Medulloepithelioma
Posterior
cerebral fossa medulloepithelioma: report of a case. The axial T1 (a), T2 W/FLAIR (b) image shows cerebellar vermis lesion that was hypointense on T1WI and heterogeneous on T2WI, vith perilesional edema, and triventricular hydrocephalus
Successful
treatment of ciliary body medulloepithelioma with intraocular melphalan chemotherapy: a case report. a. Anterior segment chamber photography of the right eye at diagnosis showing iris neovascularization, multiple free-floating cysts in the pupillary area as well as a subluxated and notched lens. b. Ultrasound biomicroscopy (35 MHz) on the 6 o’clock meridian displaying an iridocorneal angle closure and an irregular-surfaced mass with intratumoral cysts adjacent to the ciliary body, growing from the ciliary epithelium into the posterior chamber without invading the ciliary muscle. Clumps of hyperechogenic material are found in the posterior chamber (cysts) and the anterior vitreous (dust). c. Cytology analysis of the aqueous tap performed before treatment showing aggregates of malignant small cells with high nuclear/cytoplasmic ratio and finely granular chromatin. d-e. Anterior segment photography and ultrasound biomicroscopy one month after 3 intracameral and 3 intravitreal melphalan injections showing complete response of the primary tumor and the aqueous/vitreous seeding. Iris neovascularization had transiently regressed after concomitant intravitreal anti-vascular endothelial factor and cryoablation of the peripheral ischemic retina
Peripheral
medulloepithelioma: a rare tumor with a potential target therapy. A: CT scans showing the tumor at diagnosis right hydronephrosis is also present; scans after 4 courses of CT, demonstrating partial response to therapy; C: recurrence of the tumor after six months from stop therapy. CT at diagnosis (A), after chemotherapy (B) and at relapse (C).
Atypical
clinical presentation and long-term survival in a patient with optic nerve medulloepithelioma: a case report. Clinical presentation and computed tomography images of a 12-year-old Caucasian boy. (A) His right optic nerve appears elevated and enhanced on computed axial tomography (arrow). His left optic nerve appears normal. (B) A well-defined whitish mass at the optic disc (arrow) with tortuous and dilated retinal vessels (arrowhead) by fundoscopy.
Medulloepitheliomas are rare, highly malignant, primitive embryonic tumor derived from the primitive medullary plate and neural tube. It is essentially a tumor of childhood with the age of onset between 6 months and 5 years.
Pathology
Medulloepitheliomas are classified as embryonal tumors in the WHO classification of CNS tumors .
Radiographic features
On non-contrast CT scan, the lesion is either isodense or hypodense with variable heterogeneity and calcification. The tumor enhances with contrast.
Treatment and prognosis
Medullary epithelioma carries dismal prognosis with a median survival of 5 months. On the other hand, medulloepitheliomas occurring in the eye or the orbit generally benefit from a gross-total resection and may present a good prognosis.
