Intimal sarcoma of the pulmonary artery with unusual findings: a case report

Abstract

Sirs:Intima sarcoma is a rare tumor often localized in the pulmonary artery. Previous descriptions can be found dating all the way back to 1923 [9].This tumor's evolvement is characterized by endoluminal growth with later vessel obstruction. This type of tumor can also originate from the heart valves and the right ventricular outflow tract. Peripheral thromboembolism is a common concomitant finding, often leading to the false diagnosis of chronic thromboembolism.The prognosis of inoperable patients with extensive disease is very poor with a median survival of 6 weeks [7]. Due to the rareness of this tumor, prospective data regarding the benefit of neoadjuvant or adjuvant chemo-or radiotherapy are not available.We present the case of a 76-year-old female with persisting shortness of breath over 6 months. The patient has a smoking history of more than 30-pack years. On physical examination, the patient exhibited tachypnea, with 21 breaths per minute, a regular pulse at 92 beats per minute and a blood pressure of 123/80 mmHg. Cardiac auscultation revealed a 3/6 systolic murmur over the second intercostal space at the left sternal border. Further physical examination was unremarkable.Blood count and chemistry profile were within normal range, including the level of brain natriuretic peptide. DDimers were at 0.5 (normal range\0.55 mg/l); CRP was at 1.1 (normal range \0.5 mg/dl).The transthoracic echocardiography showed a hypertrophic free wall of the right ventricle with a mild dilation of the right atrium (area was assessed as 2.2 cm 2 ). The color Doppler at the level of the tricuspidal annulus revealed a moderate tricuspidal regurgitation and the systolic pulmonary arterial pressure was assessed with continuous wave Doppler at 78 mmHg plus central venous pressure. The left ventricular ejection fraction was 70% and the transesophageal echocardiography showed an uncommon structure on the wall of the right pulmonary artery (Fig. 1). All echocardiographic measurements of the right ventricle were made in line with similar studies for evaluation of pulmonary artery hypertension [2].CT diagnostics showed no sign of peripheral pulmonary embolism; however, it revealed a mural thrombus in the right pulmonary artery stem, which seemed to arise from the pulmonary valve (Fig. 1b). Left and right heart catheterization and pulmonary angiogram followed for further diagnostics. A nearly 100% stenosis across the right pulmonary artery trunk was shown. We initially hypothesized the existence of a massive mural thrombus and a concomitant chronic thromboembolic disease (CTEPH) (Fig. 2. The right catheterization recorded a systolic pressure of 78 mmHg proximal and 36 mmHg distal to the stenosis. The same systolic parameter was recorded from the hemodynamic measurements in the left pulmonary artery. The wedge pressure was 3 mmHg.IVUS was performed and confirmed an occluding mass (Fig. 3). The hemodynamic results revealed an isolated

Multimodal Approach of Pulmonary Artery Intimal Sarcoma: A Single-Institution Experience

“…Several authors have reported that FDG-PET has been useful in diagnostic workup for differentiating among or staging malignant diseases and monitoring the response to treatment [ 17 , 18 ]. Among unusual findings, other authors reported case reports of patients with PAS with a lack of uptake of FDG in PET [ 19 , 20 ]. Therefore, the role of this procedure in the diagnostic workup of PAS remains controversial and requires further investigation.…”

Pulmonary artery intimal sarcoma: poor 18F-fluorodeoxyglucose uptake in positron emission computed tomography

“…al. [6] reported a pulmonary intimal sarcoma with a lack of uptake of FDG in PET. It also revealed highly malignant cells, but its cellularity was low with marked interstitial myxoid tissue.…”

An intimal sarcoma of pulmonary artery mimicking pulmonary embolism: a case report and literature review

“…The use of radiation therapy and chemotherapy after surgical treatment has been found to prolong survival time compared with surgery alone . Relative studies have shown that doxorubicin, and ifosfamide may be effective . Clinical trials to better understand the role of surgery, chemotherapy, and radiation therapy are difficult to carry out due to the rarity of the disease.…”

“…Surgical resection has been reported to decrease clinical symptoms and lengthen survival . The prognosis seems to depend on the location and vascular extension . Death is usually due to right heart failure because of obstruction of the pulmonary arteries .…”