Abstract 17233: Primary Intimal Sarcoma Of The Pulmonary Artery In A Patient With Laboratory Confirmed Heterozygous Factor V Leiden Mutation: A Case Report

Pulmonary artery intimal sarcoma (PAIS) is a rare tumor associated with progressively increasing dyspnea, atypical chest pain, and pulmonary hypertension. Owing to overlap in presentation, PAIS can be misdiagnosed as pulmonary thromboembolism (PTE). Here we present the case of a 59-year-old male with heterozygous Factor V Leiden mutation, history of deep vein thrombosis (DVT), and recent PTE presenting to urgent care with cough, mild orthopnea, and exertional dyspnea. Transthoracic echocardiogram (TTE) revealed severely dilated right ventricle with moderately decreased function and severe pulmonary hypertension with pulmonary arterial (PA) pressure of 93mmHg. Computed tomography angiography of the chest revealed massive central PTE. Given hemodynamic stability, saturating well on room air and simplified PESI score of 0, Pulmonology recommended discharge with anticoagulation. The patient returned a month later with three weeks of increased dyspnea on exertion, worsened right ventricular function as well as possible new mobile thrombus extending into the right ventricular outflow tract (RVOT). Due to presumed failure of medical therapy, Cardiology placed the patient on venoarterial extracorporeal membrane oxygenation (VA-ECMO) followed by attempted mechanical thrombectomy using both the Angiovac and Penumbra CAT12 systems. Both systems failed to remove substantial material from the PA. The next day, TTE revealed increased PA pressure of 132mmHg.The patient underwent emergent bilateral thromboendarterectomy with a mass discovered involving the pulmonary valve (PV), RVOT, both left and right PAs, along with invasion into the PA wall. The mass was resected, the PV valve replaced, the main PA and left PA reconstructed, and pericardial patch reconstruction of the RVOT was performed. The mass was determined to be a PAIS of French Federation of Comprehensive Cancer Centres (FNCLCC) Grade 3. Two months after uncomplicated recovery, chemotherapy was initiated. Securing the diagnosis of PAIS is challenging. However, collaboration between clinicians, radiologists, and pathologists increases the likelihood of recognizing subtle markers that can differentiate PAIS from illnesses with a similar presentation.

A Case Report of primary cardiac intimal sarcoma presenting with atrial fibrillation and a Left Atrial Mass

Background Intimal sarcoma is an exceedingly rare type of primary cardiac tumour. It is characterized by poorly differentiated spindle-shaped cells that can mimic smooth muscle and is strongly associated with MDM2 genetic amplification. Owing to its rarity and nondistinctive histological features, diagnosis remains a significant challenge. Case Summary In this case report, we describe a case of primary cardiac intimal sarcoma in a 37-year-old woman who presented with atrial fibrillation and a left atrial mass. Despite having a histological sample from an excised left atrial mass, the diagnosis was not made until she presented with back pain secondary to metastatic disease to the spine. Discussion Primary cardiac intimal sarcoma is an extremely rare diagnosis. The mainstay management of intimal cardiac sarcoma is aggressive surgical resection. Unfortunately, the prognosis of cardiac sarcomas remains very poor, with a mean survival between three months to one year. This case of cardiac intimal sarcoma highlights the difficulty in establishing a diagnosis, particularly given the unusual presentation of atrial fibrillation.

Small Bowel Intussusception Due to Rare Cardiac Intimal Sarcoma Metastasis: A Case Report

Background: Intimal sarcomas are rare malignant mesenchymal tumors arising from the heart and large blood vessels. Their intraluminal growth leads to vascular obstructive symptoms and peripheral neoplastic embolization. Direct infiltration of the lungs or metastases to the pulmonary system, occur in 40% of cases and extrathoracic spread is frequent, also in presentation. Intussusception is an unusual event in adults, accounting for <5% of bowel obstructions. In most cases it is caused by a malignancy and requires surgical resection.Case Presentation: We describe a rare case of a 50-year-old man suffering of bowel obstruction due to intussusception sustained by a small bowel metastasis of a primary cardiac intimal sarcoma. One year and a half before the onset of abdominal symptoms, a grade II intimal sarcoma was removed from his left atrium and consequently he followed a chemotherapy protocol. Four months later a CT scan revealed local recurrence. Eighteen months after heart surgery he referred to the ER with abdominal pain. CT scan showed an ileal intussusception and the patient was scheduled for surgery. A tract of 10 cm ileus was removed containing an intramural polypoid solid mass. Histological analyses revealed a grade II intimal sarcoma consistent with his first diagnosis.Conclusion: Primary heart tumors are late found and often partially resected, therefore metastatic pathways are to be expected. Adult small bowel intussusception is a rare event and caused by a malignancy in one third of cases. Therefore, our recommendation is to always resect the tract involved in order to perform a proper diagnosis.

A case report of primary pulmonary artery intimal sarcoma

Background Pulmonary 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 presentation Here, 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. Conclusion We 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.

Pulmonary Artery Intimal Sarcoma Diagnosed as Pulmonary Embolism

Background: Pulmonary artery intimal sarcoma (PAIS) is a rare malignancy of vasculature that carries a very poor prognosis that is often misdiagnosed as a pulmonary embolism due to overlapping clinical and radiological features. It is a very aggressive malignancy with surgery the mainstay of initial management. Chemotherapy is normally given postoperatively, although it is not clear if chemo and radiotherapy bring any improvement, some data report an increase in survival in patients who received multimodality therapy vs single therapy. Case: A 48 year-old female was initially diagnosed with a pulmonary embolism based on clinical presentation and imaging. She was treated vigorously but continued to show no improvement after two weeks. She underwent an open thrombectomy and a large concurrent pulmonary artery intimal sarcoma (PAIS) was found. The mass was adherent to the intima and extended into the left and right pulmonary arteries. The tumor was not fully resected and tissue results showed poorly differentiated sarcoma. A treatment plan was then initiated to include systemic chemotherapy. She developed metastatic disease despite receiving multimodal therapy and died with within 2 years from the initial diagnosis. Conclusion: Persistent symptoms in patients diagnosed and treated adequately for pulmonary artery thrombosis should indicate a possible comorbid condition including sarcoma. This is particularly the case in the older age group with imaging that suggests central embolism despite treatment.

A Case Report of Primary Pulmonary Artery Intimal Sarcoma

Background: Pulmonary artery intimal sarcoma(PAS) is a very rare disease, the prevalence of it is about 0.001%-0.003%1. 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 presentation: Here we report a case in a Chinese male which the symptom as 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 histopathology2. In this case the initial differential diagnosis included pulmonary embolism. Because the initial symptom of primary pulmonary artery sarcoma is extremely similiar 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 a adjuvant treatment3.Conclusion: We report a very rare case of pulmonary artery intimal sarcoma, due to late diagnosis and delayed treatment in this case, the patient display a poor prognostic. Early diagnosis and right treatment can improve the prognosis of PAS and optimize overall health.