Recurrence of an undifferentiated pleomorphic pulmonary artery sarcoma 8 years after initial presentation: a case report.

Background: Primary cardiac tumors remain exceptionally rare, characterized by a poor prognosis. Among them, sarcomas originating in the pulmonary arteries constitute the most infrequent subgroup within primary cardiac sarcomas. Case summary: This report presents the case of a 76-year-old female experiencing a recurrence of an undifferentiated pleomorphic intracardiac pulmonary artery sarcoma located in the right ventricular outflow tract, manifesting 8 years after initial remission. Successful outcomes were attained through a combination of surgical resection, state-of-the-art radiotherapy, and chemotherapy. This comprehensive approach proved essential for optimizing both survival and quality of life. Discussion: The unexpectedly prolonged recurrence-free survival observed in this case underscores the effectiveness of the comprehensive multimodal treatment approach outlined in the existing literature. This highlights the pivotal role of a multidisciplinary strategy in addressing primary cardiac sarcomas, particularly those arising in the pulmonary arteries.

The diagnostic value of [18F]FAPI-42 PET/CT for pulmonary artery masses: comparison with [18F]FDG PET/CT.

OBJECTIVES: To investigate the potential utility of [18F]fibroblast activation protein inhibitor (FAPI) positron emission tomography/computed tomography (PET/CT) for evaluating pulmonary artery (PA) masses, and compare it with [18F]fluorodeoxyglucose (FDG) PET/CT. METHODS: Participants with clinically suspected PA malignancy were prospectively enrolled and underwent dual-tracer PET/CT ([18F]FAPI-42 and [18F]FDG) imaging. Visual analysis and semi-quantitative parameters were compared between the two types of radiotracers. The tissue specimen underwent immunohistochemical staining to verify FAP expression in the tissue. RESULTS: Thirty-three patients (18 males/15 females; mean age 53.1 ± 15.4 years) were enrolled. All 21 patients with malignant PA masses were FDG-positive (100%), whereas 20 out of 21 patients were FAPI-positive (95.2%). All 12 patients with benign PA masses were both negative in FDG and FAPI PET. The mean maximum standardized uptake value (SUVmax) and target-to-background ratio (TBR) of FAPI and FDG in malignant PA masses were significantly higher than those of benign masses. Although there was no significant difference in SUVmax between FDG and FAPI in malignant PA masses (11.36 vs. 9.18, p = 0.175), the TBR (liver) and TBR (left ventricle) were more favorable for FAPI than for FDG (13.04 vs. 5.17, p < 0.001); (median: 7.75 vs. 2.75, p = 0.007). Immunohistochemical analysis (n = 16) validated that the level of FAP expression corresponded strongly to the uptake of FAPI in PET/CT scans (rs = 0.712, p = 0.002). For clinical management, FAPI PET found more metastatic lesions than FDG PET in 4 patients, with 2 patients upgrading and 1 patient changing treatment decisions. CONCLUSIONS: FAPI PET/CT is feasible in the diagnosis of PA masses. Although not superior to FDG PET/CT, FAPI PET/CT showed better target-to-background contrast. CLINICAL RELEVANCE STATEMENT: This study found that FAPI PET/CT is not superior to FDG PET/CT in diagnosing PA masses, but FAPI PET/CT displays better target-to-background contrast and more positive lesions, which may help improve disease management. KEY POINTS: Pulmonary malignancies lack specificity in clinical manifestations, laboratory tests, and routine imaging examinations. FAPI PET/CT is not diagnostically better than FDG PET/CT but displays better target-to-background contrast and more positive lesions. Dual-tracer PET/CT ([18F]FAPI-42 and [18F]FDG) imaging improves clinical management of pulmonary artery masses.

Pulmonary artery sarcoma affecting the pulmonary valve mistaken as pulmonary vasculitis: a case report and comparative literature review.

Pulmonary arterial sarcomas (PAS) are rare aggressive tumours occurring mainly in the pulmonary trunk. We report a case of PAS involving the pulmonary trunk wall and valve, with uniform wall thickening which represents an atypical imaging manifestation of this tumour. A 63-year-old male presented with vague respiratory symptoms with rapid progression. CTPA showed low density filling defects in both pulmonary arteries and PET scan showed increased uptake in the pulmonary trunk, which along with raised ESR suggested Pulmonary Vasculitis. Echo imaging showed Right ventricular hypertrophy and pulmonary stenosis. Response to steroid therapy was minimal and his symptoms worsened. A referral for second opinion was made and he was diagnosed with PAS. He underwent Pulmonary thromboendarterectomy with Pulmonary valve replacement. Post-operative histopathology confirmed the diagnosis. PAS is rare and frequently misdiagnosed. Surgical resection is not curative, but together with chemotherapy can prolong survival.

Comparison of pulmonary artery sarcoma and pulmonary thromboembolism according to clinical and computed tomography pulmonary angiography and magnetic resonance imaging characteristics: a single-center retrospective study.

Background: Pulmonary artery sarcoma (PAS) is a very rare malignancy with a poor prognosis; however, its clinical manifestations and imaging findings are often indistinguishable from pulmonary thromboembolism (PTE). We thus aimed to accurately diagnose PAS by comparing the clinical and computed tomography pulmonary angiography (CTPA) and magnetic resonance imaging (MRI) imaging characteristics of PAS and PTE. Methods: This case-control study retrospectively enrolled 20 patients with PAS (from March 2017 to September 2022), 40 patients with central acute PTE, and 40 patients with central chronic PTE (from January 2021 to December 2022) in the China-Japan Friendship Hospital. The following clinical and imaging findings were compared between the three groups: initial symptoms; D-dimer, C-reactive protein, and N-terminal pro B-type natriuretic peptide levels; wall-eclipsing sign (WES); scope of lesion involvement; and morphological characteristics. Signal intensity was also observed on different MRI sequences. Results: The D-dimer level in PAS was significantly lower than that in central acute PTE (P<0.001). The WES was present in 17 cases of PAS (85.0%), which was a greater proportion than that of the central acute PTE and chronic PTE groups (all P values <0.001). The involvement of the pulmonary valve or right ventricular outflow tract was observed in five PAS cases but none of the central acute PTE or chronic PTE cases (all P values =0.001). In 19 PAS cases (95.0%), the lesions grew expansively in the central pulmonary artery. The proximal margin of 18 patients with PAS (90.0%) was bulging or lobulated. Nine cases of PAS (45.0%) showed aneurysm-like dilatation (grape-like sign) of the distal pulmonary artery, representing significantly greater proportion than that of the central acute PTE and chronic PTE groups (all P values <0.001). In 37 patients with central acute PTE (92.5%), the clots were observed to be floating in the pulmonary artery lumen with saddle, tubular or polypoid shape. Eccentric filling defects attached to the pulmonary artery wall were observed in 32 cases of central chronic PTE (80.0%). On MRI, PAS lesions were hyperintense on fat-suppressed T2-weighted imaging and diffusion-weighted imaging, demonstrating heterogeneous enhancement. Conclusions: Comprehensive analysis of the clinical data and imaging features on CTPA and MRI can aid in the accurate differential diagnosis of PAS and PTE.

Pulmonary artery sarcoma and severe valvular diseases in late-septuagenarian women: was 2-stage surgery an appropriate strategy? A case report.

BACKGROUND: Pulmonary artery sarcomas (PASs) are rare, and complete tumor resection is often difficult at the time of detection. We encountered a case of PAS that was thought to be resectable; however, the patient had severe symptomatic valvular disease. We faced a difficult decision regarding the surgical strategy. CASE PRESENTATION: A 76-year-old female presented with a history of polysurgery for multiple primary cancers. She was referred to our department with a calcified mass in the right pulmonary artery (PA) and severe symptomatic valvular disease. After a discussion with the cardiovascular surgeon, we decided to perform a two-stage surgery. She underwent valvuloplasty through a median sternotomy, resulting in an improvement in her exertional dyspnea. The tumor was removed three months later with a right upper lobectomy and PA patch reconstruction through a posterolateral thoracotomy. When the PA was opened, the edge of the tumor was entrapped by vascular clamp forceps because of insufficient dissection of the adhesions between the superior vena cava and the right main PA resulting from the first operation. The patient underwent proton therapy twice for chest wall metastases which recurred three months after surgery, and local recurrence in the PA was diagnosed five months after surgery. The patient was alive with stable disease 25 months after surgery. CONCLUSION: Two-stage surgery for PAS and valvular disease resulted in incomplete resection of the PAS in the right PA. It is important not to underestimate surgical adhesions due to the initial surgery and to consider and implement measures to prevent adhesions of critical vessels during the second operation.

A primary pulmonary artery sarcoma masquerading pulmonary embolism: a case report and literature review.

BACKGROUND: Primary pulmonary artery sarcoma (PAS) is an extremely rare malignant tumor with a poor prognosis. The clinical manifestations of PAS are diverse, including dyspnea, chest pain, cough, and hemoptysis. The poor prognosis is often due to delayed diagnosis caused by similarity in imaging findings with pulmonary thromboembolism (PTE). These cues of diagnosis include the "wall eclipsing sign", lobulated bulging margins, gadolinium enhancement during MRI imaging, and FDG uptake during PET/CT imaging. However, there are still many misdiagnoses. CASE PRESENTATION: This article reports a woman of reproductive age presenting with a pulmonary artery mass. The computed tomographic pulmonary angiography and positron emission tomography/computed tomography did not show obvious signs of pulmonary artery sarcoma, however, contrast-enhanced echocardiography showed moderate perfusion, which helped differentiate between pulmonary artery sarcoma and pulmonary artery thrombosis, leading to timely surgical treatment. CONCLUSIONS: PAS is a rare form of cancer that can occasionally be visually similar to PTE on radiographic images. Early diagnosis of PAS is of vital importance to the prognosis of the patients. There are several visual cues that can help differentiate between the two conditions. Additionally, contrast-enhanced echocardiography provides additional information on tumor perfusion, offering another effective approach for a prompt and accurate diagnosis.

Immune microenvironment of intimal sarcomas: Adaptive immune resistance with potential therapeutic implications.

OBJECTIVES: Intimal sarcomas are rare, aggressive neoplasms that arise from large blood vessels. Characterization of the tumor immune microenvironment may suggest new treatment strategies. METHODS: Seventeen specimens from 7 patients were labeled by immunohistochemistry for programmed cell death 1 ligand 1 (PD-L1), CD45, CD8, CD4, FOXP3, CD20, CD68, and LAG3. The immune cell density was scored as a percentage of the tumor area (1+ [<5%], 2+ [5%-50%], 3+ [>50%]); PD-L1 expression was scored on tumor cells and on intratumoral immune cells. Immune marker density was quantified using image analysis software. RESULTS: All intimal sarcomas showed immune cell infiltration (41% were 1+, 53% were 2+, 6% were 3+). Tumor and immune cell PD-L1 labeling was seen in 35% and 76% of cases, respectively; PD-L1+ intimal sarcomas had higher CD45+, CD8+, FOXP3+, CD68+, and leukocyte activation gene 3 (LAG3)+ cell densities (P ≤ .01). Similarly, PD-L1 expression on immune cells correlated with higher densities of CD8+ and FOXP3+ cells (P < .04). Higher LAG3+ cell density correlated with higher CD68+ cell density and necrosis (P < .05). One patient with prolonged survival had the highest immune cell density and PD-L1 expression. CONCLUSIONS: These data show that intimal sarcomas have an active tumor microenvironment with an adaptive pattern of PD-L1 expression. Our results suggest that immunotherapy may be an effective treatment option.

Results of surgical treatment of pulmonary artery sarcomas: Does histology affect survival?

Background: In this study, we aimed to describe our experience with primary pulmonary artery sarcoma in patients who underwent pulmonary endarterectomy and to evaluate clinical features, treatment, outcomes, and survival rates according to the histological subtypes of this malignant disease. Methods: Between March 2011 and May 2022, a total of 13 patients (7 males, 6 females; mean age: 52.6±13.0 years; range, 30 to 69 years) who underwent pulmonary endarterectomy and diagnosed with a pulmonary artery sarcoma were retrospectively analyzed. The diagnosis was confirmed histopathologically in all patients. Data including demographics, clinical characteristics, intra- and postoperative complications, length of hospital stay, morbidity, mortality, and short-term and long-term outcomes were recorded. Operative mortality was defined as death in the hospital or within 30 days of surgery. Results: Mortality was observed in one patient due to massive hemoptysis. Morbidity developed in two patients due to acute respiratory distress. Pulmonary vascular resistance improved significantly from 508 dyn/s/cm-5 to 191 dyn/s/cm-5 (p<0.004). All patients received chemotherapy following surgery. Median followup was 14 months. Median survival for the entire series was 18 months. One-year and three-year survival rates were 60.6% and 30.3%, respectively. Median survival for leiomyosarcomas (n=6) was seven months, while it was 44 months for intimal sarcomas (p=0.004). Three-year survival was 66.7% for intimal sarcomas and 0% for leiomyosarcomas. Conclusion: Pulmonary artery sarcoma may mimic chronic thromboembolic pulmonary hypertension. Patients with a suspected diagnosis of pulmonary artery sarcoma should be referred to expert pulmonary endarterectomy centers for surgery where a multidisciplinary team is available. Pulmonary endarterectomy has both diagnostic and therapeutic value and may improve survival and quality of life. Patients with intimal sarcoma have longer survival compared to those with leiomyosarcoma.

Primary pulmonary artery chondrosarcoma: case report.

Background: Primary pulmonary artery sarcoma (PAS) is a rare tumor that presents like pulmonary embolism (PE), primary chondrosarcoma in the pulmonary artery is even rarer and few studies have been reported. PAS are commonly misinterpreted as in a clinical setting, many patients initially receive anticoagulant and thrombolysis therapy, but failed to respond. Management of this condition is difficult and prognosis is poor. We report a case of primary pulmonary artery chondrosarcoma that was initially misdiagnosed as PE and Inappropriate interventional therapy was performed, but with poor response. Finally, patient received surgical treatment, postoperative pathology confirmed primary pulmonary artery chondrosarcoma. Case Description: A 67-year-old woman who had presented with cough, chest pain and shortness of breath for more than 3 months. Computed tomography pulmonary angiography (CTPA) showed filling defects were seen in the right and left pulmonary arteries, spreading to the outer lumen. The patient was initially diagnosed with PE and underwent transcatheter aspiration for pulmonary artery thrombus, transcatheter thrombolysis, and inferior vena cava filter placement at a local hospital, but with poor response. She was then referred for pulmonary artery tumor resection, endarterectomy and pulmonary arterioplasty. Histopathological examinations confirmed a diagnosis of primary PAS (chondrosarcoma). The patient developed in situ recurrence of pulmonary artery tumors in 10 months after surgery and received six cycles of adjuvant chemotherapy. The lesions progressed slowly after chemotherapy. The patient subsequently developed lung metastasis in 22 months and died of heart failure and respiratory failure 2 years after surgery. Conclusions: PAS is an extremely rare and the clinical symptoms and radiological features often mimics PE, therefore When doctors make differential diagnosis of pulmonary artery mass lesions, especially when the anticoagulation and thrombolytic effects are very poor. They need to be alert to the possibility of PAS so that early diagnosis and early treatment can prolong the survival of patients.

Pulmonary artery sarcoma: an unexpected settler in the right ventricular outflow tract.

Pulmonary artery sarcoma (PAS) is a sporadic malignant tumor that mainly originates from the pulmonary arteries. However, PAS may also involve the right ventricular outflow tract (RVOT) and lead to obstruction, syncope, or sudden death. Early diagnosis and complete surgical resection are essential to prolong survival and improve the quality of life of patients with PAS. Herein, we report a case of a young female patient admitted for pulmonary malignancy and acute pulmonary embolism. The patient had a mass in the RVOT, which was detected by transthoracic echocardiography. Computed tomography and magnetic resonance imaging revealed the invasion depth and extent of the lesions. Surgical resection improved hemodynamics, while pathological and immunohistochemical tests confirmed the diagnosis of a pulmonary artery sarcoma. Local recurrence was detected in the adjacent tissues about two months after the surgery. Given the potential risk of reoperation, the patient was suggested to undergo conservative treatment.

A case report of pulmonary artery intimal sarcoma negative for 18F-FDG mimicking pulmonary thromboembolism.

Background: Pulmonary artery sarcoma is a rare malignant neoplasm arising from intimal mesenchymal cells in the pulmonary artery wall and is often difficult to differentiate from pulmonary embolism, however, 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) can be useful for a differential diagnosis. Here, we present a rare case of pulmonary sarcoma undetectable by PET. Case summary: A 77-year-old woman who had worsening dyspnoea on effort for a month and progressive chest discomfort with nocturnal cough for a week presented to our hospital. Contrast-enhanced computed tomography (CT) demonstrated a massive filling defect in the left pulmonary artery (PA). Two major differential diagnoses were considered; pulmonary thromboembolism and tumour-like lesions. Positron emission tomography-computed tomography (PET-CT) revealed that there was no abnormal accumulation of 18F-FDG in the mass. However, even after effective anti-thrombotic treatment for 3 weeks, a follow-up CT showed no reduction at all in the size of the lesion in the pulmonary artery. Therefore, surgery for diagnostic therapeutic purposes was performed. Discussion: The present case is informative because it supports the idea that being aware of PA angiosarcoma as a potential differential diagnosis of pulmonary thromboembolism is essential, particularly in cases of no evident peripheral venous thrombosis and a negative D-dimer test, even if neither heterogenous contrast enhancement in CT and magnetic resonance imaging nor accumulation of 18-FDG in PET-CT is evident.

Pulmonary artery sarcoma misdiagnosed as a pulmonary embolism: A case report.

Pulmonary artery sarcoma is a rare type of tumor and is easily misdiagnosed. We report a case of pulmonary artery sarcoma in a 26-year-old young man who presented with acute onset of dyspnea. Computed tomographic angiography of the chest had revealed a large filling defect within the main pulmonary artery (PA) that extended into both right and left PAs. Because pulmonary embolism was suspected, both anticoagulant and thrombolytic therapies were initiated. The patient responded poorly to these therapies, leading to the resection of the mass. After an uneventful postoperative course, the patient was discharged from the hospital on postoperative day ten. He was subsequently treated with chemotherapy and continued to show no evidence of disease. Multimodal therapy can provide prolonged survival.

Multimodal treatment and imaging of primary pulmonary artery sarcoma: a case report.

Primary pulmonary artery sarcoma (PAS) is a malignant neoplasm of the pulmonary vascular system originating from the intima, with clinical and imaging manifestations similar to those of pulmonary thromboembolism (PTE), and is often misdiagnosed. Spectral CT and PET/CT can clearly show the extent and metastasis of PAS and are valuable in visualizing adjacent structures such as lungs, bronchi, and mediastinum, providing a strong imaging basis for the diagnosis and differentiation of PAS, allowing treatment monitoring and follow-up.

Complete resection of a pulmonary artery sarcoma involving the pulmonary valve and right ventricle outflow tract: a case report.

Background: Pulmonary artery sarcoma (PAS) is an extremely rare tumour, preferably treated by surgery. However, the surgical management remains largely debatable, as only less than half of patients with PAS can undergo thorough excision. Case summary: A 32-year-old man with a tumour involving the right ventricle outflow tract, pulmonary trunk extending into the bifurcation, and right pulmonary arteries underwent complete resection using a homologous pulmonary valve and vascular grafts for reconstruction, combined with right pulmonary endarterectomy (PEA) for potential seeding metastasis. Histopathological examination demonstrated undifferentiated pleomorphic sarcoma with surgical margins free of disease. The patient remains asymptomatic, and follow-up computed tomography 5 months after surgery indicated no recurrence or metastasis. Discussion: Radical resection of a PAS with reconstruction using pulmonary valve allograft and polytetrafluoroethylene vascular grafts is technically feasible and successful. Additionally, PEA may eliminate the potential intima implantation metastasis.

A Rare Case of Multicentric Primary Pulmonary Artery Sarcoma: Eliminating the Masquerade with Multimodality Imaging.

A 68-year-old male presented with a short history of exertional dyspnea and a provisional diagnosis of pulmonary thromboembolism was made. However, chest radiograph and further investigations in the form of computed tomography pulmonary angiogram, magnetic resonance imaging of thorax, and whole body fluorodeoxyglucose (FDG) positron emission tomography-computed tomography revealed a large mass arising from the distal left pulmonary artery extending into adjacent lung and another lesion near the root of the main pulmonary artery, both of which showed post-contrast enhancement and intense FDG uptake. Tissue sampling by transthoracic computed tomography-guided biopsy and immunohistochemistry confirmed the diagnosis of pulmonary artery angiosarcoma. Here, we present such a case of very rare occurrence which, in view of multicentricity and substantial extension into adjacent lung, is the first of its kind to be reported, to the best of our knowledge.

Primary pulmonary artery sarcoma complicated with pulmonary embolism and pulmonary tuberculosis: A case report and literature review. / åŽŸå‘æ€§è‚ºåŠ¨è„‰è‚‰ç˜¤å¹¶è‚ºæ “å¡žã€è‚ºç»“æ ¸æ‚£è€ 1ä¾‹æŠ¥é“å¹¶æ–‡çŒ®å¤ä¹ .

A case of primary pulmonary artery sarcoma (PPAS) complicated with pulmonary embolism and pulmonary tuberculosis is reported. This patient, a 48-year old woman, was diagnosed as pulmonary tuberculosis at the initial stage of the disease, whose condition was improved after anti-tuberculosis treatment, and was finally diagnosed as PPAS combined with pulmonary embolism due to recurrence of the symptoms. PPAS is a very rare malignant tumor originating from the inner or subintima of the pulmonary artery. The clinical manifestations of PPAS have no obvious specificity which can be dyspnea, chest pain, cough, hemoptysis, and so on. Enhanced CT, enhanced MRI, and positron emission computed tomography (PET/CT) is beneficial to the differential diagnosis of PPAS, but definitive diagnosis needs intravascular biopsy or surgical biopsy. Because PPAS often presents as pulmonary embolism, patients with PPAS were often misdiagnosed as pulmonary embolism, resulting in delayed diagnosis and treatment. There are few reports of PPAS presenting as pulmonary embolism complicated with pulmonary tuberculosis at home and abroad, which is also easy to be misdiagnosed. The disease has a high degree of malignancy, which is short of effective treatment at the late stage, with short survival time and poor prognosis. Therefore, attention to the various clinical manifestations of PPAS and early diagnosis and treatment are crucial to the prognosis of PPAS patients.

Clinical utility of 18F-FDG PET/CT imaging in patients with pulmonary artery sarcoma.

BACKGROUND: Pulmonary artery sarcoma (PAS) is a rare and fatal malignancy. Due to the lack of specific clinical and radiological features, PAS is always misdiagnosed as pulmonary thromboembolism (PTE). This study aimed to investigate 18F-FDG PET/CT in distinguishing PAS from PTE, and analyze its correlation with clinical and radiological findings and outcome of PAS. METHODS: Clinical, contrast-enhanced CT, and 18F-FDG PET/CT characteristics of 14 patients with PAS and 33 patients with PTE were retrospectively reviewed. The correlation between PET/CT metabolic parameters vs. clinical and CT findings was investigated in patients with PAS. The overall survival (OS) was analyzed in PAS patients. RESULTS: The SUVmax of PAS (median: 8.0, range 3.0-17.2) was significantly higher than PTE (1.8[0.8-3.7]) (P < 0.001), and at a cutoff value of 2.9, the sensitivity and specificity were 100.0% and 93.9%, respectively. Compared with PTE, PAS more frequently occurred in younger population (P = 0.011), involved pulmonary trunk (P < 0.001), and displayed higher enhanced CT (P < 0.001) and ΔCT (enhanced CT compared to non-enhanced CT) (P < 0.001) values. SUVmax of PAS was associated with tumor staging (P = 0.022) and enhanced CT (P = 0.013) and ΔCT (P = 0.005) values. The median OS of PAS patients was 10.5 months, and 12-month and 24-month OS rates were 58.0% and 12.0%, respectively. Only D-dimer level (P = 0.038) and tumor staging (P = 0.019) were associated with OS. CONCLUSIONS: Most PAS displayed high glucometabolism, and SUVmax of 18F-FDG PET/CT was useful in distinguishing PAS from PTE.

The Diagnosis and Treatment of Primary Pulmonary Artery Sarcoma: Two Case Reports.

Pulmonary artery sarcoma (PAS) is considered a very rare tumor with a poor prognosis. We herein report two cases of PAS that were diagnosed by positron emission tomography (PET)/computed tomography (CT). In both cases, PET was an effective option for diagnosing tumors, and surgical resection was a valid treatment for these diseases. If a pulmonary artery tumor is suspected, PET/CT is useful for diagnosing PAS and very helpful for choosing the surgical treatment strategy.

Clinical Features and Outcomes of Pulmonary Artery Sarcoma.

OBJECTIVES: A retrospective cohort study was designed to describe the clinical features and outcomes of pulmonary artery sarcoma (PAS). METHODS: Twenty-two (22) consecutive patients diagnosed with PAS by pathological examination were enrolled and followed up until they died or until January 2020. The medical records were retrospectively reviewed to evaluate the clinical characteristics, image findings, and outcomes. RESULTS: 1) Twenty-one (21, 95.5%) patients were firstly misdiagnosed. Dyspnoea was the most common presenting symptom (19 of 22, 86.4%). 2) Filling defects in the right pulmonary artery were seen in 17 patients (77.3%) with computed tomography pulmonary angiography or magnetic resonance pulmonary angiography. Among those patients, 14 underwent positron emission tomography-computed tomography detection and 13 (92.9%) were found to have increased uptake value in the pulmonary artery. 3) The median survival (from diagnosis to death or January 2020) of the total series was 11.6 months (range, 0.7-68.5 months). The estimated cumulative survival rates at 1, 2, and 3 years were 52.6%, 32.8%, and 19.7%, respectively. Patients who received surgery and/or chemo-radiotherapy treatment had a better survival rate compared with patients without treatment (the estimated cumulative survival rates at 1, 2, and 3 years were 60.3%, 39.1%, and 29.3%, respectively, vs 33.3%, 16.6%, and 0, accordingly) and better survival time (median survival 17.02 vs 3.16 months, respectively) (p=0.025). CONCLUSIONS: Pulmonary artery sarcoma is easily misdiagnosed, as the symptoms and routine image detection are nonspecific. Positron emission tomography-computed tomography may be helpful in diagnosis. Surgery and/or chemo-radiotherapy offer a chance for better outcomes.

Giant Pulmonary Artery Thrombotic Material, Due to Chronic Thromboembolic Pulmonary Hypertension, Mimics Pulmonary Artery Sarcoma.

In this article, we present the case of a 38-year-old female who suffered from serious respiratory distress. After an extensive pulmonary artery imaging diagnostic work-up (CTPA, MRA and PET), we were unable to differentiate between chronic thromboembolic pulmonary hypertension (CTEPH) vs. pulmonary artery sarcoma (PAS) due to extensive filling defects and extraluminal findings. Although surgery was postponed for nine months due to the COVID-19 pandemic, CTEPH diagnosis, due to a high-thrombus burden, was finally confirmed after pulmonary endarterectomy (PEA). Conclusively, imaging findings of rare cases of CTEPH might mimic PAS and the surgical removal of the lesion are both needed for a final diagnosis. What is Already Known about This Topic? Pulmonary artery sarcoma (PAS) is a rare but aggressive malignancy, which originates from the intimal layer of the pulmonary artery (PA); Chronic thromboembolic pulmonary hypertension (CTEPH) is based on chronic, organized flow-limiting thrombi inside PA circulation and subsequent pulmonary hypertension. What Does This Study Contribute? Since radiological findings of CTEPH cases might rarely mimic PAS, pulmonary artery endarterectomy and subsequent histopathologic study are needed for a final diagnosis.