An immunohistochemical study of the primitive and maturing elements of human cerebral medulloepitheliomas

Abstract

Four examples of human cerebral medulloepithelioma were studied immunohistochemically with a panel of antibodies and antisera to neuronal and glial proteins. The tumors, in addition to primitive medullary epithelium, contained areas of neuroblastic, ganglionic, astrocytic, ependymoblastic and ependymal differentiation, and in one tumor, areas resembling polar spongioblastoma. Tumor cells throughout the primitive medullary epithelium displayed focal immunoreactivity for vimentin, glial fibrillary acidic (GFA) protein and for the neuron-associated class III beta-tubulin isotype. Neuroblasts showed immunoreactivity for the class III beta-tubulin isotype, microtubule-associated protein 2 and neuron-specific enolase. Immunoreactivity for neurofilament epitopes and synaptophysin was detected in areas of ganglionic differentiation and coincided with the demonstration of neurofibrils in Bielschowsky's silver impregnations. Vimentin was the only marker detected in ependymoblastic and ependymal rosettes or in areas of polar spongioblastoma, as well as in mesenchymal cells. The results indicate that, even in very primitive neoplastic neuroepithelium, immunocytochemical evidence of early commitment of some of the cells to a neuronal or glial lineage can be demonstrated. The neuron-associated class III beta-tubulin isotype appears to be one of the earliest markers indicative of neuronal differentiation in normal and neoplastic primitive neuroepithelium.

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,…”

“…25,38) These tumors tend to be fatal despite treatment by surgery and/or irradiation and/or chemotherapy. 1,6,10,11,21,22,28,31,38,40,44) On the other hand, intraorbital, 7,8,15,30) peripheral, 26) and cauda equina 22) medulloepitheliomas have a much less malignant course and even those treated primarily or only by enucleation tend not to recur for a long time. 7,15,19,27) Extracranial and intracranial medulloepitheliomas have similar histological features, 8,30) and the great difference in the prognosis remains to be elucidated.…”

“…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.…”

Class III β‐tubulin in human development and cancer

“…Class I (Constitutive; Human h␤1) ␤I EEEEDFGEEAEEEA Class II ('Major brain' or 'major neuronal') ␤II DEQGEFEEEEGEDEA Class III ('Minor brain' or 'minor neuronal'; Human h␤4) ␤III EEEGEMYEDDDEESEAQGPK Class IVa ('Brain specific'; Human ␤5) ␤IVa EEGEFEEEAEEEVA Class IVb ('Major testis'; Human h␤2) ␤IVb EEEGEFEEEAEEEVA Class V ('Avian specific'; Chick c␤5) ␤V NDGEEAFEDEDEEEEINE Class VI ('Hematopoietic') ␤VI GLEDSEEDAEEAEVEAEDKDII al., 1982;Dietrich et al, 1987;Burgoyne et al, 1988;Caccamo et al, 1989a;Moody et al, 1989;Lee et al, 1990a,b;Easter et al, 1993;Katsetos et al, 1993;Ludueña, 1998;Farina et al, 1999;Modig et al, 1999]. However, ␤III protein is not detectable in the developing or mature Xenopus nervous system.…”

“…␤III is present in the "primitive medullary neuroepithelium" and foci of divergent neuronal differentiation in the human medulloepitheliomas [Caccamo et al, 1989a], as well as in the spontaneous murine ovarian teratomas with divergent neuronal differentiation [Caccamo et al, 1989b], and in the transplantable OTT-6050 teratoma [Caccamo et al, 1989c]. ␤III is absent in cells exhibiting glial differentiation [Caccamo et al, 1989a, b].…”