomas of the pulmonary artery.
      The typical clinical presentation and radiologic findings often mimic trombembolic disease. An aneurysmatic appearance of pulmonary artery sarcoma is extremely rare.
      • Terra RM
      • Fernandez A
      • Bammann RH
      • Junqueira JJ
      • Capelozzi VL
      Pulmonary artery sarcoma mimicking a pulmonary artery aneurysm.
      Six months after radical surgery, our patient is still alive and well without evidence of metastasis.

      REFERENCES

        • Anderson MB
        • Kriett JM
        • Kapelanski DP
        • Tarazi R
        • Jamieson SW
        Primary pulmonary artery sarcoma: a report of six cases.
        Ann Thorac Surg. 1995; 59: 1487-1490
        • Kruger I
        • Borowski A
        • Horst M
        • de Vivie ER
        • Theissen P
        • Gross-Fengels W
        Symptoms, diagnosis, and therapy of primary sarcomas of the pulmonary artery.
        Thorac Cardiovasc Surg. 1990; 38: 91-95
        • Terra RM
        • Fernandez A
        • Bammann RH
        • Junqueira JJ
        • Capelozzi VL
        Pulmonary artery sarcoma mimicking a pulmonary artery aneurysm.
        Ann Thorac Surg. 2008; 86: 1354-1355