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

Abstract

Pulmonary artery intimal sarcoma is a highly aggressive disease, and is most often misdiagnosed as pulmonary thromboembolism (PTE) due to the similar clinical symptoms and its rarity, which leads to the use of inappropriate treatments such as prolonged anticoagulant therapy. We reported a case of pulmonary artery intimal sarcoma in a patient who was misdiagnosed as having PTE. Pathology after surgery confirmed malignant disease. We concluded that when a patient presents with mild clinical manifestations yet with strong imaging manifestations, pulmonary artery malignancy should be suspected.

Intimal sarcoma of the pulmonary artery without 18F-fluorodeoxyglucose uptake

“…The impact of chemotherapy and radiotherapy in PAIS remains unclear, although some studies have demonstrated that these therapies prolong survival after surgical treatment. 3,4 Our patient underwent chemotherapy with paclitaxel. Because early and proper diagnosis is necessary for appropriate therapy, clinicians should consider the possibility of PAIS even if imaging findings are inconsistent with the disease.…”

“…PAIS is often misdiagnosed as PTE. [2][3][4][5] PAIS has a poor prognosis due to delayed diagnosis and distal spread of the disease, with survival outcomes of 36.5 AE 20.2 and 11 AE 3 months for patients having curative resection and incomplete resection, respectively. 4 CT findings differentiating PAIS from PTE include hyperdense lesions with nonhomogeneous attenuation.…”

“…The roles of chemotherapy and radiotherapy remain undefined, 1,2 although some studies have demonstrated their effectiveness. 3,4 The usual therapy is surgery with adjuvant chemotherapy. After resection, regular follow-ups include complete anamnesis, physical examination, and contrast-enhanced computed tomography (CT) for detecting early recurrence.…”

Mimickers of chronic thromboembolic pulmonary hypertension on imaging tests: a review

“…21,22 PAS appears as a delayed contrast enhancement on CT angiography scans, more evident in the venous phase. 21,24,25 The presence of multiple lung nodules can raise a suspicion for a malignancy like PAS. 19…”

“…25 Reported surgical treatments include lobectomy or pneumonectomy for unilateral cases, pulmonary endarterectomy, and tumor debulking with or without graft reconstruction of the pulmonary artery for bilateral cases. 23,25,29 The goal of pulmonary endarterectomy is to relieve the obstruction caused by the tumor, which causes significant improvement in symptoms but this is not curative. 23 The role of chemotherapy and radiation post-surgery is yet unclear.…”