Pulmonary intimal sarcoma involving the pulmonary valve and right ventricular outflow tract: A case report and literature review.

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.