Rare primary cardiac myxofibrosarcoma in adolescence: Diagnostic challenges and surgical insights

• Published in: Cardiovascular pathology Vol. 70; p. 107623
• Main Authors: Machuca-Aguado, Jesús, Rendón-García, Rosa, Miraglia, Alessia, Téllez-Cantero, Juan Carlos, Gutiérrez-Domingo, Álvaro
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.05.2024
• Subjects: Acute hearth failure; Adolescent; Biopsy; Cardiac myxofibrosarcoma; Cardiac Surgical Procedures; Fatal Outcome; Female; Fibrosarcoma - diagnosis; Fibrosarcoma - pathology; Fibrosarcoma - surgery; Heart Failure - etiology; Heart Failure - surgery; Heart Neoplasms - diagnostic imaging; Heart Neoplasms - pathology; Heart Neoplasms - surgery; Humans; Neoplasm Grading; Primary cardiac neoplasm; Primary cardiac neoplasm; Acute hearth failure; Cardiac myxofibrosarcoma
• ISSN: 1054-8807; 1879-1336
• DOI: 10.1016/j.carpath.2024.107623

•Myxofibrosarcoma is an exceptionally uncommon cardiac neoplasm.•Unusual in teenagers and a rarity even among young adults.•It is a condition that requires ruling out other entities for its diagnosis. This article presents a unique case of primary myxofibrosarcoma, an exceptionally rare cardiac neoplasm, in a 16-year-old girl. With fewer than 40 documented cases, this tumor's occurrence in adolescence is particularly uncommon. The patient presented with right-sided paresthesia and subsequent right hemiparesis and dysarthria, along with bilateral pleural effusion. Imaging tests revealed a cerebral ischemic lesion and the presence of a left atrial mass. Initially suspected as a myxoma, the tumor exhibited significant infiltration. Despite prompt excision and cardiac intervention, the patient experienced rapid decompensation and succumbed to heart failure 2 days later. Histopathological analysis revealed an intermediate grade myxofibrosarcoma. This case underscores the diagnostic complexity, emphasizing the need for early identification given the grim prognosis associated with cardiac myxofibrosarcomas, requiring detailed imaging, surgical, and histopathological insights.