Surgery for Primary Cardiac Tumors in Children: Successful Management of Large Fibromas

Abstract

BackgroundPediatric primary cardiac tumors (PCTs) are rare. Its clinical features and prognoses are not well defined. The management of asymptomatic patients with cardiac fibromas remains controversial.ObjectiveWe aimed to examine our experience in surgical resection of pediatric PCT, with specific focuses on the management of large fibromas.MethodsThis study included all the children who underwent surgical resection of PCT in our institution between December 2008 and June 2021. The last follow-up was performed between June 1st and August 26th, 2021. Kaplan–Meier method was used to estimate the postoperative survival, freedom from reoperation, event-free survival, and also related risk factors. The tumor volume and volume index (volume divided by body surface area) were measured for cardiac fibromas.ResultsOf the 39 patients with median operative age of 9.5 [interquartile range (IQR): 1.2–16.5] years, 35 (89.7%) had benign tumors (fibromas for 15, myxomas for 13, and others for 7). The length and volume of fibromas were independent of age and symptoms (Ps > 0.05). The fibroma volume index was negatively correlated with age (P = 0.039), with a mean value of 105 ± 70 ml/m2. Of the 15 patients with fibromas, 5 were asymptomatic, 4 received partial resection, 4 required transmural resection, and 4 presented postoperative left ventricular (LV) dysfunction (ejection fraction <50%). During the median follow-up period of 3.1 years and maximum of 12.5 years, adverse events included 2 early and 1 late death, 4 reoperations, 4 tumor recurrences, and 1 LV dysfunction lasting over one year. The 8-year survival, freedom from reoperation, and event-free survival rates were 90.4, 81.8, and 64.2%, respectively. Malignant tumor (P < 0.001) was associated with more adverse events. Transmural resection (P = 0.022) and larger tumor volume index than LV end-diastolic volume (P = 0.046) were risk factors for LV dysfunction following fibromas resection.ConclusionPediatric surgery for PCT can be performed with low mortalities and few adverse events. The size of cardiac fibroma in children relatively decreases with the increase of age. Larger tumor volume index than LV end-diastolic volume index and transmural tumor resection predicts postoperative LV dysfunction.

Differential diagnosis of cardiac tumors: General consideration and echocardiographic approach

“…The best imaging technique in differentiating benign masses from their malignant counterpart (myxoma vs. myxosarcoma, fibroma vs. fibrosarcoma, angioma vs. angiosarcoma and so on) is PET/CT, which is based on the metabolic activity of the tissues: using 18-Fluorodeoxiglucose ( 18 FDG), a high maximum standardized uptake (SUVmax) and intratumoral glucose metabolic heterogeneity factor (HF) may discriminate well between malignant (sarcomas, lymphomas, or other malignant tumors) and benign masses, as well as in differentiating a tumor thrombus from a simple thrombus. [105][106][107][108][109][110] To improve the sensitivity and specificity of 18 FDG in the particular setting of cardiac tumors, the physiologic glucose uptake by the myocardium must be minimized. This can be obtained with a low-carbohydrate, high-fat diet and with the administration of 50 Units/Kg of body weight of unfractioned heparin before the injection of 18 FDG.…”

“…Some tumors‐like rhabdomyomas‐ may also show a spontaneous regression over time; thus a follow‐up strategy is usually adopted 8–12 . However, even benign tumors may cause clinical symptoms due to hemodynamic impairment (valve obstruction, compression of vessels and cardiac chambers), arrhythmias, and embolism requiring therapy, either medical or surgical 13–20 . Rhabdomyomas inducing atrial or ventricular arrhythmias have been reported in 16%–47% of cases; an interesting observation is that the tumor cells are structurally similar to Purkinje cells and can function like accessory pathways creating continuity at the atrioventricular junction inducing a pre‐excitation syndrome which disappears with the tumor regression 21,22 .…”

“…8 , 9 , 10 , 11 , 12 However, even benign tumors may cause clinical symptoms due to hemodynamic impairment (valve obstruction, compression of vessels and cardiac chambers), arrhythmias, and embolism requiring therapy, either medical or surgical. 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 Rhabdomyomas inducing atrial or ventricular arrhythmias have been reported in 16%–47% of cases; an interesting observation is that the tumor cells are structurally similar to Purkinje cells and can function like accessory pathways creating continuity at the atrioventricular junction inducing a pre‐excitation syndrome which disappears with the tumor regression. 21 , 22 The mainstay of treatment of hematological neoplasms (either primary, like some lymphomas, or metastatic) is chemotherapy, and a surgical approach is seldom required.…”

Current status of diagnosis and treatment of primary benign cardiac tumors in children

“…CF is children's second most common benign cardiac tumor after CR, and is also more common in infants (32,33). Male has a slight predominance, and usually single tumor.…”

Tumor cardíaco congénito simulador de malignidad: a propósito de un caso

“…El Rabdomioma es el tumor cardiaco benigno más frecuente en los niños, seguido por el teratoma y el fibroma con una frecuencia de entre 10-20%. (1)(2)(3) El pronóstico a mediano y largo plazo varía en función del crecimiento y grado de malignidad por lo que establecer el diagnóstico preciso representa un reto mediante estudios de imagen disponibles en la actualidad. No obstante, es imprescindible determinar la estirpe histológica para poder identificar el pronóstico del paciente.…”