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,

High-grade neuroepithelial tumor with medulloepithelioma-like areas out of the central nervous system in an infant with hemihypertrophy: a unique association

“…Detailed search for simultaneous presence of inguinal and coccygeal masses has also led us to focus on a few cases of peripheral medulloepithelioma (Table I a and b). [2][3][4][5][6][7][8][9][10] Medulloepithelioma is a rare embryonal tumor characterized by tubular and papillary patterns like primitive epithelium of the neural tube and medullary plate. It occurs usually in the eye or in the central nervous system and peripheral location has been reported quite rarely.…”