Open AccessPulmonary intimal sarcoma involving the pulmonary valve and right ventricular outflow tract
Author(s) -
Rui Xu,
Yixuan Zhao,
Xiaosen Xu,
Shuang Liu,
Chenyu Hu,
Dongmei Lv,
Huiying Wu
Publication year - 2020
Publication title -
medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.59
H-Index - 148
eISSN - 1536-5964
pISSN - 0025-7974
DOI - 10.1097/md.0000000000018813
Subject(s) - medicine , pulmonary artery , sarcoma , ventricular outflow tract , pulmonary angiography , radiology , ventricle , pulmonary valve , pulmonary hypertension , right pulmonary artery , surgery , cardiology , pathology
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.