Primary Pulmonary Artery Sarcoma Research Articles

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

BackgroundPrimary 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 presentationThis 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.ConclusionsPAS 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.

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

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. 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. 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. 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.

P374 THE BAD COUSIN OF PULMONARY EMBOLISM: PRIMARY PULMONARY ARTERY SARCOMA

Abstract A young man of 29 years presented to the Emergency Room reporting worsening dyspnea for about 2 weeks. He had never had previous cardiovascular disease nor had risk factors for them. Hemodynamics were stable, but arterial blood gases showed hypocapnic hypoxia. The ECG revealed a S1Q3T3 pattern, incomplete right bundle branch block and signs of right ventricular overload. D–dimer was 4493 ng/ml. CT pulmonary angiogram (CTPA) showed massive bilateral embolism with thrombotic material in the right ventricle (RV) and almost complete obliteration of pulmonary trunk and main pulmonary arteries; moreover, there were subpleural nodularities in upper pulmonary lobes. Echocardiogram showed a dilated and hypokinetic RV, moderate tricuspid regurgitation with a sPAP of 80 mmHg, thrombotic pulmonary trunk obliteration and floating thrombi adhered to the tricuspid valve. UFH therapy was started. After ten days, there was no improvement in clinical status, echocardiography and CTPA. So, we decided to perform mechanical thrombectomy. In addition to thrombotic material, fibrous tissue was extracted and histologically analyzed. At the end of the procedure, the patient became hemodynamically unstable (BP 80/40 mmHg and HR 130 bpm) with dyspnea and tachypnea. ABG showed mixed acidosis. Right ventricular function worsened due to high afterload for the persistence of thrombotic material in the pulmonary trunk, defining a picture of obstructive shock. We started fibrinolysis (with rtPA), enoximone and norepinephrine, but asystole occurred and the patient eventually died. The histological examination revealed that the mass was a pleomorphic rhabdomyosarcoma of the pulmonary artery. Primary pulmonary artery sarcoma is an extremely rare and histologically heterogeneous disease. It develops mainly from great pulmonary arteries and then it grows up in the lumen of vessels. It may involve pulmonary valve and RV and invade pulmonary parenchyma and loco–regional lymph nodes. Diagnosis is challenging, because it simulates pulmonary embolism in clinical, echocardiographic and radiological presentation. Suspicion may arise from lack of benefit from anticoagulant therapy. Nodular pulmonary infiltrates are typical. There are two surgical approaches: pneumectomy or endarterectomy (as palliation). Prognosis is poor: mean survival of 1,5 month without therapy and 10 months with pneumectomy. In addition to the progression of the disease, death can occur for obstructive shock, as in our patient.

Surgical Management of Primary Pulmonary Artery Sarcoma

Primary pulmonary artery sarcoma is a rare cardiac tumor with a dismal prognosis without surgical therapy. It is often confused with the more common chronic pulmonary emboli which may delay the appropriate diagnosis or lead to suboptimal surgery. The objective of this study was to evaluate the short and long-term survival and local recurrence rate of pulmonary artery sarcoma cases operated on at our institution using an anatomic resection approach for the pulmonary trunk and main pulmonary arteries rather than endarterectomy. We searched our prospectively collected cardiac tumor database for cases of primary pulmonary sarcoma operated at our institution between June 2000 and September 2018 and followed until January 3, 2021. We used an anatomic resection and replacement technique for involved pulmonary root and main pulmonary arteries with endarterectomy used only for disease distal to the first arterial branch when lung preservation was possible. The primary endpoints for our study were survival from the time of initial diagnosis and survival from the time of our surgery. Secondary endpoints were operative 30-day mortality and incidence of local recurrence or metastatic disease. We identified 20 consecutive cases of surgical resection of primary pulmonary sarcoma. The median age at surgery was 52.5 years (IQR 43.5-60.5). Complete pulmonary root resection and reconstruction using a pulmonary homograft were needed in 16/20 (80%) of cases. All resections employed cardiopulmonary bypass with cardioplegic arrest. A pneumonectomy was needed in 7/20 (35%) of patients. A negative margin (R0) resection was achieved in 9 patients (45%) and margins were microscopically positive (R1) on final pathology in 9 patients (45%). Two patients (10%) had gross tumor (R2) at the resection margin. Operative mortality was 2/20 (10%). Median survival was 2.8 years from diagnosis (95% CI 1.3-8.8) and 2.7 years from surgery by our team (95% CI 0.8-5.9). Survival from first initial diagnosis at 1, 3, 5, and 10 years was 85.0%, 49.1%, 49.1%, and 16.4%. Survival from our surgery by our team at 1, 3, 5, and 10 years was 70%, 48.8%, 41.8%, and 8.4%. Surgical resection of primary pulmonary artery sarcoma with an approach utilizing an anatomic resection of the pulmonary root and main pulmonary arteries when involved and pneumonectomy or endarterectomy when there is disease distal to the first branch artery can be done with a reasonable operative risk and long-term survival when compared to the natural history of the disease.

CLOT OR NOT? AN UNUSUAL CTEPH MIMIC

TOPIC: Pulmonary Vascular Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Chronic thromboembolic pulmonary hypertension (CTEPH) is an important cause of severe pulmonary hypertension (PH) resulting from pulmonary thromboembolic events with subsequent remodeling of pulmonary vasculature. As one of the few forms of PH with potentially definitive management via pulmonary thromboendarterectomy (PTE), it is imperative that the diagnosis of CTEPH involves careful assessment of the patient's history and available studies, including echocardiograms, ventilation-perfusion (V/Q) scans, computed tomography (CT) scans, right heart catheterizations, and pulmonary angiography. This is especially true when considering other conditions that may mimic the presentation of CTEPH. Herein, we discuss a rare instance of pulmonary artery sarcoma (PAS) mimicking CTEPH in a patient with progressive shortness of breath. CASE PRESENTATION: A 53-year-old female with past medical history remarkable for breast cancer status-post lumpectomy and radiation on adjuvant anastrazole monotherapy presented to an outside hospital for progressive shortness of breath and weight loss. CT chest angiography suggested large pulmonary embolism clot burden, and the patient subsequently underwent catheter-directed thrombolysis with tissue plasminogen activator in bilateral pulmonary artery catheters for 24 hours, with minimal change in mean pulmonary artery pressures between times of insertion and removal. The patient subsequently transferred institutions for consideration of PTE.Review of her CT scans and a new V/Q scan suggested a more lobulated, large volume appearance of the obstructing material in her pulmonary vasculature, raising concern for malignant obstruction as opposed to CTEPH. Subsequent positron emission tomography (PET) CT scan showed equivocal fluorodeoxyglucose (FDG) uptake. The patient was brought to surgery, wherein bilateral intravascular tumor involving the main pulmonary artery extending into both branches was discovered consistent with PAS. The mass was extracted, and the patient was eventually discharged with oncologic follow-up, with post discharge course complicated by metastatic disease currently undergoing her third line of therapy. DISCUSSION: This case demonstrates the complexity diagnosing CTEPH. Important mimics of CTEPH outside of PAS include fibrosing mediastinitis, sarcoidosis, large vessel vasculitis, amongst others. Early clinical clues suggesting against the patient's initial diagnosis include recent weight loss and the lobulated appearance of the pulmonary arterial mass. The prognosis of PAS is extremely poor, due to diagnostic and management delays given the stark similarity to CTEPH, with mean survival rates between 12 and 18 months. (1,2) CONCLUSIONS: Diagnosis and management of CTEPH requires expert, multidisciplinary care, especially with the complexity of properly diagnosing the condition amidst other possible mimicking conditions. REFERENCE #1: Bandyopadhyay D, et al. Primary pulmonary artery sarcoma: a close associate of pulmonary embolism-20-year observational analysis. J Thorac Dis 2016; 8: 2592–2601. REFERENCE #2: Wong HH, et al. Presentation and management of pulmonary artery sarcoma. Clin Sarcoma Res 2015; 5: 3. DISCLOSURES: No relevant relationships by Adam Custer, source=Web Response No relevant relationships by NEVEEN El-Farra, source=Web Response No relevant relationships by Jane Wang, source=Web Response No relevant relationships by Alexander Yuen, source=Web Response

A case report of primary pulmonary artery intimal sarcoma

BackgroundPulmonary artery intimal sarcoma (PAS) is a very rare disease, its prevalence is about 0.001–0.003%. PAS is often misdiagnosed as acute or chronic pulmonary thromboembolism due to its clinical presentation and radiological findings. Thus, early diagnosis is very crucial and may improve patient outcome.Case presentationHere, we report a case in a Chinese male where the symptom presentation was episodes of shortness of breath. Transthoracic echocardiography showed a solid mass in the pulmonary valve orifice, which was demonstrated to be a pulmonary artery intimal sarcoma diagnosed by histopathology. In this case, the initial differential diagnosis included pulmonary embolism. Because the initial symptom of primary pulmonary artery sarcoma is extremely similar to the pulmonary embolism, half of them may be misdiagnosed as pulmonary embolism. Imaging studies are very helpful. Ultrasound and CT are the best due to their resolution and ability to assess the relationship of the mass with the surrounding structures. The final diagnosis is mostly made after surgical excision and this is the most effective treatment. At the same time, radiotherapy and chemotherapy after surgery is also an adjuvant treatment.ConclusionWe report a very rare case of pulmonary artery intimal sarcoma. Due to late diagnosis and delayed treatment in this case, the patient displayed a poor prognostic. Early diagnosis and right treatment can improve the prognosis of PAS and optimize overall health.

Primary pulmonary artery sarcoma versus pulmonary thromboembolism: a multimodal imaging comparison

Primary pulmonary artery sarcoma (PPAS) is a rare malignancy that is commonly mistaken for pulmonary embolism due to similarities in clinical presentation and radiographic findings. Distinct radiographic findings to help differentiate between the two diseases are highlighted in the case presented. (1) Several nuances in various imaging modalities have been identified to help distinguish pulmonary artery sarcoma from pulmonary thromboembolic disease. (2) The wall eclipsing sign is considered pathognomonic for pulmonary artery sarcoma. (3) Positron emission tomography/computed tomography may help reduce time between diagnosis and treatment, which may ultimately prolong survival. (4) Providers should be well versed on the subtle differences on imaging to prevent future delays in diagnosis and treatment.

Primary pulmonary artery sarcoma concomitant with chronic thromboembolism: role of 18F-FDG PET/CT

Primary pulmonary artery sarcoma concomitant with chronic thromboembolism: role of 18F-FDG PET/CT

Surgical Treatment of Primary Pulmonary Artery Sarcoma

Surgical Treatment of Primary Pulmonary Artery Sarcoma

A Case Series of Long-Term Surgical Outcomes of Primary Pulmonary Artery Sarcomas With Opportunities for 3D-Printed Models in Surgical Planning.

There are limited data regarding the surgical management of primary pulmonary artery sarcomas (PPAS) because of their rarity and complicated diagnostic history. The objective of this study was to analyze our institution's long-term surgical management outcomes for PPAS in the absence of a care pathway. From May 1997 to June 2013, 8 patients (mean age 60.6 ± 11.8 years; range, 40-73 years; 5 women and 3 men) underwent surgical intervention for PPAS at our institution. The most common computed tomography finding was a luminal filling defect obstructing the pulmonary artery (PA), without evidence of extraluminal extension. Three patients underwent debulking/pulmonary endarterectomy alone and 5 patients underwent a more radical resection with PA patch angioplasty, PA resection and reconstruction, pulmonary valve replacement, and unilateral pneumonectomy. The mean postoperative survival in this series was 3.8 ± 3.6 years (range, 1-11.9 years), with 2 radical surgical resection patients alive at 4.9 and 11.9 years, respectively. For those patients with incomplete resection, 3-dimensional (3D) models were created to demonstrate the advantage of a preoperative guide for a more complete resection and what it would entail. Six patients had local recurrences with mean disease-free interval of 14 ± 10.9 months (range, 2 months-2.5 years), and 2 patients with re-resections had an overall postoperative survival of 2.8 and 11.9 years, respectively. In our small cohort of PPAS, patients treated with radical surgical resection had better survival. The small number of PPAS cases in this series makes proving this association unlikely but warrants consideration.

PULMONARY ARTERY INTIMAL SARCOMA MISTAKEN FOR AN ACUTE PULMONARY EMBOLISM

SESSION TITLE: Fellows Lung Pathology Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Pulmonary artery intimal sarcoma (ISPA) is an exceedingly rare tumor that arises from the vascular lumen of the pulmonary artery (PA). We present a patient who was initially thought to have an acute pulmonary embolism (PE) and later diagnosed with ISPA. CASE PRESENTATION: A 23-year-old woman initially presented to an outside hospital 6 months prior with progressive dyspnea. Imaging reportedly showed an acute PE in the right main PA and the patient was discharged on anticoagulation. Her symptoms worsened. Repeat imaging revealed a large 8.9 cm right hilar mass as well as a large filling defect extending from a stenosed right main PA, across the pulmonary trunk, into left lower lobe PA (Fig 1 A-C). The soft-tissue density and nodular appearance of the defect was more fitting with tumor than recurrent or chronic PE. Positron-emission tomography (PET) showed the right hilar mass was highly FDG avid and the vascular lesion mildly FDG avid, consistent with malignancy (Fig 1 D). The patient underwent percutaneous biopsy of the mass. Histopathology revealed a poorly differentiated sarcoma. FISH studies were positive for MDM2 (murine double minute 2). Together these findings were consistent with ISPA. The patient was started on chemotherapy with doxorubicin, ifosfamide, and mensa. Restage Imaging after 4 of 6 cycles showed a significant response to therapy (Fig 2), with the plan for surgical reconstruction with endarterectomy. DISCUSSION: Pulmonary artery sarcomas are an extremely rare malignancy, with an estimated incidence of 0.001-0.03% and only 200-400 cases described, the majority post-mortem. There appears to be a slight female predominance (1.3:1) with a mean age of diagnosis of 48 years (13-86 years). These tumors arise in the pulmonary trunk, either in the intimal (ISPA) or mural layer, and extend locally along the vasculature into the adjacent structures. The majority of patients present with symptoms of PE or right heart failure and are initially misdiagnosed. Diagnosis requires tissue biopsy. Histopathology often shows poorly differentiated, malignant spindle or epithelioid cells with atypia. Immunohistochemical staining is variable, sometimes with desmin or smooth muscle actin positivity. MDM2 is a proto-oncogene that regulates p53 activity and is highly expressed in the majority of ISPA. Surgical resection with endarterectomy is considered first line and confers a modest survival benefit (10 vs 1.5 months, compared to no surgery). Neoadjuvant chemotherapy improves surgical outcomes and survival, however due to its rarity, there is no consensus treatment and data is extrapolated from extrapulmonary sarcomas, often using doxorubicin-based regimens. CONCLUSIONS: ISPA is an incredibly rare malignancy that is often misdiagnosed a PE and carries an overall grim prognosis. Reference #1: Mussot S, et al. Retrospective institutional study of 31 patients treated for pulmonary artery sarcoma. Eur J Cardiothorac Surg. 2013 Apr;43(4):787-93. PMID 22843511 Reference #2: Assi T, et al. A comprehensive review of the diagnosis and management of intimal sarcoma of the pulmonary artery. Crit Rev Onc. 2020 Jan;147: 1040. Reference #3: Blackmon SH, et al. Management of primary pulmonary artery sarcoma. Ann Thorac Surg. 2009 87(3):977-984. DISCLOSURES: Speaker/Speaker's Bureau relationship with Boehringer-Ingelheim Pharmaceuticals Please note: $5001 - $20000 Added 04/07/2020 by Jaime Betancourt, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Vapotherm, Inc Please note: $5001 - $20000 Added 04/07/2020 by Jaime Betancourt, source=Web Response, value=Honoraria No relevant relationships by Richard Watson, source=Web Response

Surgical treatment of primary pulmonary artery sarcoma

ObjectivePrimary pulmonary artery sarcoma (PAS) is a rare tumor that originates from the intimal layer of the pulmonary artery or pulmonary valve and has a poor prognosis. The standard treatment for this devastating disease remains unclear. This study aimed to summarize the current standard treatments for PAS.MethodsFrom September 2015 to January 2020, six patients were diagnosed with PAS and underwent pulmonary endarterectomy (PEA) at our department. Their medical records were retrospectively reviewed to analyze the clinical characteristics, histopathological features, and postoperative outcomes. Fourteen articles, each reporting at least 6 cases, identified 201 patients diagnosed with PAS, and 158 patients had detailed treatments and follow-up data.ResultsAll of the patients who successfully underwent PEA were alive at follow-up, with a mean survival duration of 11.6 months (7–28 months), and one patient developed recurrence in the right upper lobe lung. Two patients received postoperative chemotherapy. In one patient, the tumor invaded the pulmonary valve.ConclusionsPAS resection combined with PEA via the aid of cardiopulmonary bypass and deep hypothermic circulatory arrest could achieve maximal tumor resection in patients without metastatic lesions. An individualized surgery strategy relies on a precise preoperative imaging examination. Moreover, postoperative adjuvant therapy could yield improved survival outcomes.

P2.15-08 Multicenter Experience with Complete Surgical Resection of Primary Pulmonary Artery Sarcoma

P2.15-08 Multicenter Experience with Complete Surgical Resection of Primary Pulmonary Artery Sarcoma

From Pulmonary Artery Repair to Right Ventricular Outflow Tract Replacement

Abstract Primary pulmonary artery sarcomas are extremely rare tumors with approximately 400 cases reported so far in the literature. These patients present with a wide myriad of symptoms ranging from dyspnea to florid right heart failure, and the presentation is usually misdiagnosed and treated as chronic thromboembolic disease. Diagnostic imaging requires the use of multiple modalities including computed tomography, magnetic resonance imaging and echocardiography. In this E-challenge, we describe the case of a patient diagnosed preoperatively with pulmonic artery stenosis. Intraoperative imaging demonstrated a pulmonary artery lesion with subsequent biopsy and a pathological diagnosis.

Occlusion of the pulmonary artery by a primary pulmonary artery sarcoma resulting in cardiac arrest: a case report

BackgroundPrimary pulmonary arterial sarcoma (PPAS) is a rare condition. Although resection is recommended to improve prognosis, optimal anesthesia management for these cases remains unclear.Case presentationA 62-year-old woman with a diagnosis of left pulmonary PPAS underwent surgical tumor resection and left lung pneumonectomy. Preoperative symptoms included a cough and hemoptysis. Computed tomography revealed a complete obstruction of the left pulmonary artery, with tumor extension into the right pulmonary artery, and mild tricuspid regurgitation was observed on the echocardiogram. Ninety minutes after anesthesia induction, the patient went into cardiopulmonary arrest. As the surgical field was sterilized, we proceeded with emergent sternotomy and cardiac massage. Extracorporeal circulation was established, and surgery proceeded once spontaneous circulation was recovered. The patient survived without neurological complications.ConclusionsBased on our experience and in the absence of evidence-based guidelines, the femoral artery and vein should be cannulated in all cases for extracorporeal circulation initiation before anesthesia induction.

Imaging and Surgical Treatment of Primary Pulmonary Artery Sarcoma.

Primary pulmonary artery sarcoma is a rare tumor originating from the pulmonary artery tree. Given the low incidence, few centers have reported on more than a handful of cases. Because of its rarity it is also commonly misdiagnosed as pulmonary embolism. Multi-modality diagnostic imaging and recognition of specific imaging characteristics along with a high index of suspicion is required to make the correct diagnosis and expedite treatment. The primary imaging modality for most cardiac tumors such as primary pulmonary artery sarcoma is now MRI. It provides superb spatial resolution as well as functional assessment of the heart and pulmonary circulation. CT imaging also is part of routing imaging and remains as the most pertinent imaging modality to evaluate the lung parenchyma and presence of metastatic disease. Here we review the pertinent imaging modalities and tissue characteristics that facilitate recognition of primary pulmonary artery sarcoma. We also provide a short overview of surgical resection and reconstruction, which is the mainstay therapy, for this rare tumor.

Clinical presentation and surgical treatment of primary pulmonary artery sarcoma.

Pulmonary artery sarcoma (PAS) is a rare and devastating disease. The diagnosis modality and optimal surgical strategy remain unclear. The aim of this study was to report our surgical experience in this disease. Between 2000 and 2017, 13 patients underwent operations for PAS at our institution. Five patients underwent tumour resection alone, whereas the other 8 patients received pulmonary endarterectomy. The medical records were retrospectively reviewed to evaluate the clinical characteristics, operative findings and the postoperative outcomes. There were 2 perioperative deaths, both had unresolved severe pulmonary hypertension. Follow-up was completed for all patients with a mean duration of 14.3 months (1-48 months). During follow-up, 6 patients died after a mean survival time of 20.2 months (4-48 months). For the 5 patients alive at follow-up (mean duration of 7.2 months), 3 already had local recurrence. For all 13 patients, the cumulative survival rates at 1, 2 and 3 years were 56.4, 37.6 and 18.8%, respectively. The 8 patients treated with pulmonary endarterectomy seemed to have a better survival rate compared with the remaining 5 patients without endarterectomy, although the difference was not statistically significant. Primary PAS is often mistaken for chronic pulmonary thromboembolism. Surgical intervention is the mainstay of treatment for PAS, but the prognosis after surgery remains poor. Compared to isolated tumour resection, pulmonary endarterectomy seemed to yield a better survival rate.

Primary pulmonary artery sarcoma masquerading as pulmonary thromboembolism: a rare diagnosis unveiled

BackgroundPrimary pulmonary artery sarcomas are rare malignant vascular tumors and carry a very poor prognosis. Due to overlapping clinical and radiological features, the differentiation between pulmonary artery thromboembolism and pulmonary artery sarcoma can be challenging.Case presentationWe herein present clinical, radiological and pathological features of primary pulmonary artery high grade sarcoma (angiosarcoma) in a 59-year-old male. The patient presented with a history of breathlessness on exertion of 2-months duration and was misdiagnosed as massive pulmonary thromboembolism on initial CT imaging.ConclusionGreat similarity with significant degree of overlap in clinical and radiologic presentation makes differentiation of pulmonary artery sarcomas and thromboembolism a diagnostic challenge. Even though they are exceptionally rare, one should always consider it as differential diagnosis especially in cases with atypical clinical or imaging presentation.

Case Report: Spindle Cell Sarcoma of the Pulmonary Artery

Primary pulmonary artery sarcomas are rare. Moreover, they are notorious for masquerading as a chronic pulmonary embolus. There is much symptom overlap that leads to this relatively common misdiagnosis. We present such a case.

Primary pulmonary artery sarcoma: a close associate of pulmonary embolism-20-year observational analysis.

Primary pulmonary artery sarcoma (PPAS) is a rare tumor that mimics pulmonary thromboembolism (PE). Similarities to PE can delay the diagnosis and misguide the treatment of PPAS. This study aimed to evaluate tumor characteristics and outcome predictors among those diagnosed with PPAS and misdiagnosed as PE. From 1991-2010, 10 PPAS cases were available from the Cleveland Clinic (CC) institutional database and another 381 cases were reported in the literature. Patient characteristics, tumor subtypes, diagnostic testing & timing, interventions and clinical outcomes were analyzed. We also noted effects of misdiagnosis as PE and clinical outcome as a result of inappropriate intervention. Among 391 confirmed cases of PPAS, the mean age at diagnosis was 52±14 years; 55% were male. The median duration of symptoms prior to diagnosis was 100 [interquartile range (IQR), 30-210] days. Nearly half (47%) of PPAS were originally misdiagnosed as PE including 39% that received thrombolytic and/or anticoagulation therapy. For every doubling of time from symptom onset to diagnosis, the odds of death increased by 46% (OR: 1.46, 95% CI: 1.21-1.82; P<0.001). The odds of death (OR: 2.66, 95% CI: 1.58-4.54; P=0.0003) and occurrence of distant metastasis (OR: 2.30, 95% CI: 1.30-4.15; P=0.049) were increased among those who did not receive chemotherapy but chemotherapy did not impact local recurrence. Those with complete resection had a better survival. PPAS has a radiological appearance similar to PE, which makes accurate and timely diagnosis challenging. More rapid diagnosis may lead to earlier, appropriate surgical treatment and improved outcomes, when combined with adjuvant treatment.