A congenital medulloepithelioma of the midbrain

Abstract

IN the histogenetic classification of the gliomas proposed by Baileyand Cushing (1926) the most primitive tumour of the series, postulated as arising from the epithelium of the medullary plate, was described as medulloepithelioma. Bailey and Cushing were able to find only two tumours in their series which they could put under this heading. One arose from the post-infundibular region of the third ventricle, the other from the pineal-sites in which medullary-plate cells, characterised by an internal limiting membrane, persist into post-natal life (Benda, 1898). Both tumours consisted of more or less differentiated tubules of columnar cells, partly stratified and possessing internal and external limiting membranes. One of the tumours showed some formation of perivascular rosettes. I n neither did the cells contain cilia or blepharoplasts, though in the second case (p. 54) a diplosome was present in many cells.Since this first description and naming of the tumour very few further examples have been reported (Davie, 1932 ;Greenfield, 1934) and its existence has been called into question (Mabon et aZ., 1950).The primitive medullary epithelium begins to develop cilia at the end of the fifth week of embryonic life. Russell (1950) has pointed out that cilia persist into adult life and that the ciliated ependymal cell is in fact the mature form ; it is the primitive cell that is free of cilia. The very early stage at which cilia develop would explain the extreme rarity with which true medullary epithelium has been found.The tumour here described, occurring in an infant of eight months, raises once more the problem of definition of the medulloepithelioma, of which it is considered to be an authentic example. Case reportThe patient was a male infant aged 8 months a t death. He was born a t the Central Middlesex Hospital on 4th April 1955, at full term following a normal pregnancy. Deep transverse arrest developed during labour and delivery was completed with forceps. A hzematoma and forceps marks were present, a left posterior parietal fracture being noted in a skull radiograph. The birth weight was 3.3 kg. The circumference of the skull was 39.5 em. at 9 days, when he had a convulsion followed by persistent head retraction. He was eventually discharged to a convalescent home. Several fits and clonic convulsions were noted there, and he was therefore re-admitted to the Central Middlesex Hospital on J. PATH. BACT.-VOL. LXXIV (1957) 357 Z A classification of the tumors of the glioma group on a histogenetic basis with a correlated study of prognosis, Philadelphia. Dtsch. rned. Wschr., xxiv, 476.

Cerebral Medulloepithelioma With Long Survival -Case Report-

“…[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][42][43][44][45] Although most medulloepitheliomas manifest in the first decade of life, 1,11,13,15,22,25,28,31,39,40,…”

“…It remains unknown why in some cases the neural tube becomes a malignant tumor and why some individuals develop medulloepitheliomas after birth. These tumors may derive from neural tube-like tissue either as a result of neoplastic transformation at some stage of fetal or early postnatal development 11,32,43) or from the anomalous gene re-expression of early neural tube determinants. 24,46) Medulloepitheliomas at initial presentation generally appear as well circumscribed, iso-to slightly hypodense neoplasms on CT scans, 28,31) and as hypoor hyperintense tumors on T 1 -and T 2 -weighted MR images.…”

“…35) The high rate of recurrence due to rapid subarachnoid spread and radioresistance of the tumor cells results in a poor prognosis with overall median survival of 5 months. 1,3,4,6,9,11,13,21,22,25,28,32,35,38,[43][44][45]47) Long-term survivors had undergone gross total resection. 25,28,46) We report a case of medulloepithelioma with longterm survival of more than 6 years in a 13-year-old boy treated by gross total resection and removal of the surrounding brain tissue infiltrated by tumor cells.…”

Ectopic intrapelvic medulloepithelioma: case Report

“…Interestingly, biochemical study of NCAM isoforms performed on the lung metastases showed similar profiles to those seen in early development of the mouse neural tube (Boisseau et al, 1991). Maturation of cells along multiple divergent neuroepithelial lines is often encountered (Treip, 1957;Deck, 1969;Fowler, 1968;Karch & Urich, 1972;Gullotta & Entzian, 1975;Pollak & Friede, 1977;Scheithauer & Rubinstein, 1979;Bonnin et al, 1984;Sat0 et al, 1980)'but, as observed in our case, ependymal differentiation alone is rare (Fowler, 1968;Zimmerman, 1958) although it represents the maturation of the residual germinal cells next to the lumen of the primitive neural tube (Rubinstein, 1972).…”

Melanotic tumours (blue naevi) of spinal nerve roots

“…PLATES XLVI-XLVIII PIGMENTED tumours of the nervous system are not common. In the central nervous system they can be classified into three main types, (1)primarymalignant melanoma of the meninges (Russell and Rubinstein, 1971), (2) metastatic malignant melanoma and (3) melanotic variants of certain types of tumour including meningioma (Keegan and Mullan, 1962;Turnbull and Tom, 1963 ;Abbott, Killefer and Crandall, 1968 ;Scott, Ferrara and Peale, 1971), medulloblastoma (Fowler and Simpson, 1962) and medulloepithelioma (Treip, 1957). Melanin has also been identified in peripheral nerve sheath tumours (Mandybur, 1974), variously reported as melanotic schwannonia (Hodson, 1961 ;Shillitoe, 1965;Dastur, Sinh and Pandya, 1967;Theodossiou and Segditsas, 1971) and pigmented neurofibroma (Bird and Willis, 1969;Harkin and Reed, 1969).…”