Successful treatment of non-midline primary malignant germ cell tumors with yolk sac components in neonates: report of 2 cases

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  • 1 Division of Pediatric Neurosurgery, Department of Surgery, Sainte-Justine Hospital, Montreal;
  • 2 Departments of Pediatrics and Human Genetics, McGill University and McGill University Health Centre, Montreal;
  • 3 Division of Neurosurgery, Department of Pediatric Surgery, Montreal Children’s Hospital, McGill University Health Centre, Montreal;
  • 4 Departments of Radiology and
  • 5 Pathology, Montreal Children’s Hospital, Montreal; and
  • 6 Departments of Pediatrics and Human Genetics, Montreal Children’s Hospital, McGill University and McGill University Health Centre, Montreal, Quebec, Canada
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Here, the authors present 2 cases of nongerminomatous germ cell tumor (NGGCT): a neonate with a mixed malignant germ cell tumor, 5% yolk sac tumor (YST) and 95% immature teratoma components, originating from the right mesial temporal lobe; and a 2-month-old infant with a pure YST originating from the left middle cranial fossa. These tumors with yolk sac components, which are thought to have a poor prognosis, were successfully treated with complete tumor resection alone and subtotal tumor resection with chemotherapy, respectively. Event-free survival exceeds 5 years for each patient even though neither received radiotherapy. The authors highlight the role of radical surgery and the successful treatment of neonatal YST with aggressive resection (and chemotherapy in 1 case) while avoiding radiation therapy. They also report the very rare non-midline location of these neonatal NGGCTs and emphasize the importance of considering YSTs and mixed NGGCTs with YST components in the differential diagnosis of non-midline hemispheric or skull base tumors in newborns.

ABBREVIATIONS AFP = alpha-fetoprotein; beta-HCG = beta–human chorionic gonadotropin; EFS = event-free survival; GCT = germ cell tumor; GTR = gross-total resection; NGGCT = nongerminomatous GCT; RT = radiation therapy; STR = subtotal resection; YST = yolk sac tumor.

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Contributor Notes

Correspondence Roy W. R. Dudley: McGill University Health Centre, Montreal, QC, Canada. roy.dudley@mcgill.ca.

INCLUDE WHEN CITING Published online October 30, 2020; DOI: 10.3171/2020.6.PEDS19719.

A.G.W. and N.M. contributed equally to this article.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

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