Primary cardiac lipoblastoma of the right atrium

Elsevier

Available online 29 April 2023, 107542

Cardiovascular PathologyAuthor links open overlay panel, , , , , ABSTRACT

Lipoblastoma is a rare neoplasm of the embryonal white fat. It occurs most commonly in children under the age of 3 years and usually inflicts the superficial soft tissues of trunk and extremities. We present the case of a 3-year-old male patient with a successfully resected primary cardiac right-atrial lipoblastoma with COL1A2::PLAG1 gene fusion.

Section snippetsINTRODUCTION

Primary cardiac neoplasms are considered rare. Although uncommon, they are clinically significant. Even histopathologically benign entities may lead to troubling symptoms given their local involvement and exertion of pressure on surrounding structures – they may impair the heart´s mechanical function, cause blood flow obstruction, valvular dysfunction or interfere with the heart conduction system. Clinical presentation of tumours of the heart varies depending on the size and location [1].

The

CASE REPORT

A 3-year-old apparently asymptomatic boy presented with a heart murmur on a routine medical check-up. No other symptoms such as coughing, wheezing or shortness of breath were present. The patient was born by a vaginal delivery without complication to a gravida 3 para 3 mother, weighing 3040 g at birth. No developmental issues or congenital heart disease were diagnosed. He only had a history of more frequent respiratory infections.

Upon the finding of the heart murmur, the patient was admitted

DISCUSSION AND CONCLUSION

Lipoblastoma is a rare benign neoplasm and a the cardiac involvement is even rarer. There are only very few cases reporting a primary cardiac lipoblastoma and there has been no other cardiac case with reported molecular analysis of the neoplasm [3], [4], [5], [6]. We found COL1A2::PLAG1 fusion gene in our case, which is one of the typical fusion genes in lipoblastomas [7] occuring in more frequent soft tissue locations. This molecular finding further supports the diagnosis and demonstrates,

Declaration of Competing Interest

The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Miroslav Koblizek reports financial support was provided by Ministry of Health of the Czech Republic.

Funding

This work was supported by Ministry of Health, Czech Republic – conceptual development of research organization, Motol University Hospital, Prague, Czech Republic 00064203.

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