Pulmonary intimal sarcoma involving the pulmonary valve and right ventricular outflow tract

INTRODUCTION: Pulmonary artery intimal sarcoma (PAIS) is a rare and highly aggressive tumor, and approximately 80% of pulmonary cases occur in the pulmonary trunk. We report herein a case of retrograde extension of the sarcoma to the pulmonary valve and right ventricle, which is an uncommon manifestation of this lethal tumor. PATIENT CONCERNS: A 41-year-old woman was initially diagnosed with pulmonary thromboembolism (PTE) and transferred to our hospital. DIAGNOSIS: Computed tomographic pulmonary angiography (CTPA) showed that there are low-density filling defects in both pulmonary arteries, and the patient was diagnosed with PTE. However, the ultrasonographers considered that the lesion is a space-occupying type that involves the right ventricular outflow tract and pulmonary valve instead of PTE. Postoperative pathology confirmed the diagnosis of PAIS. INTERVENTIONS: The patient underwent resection of pulmonary artery sarcoma and endarterectomy. OUTCOMES: During the follow-up via telephone 1 month after discharge, the patient reported to have been feeling well. CONCLUSION: Owing to the rarity of the disease and its non-specific clinical manifestations, approximately half of the PAIS cases are misdiagnosed or have a delayed diagnosis. Thus, improving our understanding of the disease and facilitating its early diagnosis are essential.