Why the study?
Cardiac papillary fibroelastoma is a rare tumor, and it is especially rare when located on the pulmonary valve.
This case highlights the successful surgical management of a rare pulmonary valve papillary fibroelastoma and the challenges of its preoperative imaging diagnosis.
BACKGROUND: Cardiac papillary fibroelastoma (PFE) is a rare tumor, and especially rare when found on the pulmonary valve. CASE PRESENTATION: We report the case of a 70-year-old woman patient with a pulmonary valve PFE diagnosed incidentally during a follow-up of aortic regurgitation. Computed tomography and magnetic resonance imaging showed no suggestive signs of malignant tumors, and thrombus or myxoma was initially suspected. However, an initial transthoracic and transesophageal echocardiogram did not exclude the possibility of a malignant tumor attached to the wall of the pulmonary artery. Considering the embolization risk, we opted to perform tumorectomy, in which additional surgical procedures could then be conducted if intraoperative diagnosis showed a malignant tumor. Indeed, intraoperative findings showed the tumoral mass attached on the left semilunar cusp of the pulmonary valve, and intraoperative diagnosis of the tumor showed no malignancy. Planned tumorectomy was performed concomitantly with AVR. The pathologic examination of the removed tumor confirmed the diagnosis of PFE. Her postoperative course was uneventful without any sign of recurrence. CONCLUSION: This case highlights the difficulty of accurate diagnostic imaging and provides valuable insight into a successful surgical treatment of pulmonary valve PFE without any complications.
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Uehara et al. (2022) studied this question.
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