Biomedical subjects
Publications and source records attributed to W Höpping.
At present there is no satisfactory classification or staging system. In order to compare the Reese classification (Reese, 1963), the Essen classification (Höpping, 1983) and a new classification, a method of evaluating different parameters was created in order to get an optimal classification of survival.
Sections of 51 surgically enucleated eyes from cases of retinoblastomas were examined immunohistochemically to delineate patterns of cellular differentiation. Employing the avidin-biotin immunoperoxidase technique, antibodies were used against glial fibrillary acidic protein (GFAP), S-100 protein and neuron specific enolase (NSE). Areas of uninvolved retina and/or optic nerve were used as built-in positive control. Most of the tumors showed GFAP and S-100 protein-positive perivascular glial cells that were interpreted as reactive astrocytes. In three well-differentiated retinoblastomas, glial cells were found to be interspersed randomly among tumor cells and not associated with blood vessels. These glial cells were interpreted as neoplastic based on their distribution pattern and cytologic features. In about half of the tumors, the retinoblastoma cells stained positively for NSE, indicating their neuronal tumor cells that stained positively for NSE but failed to show any evidence of photoreceptor differentiation. Based on these observations and similar differentiation patterns described in other primitive neuroectodermal tumors of the brain, it is suggested that retinoblastoma cells can differentiate not only into photoreceptor cells but also along other neuronal cell lines and rarely into glial cells.
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A 6-month old girl had unilateral leukokoria. Because retinoblastoma could not be excluded, the blind left eye was enucleated. Histopathologically, a total retinal detachment associated with a widespread cavernous hemangioma of the retina and a preretinal membrane were found. The specimen was further examined by immunohistochemistry and electron microscopy to determine the origin of the preretinal membrane and to study the anatomy of the telangiectatic retinal vessels. The preretinal membrane was mostly composed of spindle-shaped cells that demonstrated immunohistochemically the presence of glial fibrillary-acidic protein and showed, ultrastructurally, numerous glial filaments in their cytoplasm. These findings support the view that this membrane was of glial origin. By electron microscopy, the telangiectatic retinal vessels displayed the anatomic features normally encountered in retinal vessels, which accounts for the absence of exudates observed clinically and angiographically in cases of cavernous hemangioma of the retina.
A total of 60 eyes with retinoblastoma were examined by echography using the A-scan (Kretz 7200 MA) and B-scan (Ocuscan 400) at different sensitivity levels. The results documented by polaroid photographs were analysed quantitatively. Some additional diagnostic criteria were obtained. The differential diagnosis of a broad spectrum of vitreoretinal diseases is discussed.
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In 47 patients (46 children and 1 adult) with unilateral and bilateral retinoblastoma, the titer of the carcinoembryonic antigen (CEA) was determined. The CEA titer of children with active retinoblastomas was 1.44 +/- 0.26 ng/ml (x +/- SEM). Those patients whose retinoblastoma was inactivated by therapy did not show a significantly lower CEA titer. In the group of children with one eye removed because of a unilateral retinoblastoma, the CEA titer was significantly (P less than 0.1) lower. The globes of 25 children were examined histologically. In those cases with invasion of the optic nerve or choroid, the CEA titer was significantly higher (P less than 0.1) as compared with those where the tumor was limited to the retina alone.
18 patients of 22 lethal cases of retinoblastoma died of recurrence or metastases of the primary tumor, which were mostly located in the head. Two clinical groups can be distinguished. In the patients with recurrence from the side of the enucleated eye, the mean survival time was 26 months, whereas the four children with a relaps originating in the conservatively treated eye survived 23 to 65 months. Two other children died of secondary tumors, and two of unrelated disease.
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