Perinatology.  2018 Dec;29(4):189-194. 10.14734/PN.2018.29.4.189.

Mirror Syndrome Resulting from Metastatic Congenital Neuroblastoma to Placenta

• Park SH
• Namgoong J
• Ko KN
• Kim CJ
• Lee PR
• Jung E
• Lee BS
• Kim KS
• Kim EA

• KMID: 2430224
• DOI: http://doi.org/10.14734/PN.2018.29.4.189

Abstract

Congenital neuroblastoma is a rare disease. Placental metastasis is extremely rare and poor prognosis has been reported in neonates. Mirror syndrome could occur in mother with placental metastasis with possibilities of hypertension and edema. We report a case of detection of left suprarenal mass in fetus at 31⁺⁵ weeks' gestation. Mother presented with palpitation, edema, headache, and hypertension. Maternal 24 hours urine vanillylmandelic acid (VMA) and normetanephrine (NME) level at 34 weeks' gestation were elevated. Consequently, emergent cesarean section was done. Based on abdominal ultrasonography and whole body magnetic resonance imaging, left adrenal tumor with liver metastasis was suspected. Neuroblastoma was confirmed by liver and placenta biopsy. Chemotherapy was started with Pediatric Oncology Group 9243 at day 7 and changed into Children's Oncology Group 3961 due to cholestasis and poor response during 2nd cycle. Plasma exchange was done for aggravated direct hyperbilirubinemia. The baby expired at 73 days due to multi-organ failure. Maternal symptoms were completely resolved in 2 weeks after delivery along with normalization of the elevated level of VMA and NME. We report a first case of mirror syndrome in Korean mother and fetus resulting from metastatic congenital neuroblastoma to placenta.

Keyword

Neuroblastoma; Neonate; Metastasis; Placenta; Mother

MeSH Terms

Biopsy
Cesarean Section
Cholestasis
Drug Therapy
Edema
Female
Fetus
Headache
Humans
Hyperbilirubinemia
Hypertension
Infant, Newborn
Liver
Magnetic Resonance Imaging
Mothers
Neoplasm Metastasis
Neuroblastoma*
Normetanephrine
Placenta*
Plasma Exchange
Pregnancy
Prognosis
Rare Diseases
Ultrasonography
Vanilmandelic Acid
Normetanephrine
Vanilmandelic Acid

Figure

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  Fig. 1 (A) Fetal ultrasonography. 5.3×4.8 cm left suprarenal mass was detected at gestational age 31+5 weeks. (B) Whole body magnetic resonance imaging. Six cm sized left suprarenal encapsulated heterogenous mass and disseminated numerous small nodular lesions in enlarged liver was detected.

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  Fig. 2 (A) Nodular lesion and parenchymal hemorrhage in wedge resected liver. (B) Liver parenchyma is filled with small round hyperchromatic cells. Viable hepatocytes are entirely replaced by metastatic tumor cells (Hematoxylin and eosin, ×200). (C) The tumor cells are diffusely positive for CD56 immunohistochemical stainings (×400). (D) The tumor cells are focally positive for synaptophysin immunohistochemical stainings (×400). (E) Patchy tumor emboli in the chorionic villous capillaries (Hematoxylin and eosin, ×400). (F) Negative for the copy number change of the N-Myc gene by fluorescent in situ hybridization.

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  Fig. 3 Changes in maternal systolic and diastolic pressure before and after delivery. Maternal systolic blood pressure decreased from 151 to 128 mmHg after delivery. Maternal diastolic blood pressure decreased from 87 to 79 mmHg after delivery.

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  Fig. 4 (A) Maternal 24 hours urine normetanephrine level decreased from 3,964.5 µg at gestational age 34 weeks to 110 ug after delivery. (B) Maternal 24 hours urine vanillylmandelic acid level decreased from 20.1 mg at gestational age 34 weeks to 2.6 mg after delivery.

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