Clear cell ependymoma with a lipidized component that developed in the thoracic spinal cord

Abstract

The authors report a case of clear cell ependymoma with a lipidized component that developed in the thoracic spinal cord. A 59-year-old man was admitted to the hospital with an itchy pain in the left forearm to the left anterior and lateral chest for the past three years. Neurological findings on admission included dissociated sensory disturbance below the C8 level and increased deep tendon reflex in both lower extremities. An MRI scan of the spinal cord revealed an intramedullary tumor with a longer diameter of 3.5 cm at the T3-T4 level and a distended syrinx at the T2-T3 level. Surgery was performed after T1-T5 laminectomy. The gray, soft and well demarcated tumor was removed subtotally. Light microscopy revealed a portion where clear cells proliferated and a portion where foamy cells proliferated. In some tissue, there were a very few nuclear areas suggestive of a perivascular pseudorosette. Neither nuclear division nor necrosis was observed. Immunohistochemically, the tumor cells were positive for glial fibrillary acidic protein (GFAP), epithelial membrane antigen (EMA), vimentin, and negative for cytokeratin, synaptophysin. The MIB-1 staining index was 0.25%. Based on these findings, diagnosis of clear cell ependymoma with a lipidized component was made.

Clear Cell Ependymoma of the Temporal Lobe in a Child: A Case Report

“…There were no anaplastic features in the other reported cases. However, Min and Scheithauer [2] reported that the biological malignancy of CCE was the same as that of other ependymomas and varied with individual cases, and that the MIB-1 staining index was required for evaluation of malignancy [8]. In our case, the proliferation index was 5% at the lowest point and 10% at the highest by MIB-1, and with this finding the tumor was suggested to have a tendency to anaplasia.…”

“…Some authors [21, 22] did not observe any EMA-positive reaction, whereas some authors reported EMA-positive ependymomas [1, 5, 23, 24]. Neuronal markers including synaptophysin, neurofilaments, neuron-specific enolase and chromogranin A are usually negative [8,9,10]. However, some cases have neuron-specific-enolase-positive tumor cells [3, 4, 11].…”

“…microvilli, cilia, junctional complexes and microrosettes. However, even in a case in which no electron-microscopic specimens have been prepared, Kim et al [8] suggested that diagnosis can be made based on light-microscopic findings, if it is positive for GFAP and EMA. In such a case, the presence of EMA-positive microvesicular and granular structures is important [8].…”

“…However, even in a case in which no electron-microscopic specimens have been prepared, Kim et al [8] suggested that diagnosis can be made based on light-microscopic findings, if it is positive for GFAP and EMA. In such a case, the presence of EMA-positive microvesicular and granular structures is important [8]. Hirato et al [15] pointed out that these EMA-positive structures under the light microscope were consistent with intracytoplasmic lumina containing microvilli and intercellular microrosettes under the electron microscope.…”

Differentiation of Clear Cell Ependymoma of the Cerebellum From Hemangioblastoma by Thallium-201 Single Photon Emission Computed Tomography -Case Report-

“…[1][2][3][4][5][7][8][9][10][11][12][13][14][16][17][18][19][20][23][24][25][26][27][28] CCE seems to have a predilection for the supratentorial region (supratentorial, 42; infratentorial, 16; spinal, 4) in contrast to the classical subtypes of ependymomas. The recurrence rate of CCE in all locations was about 60% during the follow-up period, [1][2][3][4][5][7][8][9][10][11][12][13][14][16][17][18][19][20][23][24][25][26][27][28] which is higher than other subtypes of ependymoma. 15) The clinical course of CCE was the worst among benign ependymomas, with a significantly higher expression of M...…”