Pulmonary artery intimal sarcoma (PAIS) is a rare malignancy which closely mimics acute or chronic pulmonary thromboembolism. There are clinical and radiological characteristics which may raise suspicion of this important differential diagnosis. These include disproportionately low
d
‐dimer, troponin T or NT‐proBNP, as well as characteristic findings on CT pulmonary angiography such as the ‘wall eclipsing sign’ and an non‐dependent position of filling defects in the large arteries. Prompt diagnosis avoids inappropriate anticoagulation and facilitates early surgical management which may improve prognosis. There is emerging evidence of an effective treatment paradigm with surgical resection and adjuvant chemotherapy. We present two cases of PAIS diagnosed at a single centre within a 2‐year period. We review the literature and demonstrate the features at presentation in our cases which were suggestive of the diagnosis.
Background
Mantle field radiotherapy has been known to cause cardiovascular complications even years after therapy. Complications include pericardial disease, coronary artery disease and conduction abnormalities.
Case Summary
We present a case series of two patients who developed cardiovascular complications years after receiving mantle radiation. Patient 1 is a 52-year-old male who presented with symptoms of heart failure. He had a neurostimulator which precluded him from cardiac MRI. Haemodynamic findings on right heart catheterisation raised suspicion for constrictive pericarditis and pericardiectomy was performed. Histopathological analysis reported dense, sclerotic fibrous tissue consistent with radiation-related changes. Patient 2 is a 37-year-old male with a 2-month history of chest pain and exertional dyspnoea who was admitted for management of coronary artery disease. Coronary angiography demonstrated bilateral subclavian artery stenosis and an elevated left ventricular end-diastolic pressure (50 mmHg). He had bilateral percutaneous subclavian artery stenting. Both patients had complete resolution of symptoms on Follow-up.
Discussion
Our case series emphasises the need for an index of suspicion for radiation-related cardiovascular changes in patients who have a history of mantle radiation, especially in younger patients. This was especially pertinent in the case of our first patient who presented a diagnostic challenge due to certain patient factors. Our second patient is a case of subclavian artery stenosis which is less frequently reported as a complication of mantle radiation in the literature.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.