Elsevier

Journal of Pediatric Surgery

Volume 45, Issue 12, December 2010, Pages 2312-2316
Journal of Pediatric Surgery

PAPS Papers
Concordance for neuroblastoma in monozygotic twins: case report and review of the literature

https://doi.org/10.1016/j.jpedsurg.2010.08.025Get rights and content

Abstract

The patients were infant male twins born by cesarean delivery following a healthy pregnancy at 36 weeks' gestation to unrelated parents. At 4 months of age, twin 2 presented with hepatomegaly and a right suprarenal mass. Resection of an adrenal tumor and a liver tumor biopsy were performed. Twin 1 had no symptoms at 4 months of age. Screening by abdominal ultrasonography showed multiple masses in the liver but no adrenal mass. Metaiodobenzylguanidine scintigraphy showed positive findings in multiple liver masses. A laparoscopic biopsy for a liver tumor was performed. All primary tumor and liver tumor specimens from twin 2 and the liver tumor of twin 1 had the same histologic classification of neuroblastoma and nearly identical genetic aberrations, including a chromosome gain or loss using array–comparative genomic hybridization. From these clinical and pathologic findings and genetic analyses, we strongly demonstrate the transplacental metastatic spread from twin 2 to twin 1. In the literature, 9 pairs of concordant twin neuroblastomas, including the current twin, have been presented; and the clinical findings of 5 twin pairs may represent placental metastases from one twin with congenital neuroblastoma to the other twin. This study is the first report presenting the possibility of twin-to-twin metastasis in monozygotic twins with neuroblastoma based on an analysis of the clinical features and genetic aberrations.

Section snippets

Clinical features and outcomes

The patients were infant male twins born by cesarean delivery following a healthy pregnancy at 36 weeks' gestation to unrelated parents. During the gestational course, the twins did not undergo exposure to extrinsic factors that might affect neuroblastomas. At 4 months of age, twin 2 presented with hepatomegaly and a right suprarenal mass (Fig. 1A-D). The patient's urinary vanillylmandelic acid (339 µg/mg Cr) and homovanillic acid (447 µg/mg Cr) were markedly elevated. Metaiodobenzylguanidine

Discussion

Neuroblastomas can be multifocal and can be concordant or discordant (only one of the twins has the tumor) among monozygotic twins. To the best of our knowledge, as shown in Table 1, only 9 sets of monozygotic twins concordant for neuroblastoma, including the current cases, have been described; and no concordant neuroblastoma cases have been described for dizygotic twins [4], [5], [6], [7], [8], [9], [10], [11].

Neuroblastomas can present in multiple ways. The most frequent presentation occurs

Acknowledgments

We thank Dr Tomoko Iehara, Pediatrics, Kyoto Prefectural University of Medicine, Kyoto, Japan, for the personal communication. The English used in this manuscript was reviewed by Brian Quinn (Editor-in-Chief, Japan Medical Communication).

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