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A Catecholamine-Secreting Neuroblastoma Leading to Hydrops Fetalis

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A Catecholamine-Secreting Neuroblastoma Leading to Hydrops Fetalis Journal of Perinatology (2014) 34, 405–407 & 2014 Nature America, Inc. All rights reserved 0743-8346/14 www.nature.com/jp PERINATAL/NEONATAL CASE PRESENTATION A catecholamine-secreting neuroblastoma leading to hydrops fetalis T Inoue1,YIto1, T Nakamura1, K Matsuoka2 and H Sago1 A case of fetal neuroblastoma of the right adrenal gland, with rapid development of hydrops fetalis due to catecholamine-induced cardiomyopathy, is reported. A fetus with a right suprarenal mass detected during ultrasonography at 32 weeks gestation progressively developed into hydrops fetalis by 35.2 weeks gestation. An emergent cesarean section was performed. At birth, the female neonate was hypertensive, with markedly elevated catecholamine levels; echocardiography showed poor contractility. Morphine, human atrial natriuretic peptide, milrinone, nitroprusside and dobutamine were initiated and her blood pressure was maintained within the normal range and her cardiac contractility improved 2 weeks after birth. Neuroblastoma cells were detected in the placenta, resulting in the right adrenal mass being diagnosed as a neuroblastoma. She was well, and the mass diminished in size within 4 months, without surgery. A fetus with suspected neuroblastoma, indicated by a suprarenal mass, should be managed with appropriate consideration of hydrops. Journal of Perinatology (2014) 34, 405–407; doi:10.1038/jp.2014.19 Keywords: congenital neuroblastoma; catecholamine-induced cardiomyopathy; placental metastasis; paroxysmal hypertension INTRODUCTION Upon delivery, the female neonate had generalized edema and Neuroblastomas constitute 25% of congenital malignancies,1 and weighed 3268 g, with 1 and 5 min Apgar scores of 8; she was in some secrete catecholamine, causing hypertension. Catechola- respiratory distress, necessitating mechanical ventilation. The baby mine excess can cause oxygen free radical development, calcium was hypertensive, with a blood pressure (BP) of 76/47/59 mm Hg overload in the heart, downregulation of cardiac b-adrenergic (systolic/diastolic/mean). Cardiac arrhythmia was absent, and her À 1 receptors and myofibril loss, all of which lead to myocyte injury,2 hemoglobin level was 11.7 g dl . An echocardiography showed which is described as ‘catecholamine-induced cardiomyopathy’. normal cardiac structure and a patent ductus arteriosus; her left Hydrops fetalis results from fetal cardiac failure owing to various ventricular ejection fraction was low (46%). Her plasma and mechanisms, including fetal cardiomyopathy. Excessive catecho- urinary catecholamine and metabolite levels were markedly lamine secretion from a fetal neuroblastoma may lead to fetal increased, without an elevation in adrenocorticotropic hormone, cardiomyopathy and results in hydrops fetalis. However, previous cortisol, renin activity or aldosterone (Table 1). We speculated that reports of this have not been described. her hypertension and heart failure resulted from sustained fetal Here we report a neonate who developed hydrops fetalis catecholamine elevation. because of catecholamine-induced cardiomyopathy, secondary to A continuous infusion of morphine was started after birth, and a fetal catecholamine-secreting neuroblastoma. beginning on day 1, human atrial natriuretic peptide, milrinone and nitroprusside were started to treat the patient’s hypertension; dobutamine was added to treat low cardiac function on day 2. The patient’s mean BP began to fluctuate between 20 and 70 mm Hg CASE for 12 h and then gradually normalized (Figure 2). Dobutamine A 33-year-old, primiparous Japanese woman with an unremark- infusion was discontinued on day 3 but episodes of periodic able personal or family medical history was referred to our paroxysmal hypertension recurred on days 4 and 5. As the hospital because of a fetal, right, suprarenal mass detected during patient’s BP normalized, we discontinued the administration of a routine, prenatal ultrasonogram at 32 weeks gestation. A solid, nitroprusside, morphine and human atrial natriuretic peptide by 3-cm diameter mass was detected on the anterior and superior day 6. Thereafter, the infant’s BP stabilized and her cardiac pole of the fetus’ right kidney (Figure 1). Although hydrops was contractility improved. On day 14, we discontinued milrinone. not evident at 34.0 weeks gestation, hydrops fetalis (ascites, Scintigraphy, using metaiodobenzylguanidine, showed a cold pleural effusion and edema) and cardiomegaly (cardiothoracic spot on the right suprarenal mass but no other metastatic spots. area ratio, 46%) developed progressively by 35.2 weeks gestation; Placental histopathology revealed several neuroblastoma cells in a the fetus was delivered by emergent cesarean section. The blood small, angiomatous tissue section of the placenta. The neuro- gas analysis of blood from the umbilical artery revealed a pH of blastoma cells were diagnosed as a poorly differentiated subtype, 7.289, a CO2 partial pressure of 47.6 mm Hg, an O2 partial pressure without N-myc amplification. On the basis of the patient’s of 16.1 mm Hg, a HCO3 À concentration of 22.8 mmol l À 1 and a biochemical data, clinical course and placental pathology, we base excess of À 3.5 mmol l À 1. speculated that the catecholamine-induced cardiomyopathy 1Center for Maternal–Fetal and Neonatal Medicine, National Center for Child Health and Development, Tokyo, Japan and 2Department of Pathology, National Center for Child Health and Development, Tokyo, Japan. Correspondence: Dr Y Ito, Center for Maternal–Fetal and Neonatal Medicine, National Center for Child Health and Development, 2-10-1 Okura Setagaya-ku, Tokyo 157-8535, Japan. E-mail: [email protected] Received 20 October 2013; revised 11 December 2013; accepted 13 January 2014 Neuroblastoma leading to hydrops fetalis T Inoue et al 406 resulted from a sustained elevation of catecholamine secreted by The patient’s right adrenal mass did not require surgical the right adrenal neuroblastoma, which led to hydrops fetalis and intervention because her neuron-specific enolase and catechola- newborn hypertension. mine levels were normalizing and the tumor was poorly differentiated, without N-myc amplification. The patient was discharged on day 57; she was well and the tumor had decreased in size by 4 months of age. DISCUSSION We believe this is the first report describing a neonate who developed hydrops fetalis because of catecholamine-induced cardiomyopathy secondary to a fetal catecholamine-secreting neuroblastoma. The patient’s suprarenal mass was not histologi- cally evaluated but neuroblastoma was diagnosed through a histological examination of the placenta. A previous report emphasized the importance of placental examinations in cases of congenital tumors because the placenta is readily available at birth and the procedure is noninvasive.3 Furthermore, placental Figure 1. Fetal neuroblastoma of the right adrenal gland. (a) Trans- examination enabled our patient to avoid surgery as the histology verse ultrasonography showing the prenatal appearance of the suggested a good prognosis. neuroblastoma on the anterior and superior pole of the right kidney. The possible mechanisms of hydrops fetalis with neuroblastoma The neuroblastoma was a solid, 3-cm diameter mass. (b) Coronal are (1) invasion of erythropoietic tissue by tumor cells, resulting T1-weighted magnetic resonance image of the patient showing in severe fetal anemia;4 (2) mechanical compression of the vena the right, round, suprarenal mass displacing the right kidney 4,5 downward. The mass was well defined and heterogenous; the rim cava by the primary tumor or enlarged liver; (3) blood flow impedance by tumor cells in the hepatic and placental of the lesion was isointense and the central portion was of low 4 intensity. (c) Coronal, T2-weighted magnetic resonance image vasculature; (4) intrauterine arrhythmia and ductal arteriosus showing the low-intensity rim of the lesion and the high-intensity constriction caused by excessive catecholamine secretion, both central portion. leading to fetal heart failure;4,5 (5) hypersecretion of aldosterone, Table 1. Laboratory data Day 0 Day Day Day Day Day Day Day 5 11 27 33 46 175 265 a-fetoprotein (14 000–300 000 ng ml À 1) 63 711 227.6 Neuron-specific enolase (o10 ng ml À 1) 111 31.3 30.3 30.9 16.8 Urine vanillylmandelic acid/creatinine (1.2–4.9 mgmgÀ 1 81.85 65.98 86.09 20.46 10.47 creatinine) Urine homovanillic acid/creatinine (1.6–5.5 mgmgÀ 1 creatinine) 87.19 55.75 56.52 23.02 13.58 Adrenaline (100–400 pg ml À 1) 517 p563 Noradrenaline (o70 pg ml À 1) 311 770 1714 442 Dopamine (o30 pg ml À 1) 6523 82 97 Urine metanephrine (0.01–0.07 mg per day) o0.01 0.01 Urine normetanephrine (20–150 mg per day) 650 230 Plasma renin activity (8.8±8.7 ng ml À 1 l À 1)16 Aldosterone (62.7±48.5 ng dl À 1) 65.3 Adrenocorticotropic hormone (25.8±12.0 pg ml À 1) 82.9 21.2 Cortisol (12.8±7.1 mgdlÀ 1) 33 5.18 Brain natriuretic peptide (o18.4 pg ml À 1) 6652 14.9 Normal values are shown in parentheses. Levels of catecholamines and neuron-specific enolase were normalizing. 150 Systolic BP Diastolic BP ( mmHg ) 0 100 Mean BP ( mmHg ) 0 60 minutes Figure 2. Periodic changes in blood pressure (BP) on day 2. The duration of these changes was 12 h, with a period of 20 min. The mean BP was 20–70 mm Hg. Journal of Perinatology (2014), 405 – 407 & 2014 Nature America, Inc. Neuroblastoma leading to hydrops fetalis T Inoue et al 407 induced by tumor encroachment or irritation of the fetal adrenal a cause for frequent patient monitoring because a catecholamine- cortex;4 and (6) cardiomyopathy resulting from sustained secreting neuroblastoma may lead to hydrops fetalis. catecholamine elevation secreted from a fetal neuroblastoma. The present patient did not have severe anemia, a mass effect due to the tumor, liver metastasis or hyperaldosteronism. Furthermore, CONFLICT OF INTEREST at birth, the ductal arteriosus was patent and arrhythmia was not The authors declare no conflict of interest. detected before or after birth. Placental blood flow was also unlikely to have been interrupted because she did not have significant placental metastasis.
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