Key result
Surgical resection followed by anlotinib fails to prevent fatal recurrence of pulmonary artery intimal sarcoma.
Why the study?
Pulmonary artery intimal sarcoma often mimics pulmonary thromboembolism, leading to frequent misdiagnosis, delayed optimal treatment, and an unfavorable prognosis.
Case Report (n=1)
No
Pulmonary artery intimal sarcoma is a rare, aggressive tumor that mimics pulmonary thromboembolism; it should be considered in patients with atypical imaging findings who do not respond to anticoagulant therapy.
Does not support anlotinib after resection in pulmonary artery intimal sarcoma; leaves open optimal systemic therapy for this rare tumor.
Pulmonary artery intimal sarcoma (PAIS) is a rare and highly aggressive form of hemangiosarcoma. The non-specific clinical manifestations, signs and routine imaging findings of this sarcoma often mimic those of pulmonary thromboembolism (PTE), resulting in frequent misdiagnosis as PTE prior to pathological confirmation in most patients. This delay in achieving an accurate diagnosis poses challenges for promptly initiating optimal treatment and further contributes to the unfavorable prognosis. The current study presents the case of a 68-year-old female who presented with acute chest tightness and dyspnea. Transthoracic echocardiography revealed the presence of atypical echogenic structures within the primary pulmonary artery, while computed tomography pulmonary angiography showed marked filling defects in the main trunk and branches of the pulmonary artery. The patient was initially misdiagnosed with PTE but did not respond well to anticoagulant therapy. Subsequent surgical resection confirmed the diagnosis of PAIS through pathological examination. Despite postoperative treatment with molecular-targeted antitumor drugs, the patient experienced tumor recurrence and intrapulmonary metastasis, ultimately succumbing to disease progression. This exceptional case is being presented to enhance the clinical understanding of PAIS, to encourage further extensive research for reliable diagnostic approaches and to provide further data that will help form efficacious therapeutic strategies to ameliorate the unfavorable prognosis of affected patients.
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Huang et al. (2025) conducted a case report in Pulmonary artery intimal sarcoma (n=1). Surgical resection and anlotinib was evaluated. Surgical resection followed by anlotinib therapy in a 68-year-old female with pulmonary artery intimal sarcoma failed to prevent tumor recurrence and metastasis, ultimately resulting in death.
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