1. A rare case of type III pleuropulmonary blastoma infiltrating the left heart in an 11-year-old girl.
- Author
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Ndaumanu, Ishak, Rantesalu, Anthomina Maya, and Laksmono, Navy
- Abstract
Pleuropulmonary blastoma (PPB) is a rare primary malignant tumor in the chest that mainly occurs in children <6 years of age. Vascular extensions are even rarer, approximately 3 % of types II and III PPB, and have fatal complications. The patients of reported cases who had tumor extension to the heart are younger than three years old, whereas in this case, we reported an 11-year-old girl who was of school age. This case report aims to describe a rare case of a type III Pleuropulmonary Blastoma infiltrating the left heart of a school-age girl. An 11-year-old girl presented at an emergency department with two months of progressive dyspnea with malnutrition. A fused mass was found in LA on an echocardiogram along with moderate MR, severe MS, and mild pericardial effusion. CT scan showed a massive pleural effusion with a solid mass in the left lung obstructing the left bronchial tree, accompanied by the expansion of the tumor mass into the left pulmonary vein and LA. Total removal of the tumor was performed, aided by cardiopulmonary bypass. Type III PPB was confirmed histopathologically. PPB is a rare, aggressive tumor that has three types. Various manifestations can occur in line with the presence of metastases. The treatment consists of aggressive surgery and chemotherapy. Because of its poor prognosis, prompt recognition of the involvement of the cardiac chamber and great vessels in type III PPB should be considered before surgery. • Type III Pleuropulmonaryblastoma or purely solid PPB have the least favorable outcomes with a 53 % survival rate as they may present at more advanced age and advanced stage • Type II and III PPB are both aggressive tumors. Surgical resection and systemic chemotherapy are important roles in the treatment. • Radical resection and absence of metastasis are promising and can be done safely with the aid of chemeotherapy and possibly radiotherapy [ABSTRACT FROM AUTHOR]
- Published
- 2024
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