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Monozygotic twins and metastasis of neuroblastoma twin-to-twin through foetoplacental circulation

Introduction: Neuroblastoma is the common childhood solid tumor of neural crest cells. It may arise at any site in the sympathetic nervous system, most commonly in the adrenal medulla. We report a case of congenital neuroblastoma in a pair of monozygotic twins which is rare and only few cases have been described. Clinical History:The patients were two baby girls born at 37 week’ gestation following a riskfull pregnancy. There was no significant family history and parents had one older healthy child. The twins were born asymptomatic. The first twin was admitted to our hospital at the age of 10 days with marked abdominal distension and firmness, weight loss and hiperbilirubinemia. Abdominal ultrasound showed large liver with numerous hyperchoic nodules. A needle core biopsy of the liver revealed a histological and immunohistochemical diagnosis of neuroblastoma. The tumor is composed of small blue cells partially arranged in Homer-Wright pseudorosettes. Immunohistochemicaly tumor cells were positive for NSE and synaptophysin, but negative for LCA. Treatment commenced with standard chemotherapy protocol but soon the therapeutic course was complicated with respiratory symptoms and liver failure. After 20 days of therapy she died at age of 1 month. Autopsy showed icteric skin, general edema, well defined nodular mass in left adrenal gland measuring 2.5x2.5x2 cm and large liver with multiple nodules. A few days after her death, twin sister was admitted to hospital with similar symptoms. An extensive physical, laboratory and radiological examination and liver biopsy confirmed diagnosis of metastasis neuroblastoma in the liver. Treatment commenced with chemotherapy and after two cycles, the therapeutic course was complicated with liver failure. The biopsy specimen showed extensive toxic damage of liver with fibrosis and hemosiderosis. At 6 months of age she died. Autopsy showed postnecrotic cirrhotic changes in liver without metastasis, hypersplenism, diffuse alveolar damage of lungs and ascites. Conclusion: This is a rare case of metastasis neuroblastoma from twin-to-twin through foetoplacental circulation in utero that is confirmed at autopsy. First twin had primary tumor in adrenal gland with liver metastasis while second twin had liver metastasis without a primary tumor site.

monozygotic twins; neuroblastoma; twin-to-twin metastasis

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