Fetal neuroblastoma with fetal hypertension

Neuroblastomas are one of the most common solid tumors found in the fetus and newborn and account for 50% of neonatal malignancies1; they can be identified by prenatal ultrasonography. Catecholamines, secreted by neuroblastomas, may alter fetal cardiovascular structure. We report a case of adrenal neuroblastoma with changes in fetal heart structure diagnosed by fetal echocadiography. To the best of our knowledge, this is the first report of fetal neuroblastoma with fetal hypertension. A 30-year-old woman was referred at 37 weeks' gestation for fetal echocardiography after ultrasound screening at a local clinic had revealed marked dilation of both atria. Fetal echocardiography revealed the dimensions of the left and right atria to be 27.1 and 19.3 mm, and those of the left and right ventricles to be 17.6 and 18.2 mm, respectively. Severe mitral valve regurgitation (MVR) and severe tricuspid valve regurgitation (TVR) were noted on color Doppler ultrasound imaging. The peak MVR and TVR velocities, as measured by continuous-wave Doppler ultrasound, were 4.57 and 4.04 m/s, respectively, and the maximum pressure gradients of MVR and TVR were 83.7 and 65.3 mmHg, respectively (Figure 1). As the pulmonary artery and the aorta appeared to be normal, systolic systemic arterial pressure (SAP) and systemic pulmonary arterial pressure (SPAP) were calculated by adding the peak MVR gradient to the estimated left atrial pressure, and the peak TVR gradient to the estimated right atrial pressure. Left and right atrial pressures were estimated at 5 mmHg. Accordingly, SAP and SPAP were calculated to be 89 and 70 mmHg, respectively. From findings on fetal echocardiography and elevated SAP and SPAP levels, a suspected diagnosis of fetal hypertension of unknown origin was made. Fetal echocardiograms showing (a) mitral valve regurgitation (MVR) and (b) tricuspid valve regurgitation (TVR). On continuous-wave Doppler, the peak velocities of MVR and TVR were 4.57 m/s and 4.04 m/s, respectively, and the maximum pressure gradients of MVR and TVR were 83.7 mmHg and 65.3 mmHg, respectively. LA, left atrium; LV, left ventricle; MR, mitral regurgitation; RA, right atrium; RV, right ventricle; TR, tricuspid regurgitation. Further ultrasound imaging revealed a 5.3 × 4.3-cm tumor in the left adrenal gland that was highly echogenic and homogeneous in appearance. Color Doppler imaging of the tumor revealed rich peripheral vascularization and moderate diffuse intratumoral flow signals. The left kidney was displaced and distorted by the suprarenal tumor (Figure 2). On the basis of these findings, fetal neuroblastoma with fetal hypertension was diagnosed, and the parents elected to terminate the pregnancy at 38 weeks' gestation. Findings at autopsy confirmed the prenatal diagnosis. (a) Grayscale ultrasound image showing a homogeneously highly echogenic tumor in the left adrenal gland and measuring about 5.3 cm × 4.3 cm. The left kidney was displaced and distorted by the suprarenal tumor. (b) Color Doppler image showing the tumor with rich peripheral vascularization and moderate diffuse intratumoral flow signals. LK, left kidney. The incidence of secondary systemic hypertension from neuroblastoma is 27%2. Neuroblastoma cells secrete catecholamines, which can increase vascular resistance, decrease the circulating plasma volume, cause redistribution of blood flow, lead to hypertension and induce cardiomyopathy3, 4. Nevertheless, catecholamine-induced cardiomyopathy has rarely been reported5-9. In our case, fetal SAP and SPAP were 89 and 70 mmHg, respectively, which were higher than expected for a neonate. This finding may be explained either by the high levels of circulating catecholamines produced by the neuroblastoma, or by compression of the renal artery by the bulky neuroblastoma causing renovascular hypertension. The severe MVR and TVR may have resulted from the severe hypertension or the severe cardiomegaly that may accompany enlargement of the mitral and tricuspid annulus. In summary, ultrasound examination may be useful for locating the cause of fetal hypertension, and assessing fetal heart status in cases of neuroblastoma could help to facilitate a correct prenatal diagnosis. L. Cun*, M. Zhe*, Z. Xinfeng*, T. Guowei*, L. Shaoping*, L. Chuanxi , * Department of ultrasound, Qilu Hospital of Shandong University, No. 107#, Wenhua Xi Road, Jinan, Shandong, People's Republic of China, Department of ultrasound, Shandong Provincial Hospital of Shandong University, Jinan, People's Republic of China