medulloepithelioma
Pronunciation: meh-dul-oh-ep-ih-thee-lee-OH-mah
Rare primitive neuroectodermal tumor typically occurring in young children, resembling embryonic neural tube.
Full Definition
Medulloepithelioma is an extremely rare primitive neuroectodermal tumor that histologically resembles the embryonic neural tube or medullary epithelium. These tumors most commonly arise in the central nervous system of infants and young children, particularly in the cerebral hemispheres, brainstem, or spinal cord. Histologically, they are characterized by true rosettes or tubular structures lined by pseudostratified columnar epithelium, similar to the developing neural tube. The tumor may be classified as teratoid if it contains mesenchymal elements. Medulloepitheliomas are highly malignant with a poor prognosis, and complete surgical resection is often challenging due to their location and infiltrative nature.
Usage
Usage note: Specify teratoid subtype when mesenchymal elements are present. Extremely rare; confirm diagnosis with expert consultation.
In Context
- "The tumor displayed characteristic flexner-wintersteiner-like rosettes consistent with medulloepithelioma." — Neuropathology report
- "Immunohistochemical studies supported the diagnosis of teratoid medulloepithelioma with both neural and mesenchymal components." — Pediatric pathology consultation