Primary pulmonary angiosarcoma

Primary pulmonary angiosarcoma is a rare type of malignant vascular tumour with poor prognosis. Diagnosis is often late due to non-specific symptoms and low clinical suspicion for angiosarcoma. A 72-year-old man presented to hospital with a 6-month history of mild progressive dyspnoea, with associated cough, episodes of presyncope and weight loss. CT pulmonary angiogram (CTPA) was reported as a large saddle pulmonary embolism extending into both the right and left pulmonary arteries. Further Multidisciplinary team meeting (MDM) discussion, and review of CTPA and subsequent investigations revealed a large primary pulmonary artery sarcoma which was later confirmed histology. The patient was referred to the cardiothoracic surgeons and underwent left radical pneumonectomy.

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.

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.

Transient Response of Olaparib on Pulmonary Artery Sarcoma Harboring Multiple Homologous Recombinant Repair Gene Alterations

Primary pulmonary artery sarcoma (PPAS) is a rare malignancy arising from mesenchymal pulmonary artery cells and mimics pulmonary embolism. Palliative chemotherapy such as anthracycline- or ifosfamide-based regimens and targeted therapy are the only options. However, the evidence of clinically beneficial systemic treatment is scarce. Here, we report a case of disseminated PPAS achieving clinical tumor response to olaparib based on comprehensive genetic profiling (CGP) showing genetic alterations involving DNA repair pathway. This provides supportive evidence that olaparib could be a promising therapeutic agent for patients with disseminated PPAS harboring actionable haploinsufficiency of DNA damage repair (DDR).

Primary Pulmonary Artery Sarcoma Confined to the Left Pulmonary Artery

Primary pulmonary artery sarcomas (PPAS) are extremely rare tumors that are often mislabeled as pulmonary emboli (PE). PPAS usually involve the pulmonary trunk and are histologically classified as leiomyosarcoma, spindle cells sarcoma, fibrous histiocytoma, or undifferentiated sarcoma. Our case involved a 78-year-old man with an undifferentiated PPAS confined to the left pulmonary artery that was initially misdiagnosed as a PE. After a month-long delay in treatment in which the patient was prescribed warfarin, the correct diagnosis was made. Pulmonary artery endarterectomy and left lung pneumonectomy were performed, and he survived for 18 months before disease recurrence and death. Our case helps illustrate some of the clinical and radiographic findings that help distinguish PPAS from PE.