Pulmonary Artery Intimal Sarcoma: A Diagnostic Challenge Using a Multimodal Approach

Abstract

Pulmonary artery intimal sarcoma (PAIS) is a rare tumor without clear syndromic presentation other than nonspecific symptoms of cough, dyspnea, and weight loss. This diagnosis is difficult due to challenging radiographic interpretations of multiple imaging modalities. We present a case of a 60-year-old male, who presented to his pulmonologist and underwent a CT chest with IV contrast that initially suggested primary lung carcinoma. CT angiogram showed significant vascular filling defects suspicious of an intravascular mass, rather than vascular invasion by lung lesions. The PET/CT scans further suggested a malignant process, but indistinguishable between an extravascular or intravascular etiology. Taking these results together, they suggested an intravascular malignancy, prompting a tissue biopsy, which ultimately led to a diagnosis of PAIS with metastases. Establishing a definitive diagnosis is essential as treatment and prognosis are different for sarcoma compared to carcinoma. There is no standard treatment to date, and management often includes a multidisciplinary approach involving surgery, radiation, chemotherapy, and targeted therapy. PAIS is a rare entity that cannot be diagnosed clinically and needs a multimodality approach for its diagnosis.

Pulmonary artery intimal sarcoma mimicking pulmonary thromboembolism

“…[ 13 , 15 ] In addition to PA space-occupying lesions in chest CT, multiple pulmonary nodules or hilar tumors are usually helpful for the diagnosis of PAIS rather than PTE, which is usually accompanied by wedge-shaped pulmonary infarction. [ 16 – 18 ] In CT pulmonary angiography, PAIS mainly occurs in the pulmonary trunk, and could extend proximally to the pulmonary valve and even right ventricular outflow tract, extend distally to the left and right pulmonary arteries and branches. The proximal end of the mass in PAIS is mostly swollen, bulging or lobulated.…”