Primary malignant pericardial tumour in Lynch syndrome

Abstract

Background: This case represents the first report of malignant primary cardiac tumour in a patient with Lynch Syndrome associated with MSH2 pathogenic variant. Case presentation: A 57-year-old woman with previous ovarian cystadenocarcinoma was admitted to the emergency room for hematic pericardial effusion. Multimodal diagnostic imaging revealed two solid pericardial vascularized masses. After pericardiectomy, the final histological diagnosis was poorly differentiated pleomorphic sarcomatoid carcinoma. During follow-up she developed an ampulla of Vater adenocarcinoma. Genetic analysis identified an MSH2 pathogenic variant. Conclusion: This case contributes to expand the tumour spectrum of Lynch syndrome, suggesting that MSH2 pathogenic variants cause a more complex multi-tumour cancer syndrome than the classic Lynch Syndrome. In MSH2 variant carriers, symptoms such as dyspnoea and chest discomfort might alert for rare tumours and a focused cardiac evaluation should be considered.

Myocardial infarction with nonobstructive coronary arteries: from pathophysiology to therapeutic strategies

“…108 The beneficial effects of RASS inhibitors and statins for secondary prevention in MINOCA patients have been reported, although, no distinction on the underlying pathophysiological mechanism was made. 56,109 Takotsubo syndrome It is still controversial whether TTS should be classified as a particular form of MINOCA or a separate entity, due to its specific clinical characteristics and the still undetermined pathophysiologic mechanisms. TTS, also defined as stress-induced cardiomyopathy, is a condition mimicking an ACS/AMI, typically triggered by emotional or physical stress.…”

“…Finally, whether some types of drugs that showed good results on chronic exercise-induced microvascular angina, including RASS inhibitors and statins, may be of some benefit in CMVS-induced MINOCA remains unexplored 108 . The beneficial effects of RASS inhibitors and statins for secondary prevention in MINOCA patients have been reported, although, no distinction on the underlying pathophysiological mechanism was made 56,109 …”

“…50,52,53 Single small emboli or multiple microemboli can derive from the lysis of angiographically visible or nonvisible partial non occlusive thrombi formed on disrupted nonsignificant epicardial plaques (see above), but direct coronary embolism may originate from a thrombus located in the left atrium, left ventricle, or pulmonary veins. Furthermore, infective or noninfective endocardial vegetations of the aortic or mitral valves, as well as intracardiac tumors, can also be responsible for CAE and MINOCA, [54][55][56][57][58][59] which, in rare cases, can also be caused by paradoxical embolism due to right-left intracardiac shunts, mainly related to the presence of patent foramen ovale (PFO). 60 Finally, iatrogenic CAE may occur as a consequence of clot formation on catheters during prolonged invasive coronary or intracardiac procedures, in particular when a correct anticoagulation is not maintained and/or catheters are not well flushed.…”

Clues and pitfalls in the diagnostic approach to cardiac masses: are pseudo-tumours truly benign?

“…Conversely, symptomatic patients (dyspnoea or chest pain above all) with lesions detected in the right heart chambers or in the pericardium/great vessels and the occurrence of pulmonary embolism, must arise the suspicion of malignancy. 7 Nevertheless, the clinical and first-line echocardiographic evaluation is often inconclusive, requiring further investigations. 8 Although cardiac MR is the most powerful tool in this context, 8 we previously reported the utility of integrating CT and PET information in specific clinical settings.…”