Presacral Medulloepithelioma—de Novo or Teratomatous?

Abstract

Medulloepitheliomas are rare, childhood embryonal multipotential neuroepithelial tumors [1] involving either central nervous system (cerebrum, cerebellum, spinal cord) or the ciliary body of the eye. These are aggressive tumors, although intraocular tumors have excellent prognosis due to complete excision [2]. Peripherally, medulloepitheliomas may be a component of a malignant teratoma, or more rarely, as a primitive neuroectodermal tumor histologically distinct from Ewing's sarcoma. Two presacral medulloepithelioma's have been described [3,4], both have been localized at presentation. We report a medulloepithelioma presenting as a presacral mass with multiple liver and a solitary lung metastases.A 3-year-old boy presented with a 2-day history of fever, lethargy, and difficulty breathing. He had increasing constipation for 10 days. On physical examination, he had moderate hepatomegaly (span = 12 cm) with abdominal distention (abdominal circumference = 50 cm), but no other mass palpable in the testis or abdomen. Complete blood count, urinalysis, and chest x-ray were normal. Urinary vanillylmandelic acid (VMA) excretion,