Supratentorial ependymoma
Updates to Case Attributes
Our patient was found to have signs of raised intracranial pressure, an unusual feature for a supratentorial ependymoma, which usually presents with headache, seizures or/and focal neurological deficits.
The imaging findings were typical for an ependymoma: On CT the mass was isodense relative to gray matter, contained both (micro)calcifications and cystic components. On MRI the mass's solid component demonstrated isointensity relative to gray matter on T1WI (not shown) and hyperintensity on T2WI with the heterogeneity of enhancement post gadolinium.
This patient's histologically confirmed papillary ependymoma did not involve its spinal cord. Ependymoma's grading is reported to be less useful, but as per our pathologists, “increase proliferative activity” lesions have been associated with worse outcome.
Supratentorial ependymomas - rare primary tumours of the central nervous system - are thought to arise from the lining of cerebral ventricles or the spinal cord central canal. However, recent studies suggest that radial glial stem cells are the cell of origin. Surgical resection and radiotherayradiotherapy are the top main treatment modalities.
Prior to resection our patient's mass was endoscopically biopsied; results came back inconclusive between an ependymoma and a papillary tumor of the pinal region (PTPR). Subsequently, the mass was partly excised and histopathologically then confirmed it to be an “ependymoma. papillary variant, with increased proliferative activity”.
HISTOLOGY
MICROSCOPIC DESCRIPTION:
Specimen A: Tiny fragments of grey matter. No tumor.
Specimen B and C: Moderately cellular tumor composed of isomorphic elongated tumor cells with oval euchromatic nuclei arranged in sheets, with perivascular pseudorosettes, occasional true ependymal rosettes, and true papillae covered by polarized ciliated tumor cells. No microvascular proliferation, no necrosis. Approx.2 mitoses/10 HPF.
IMMUNOPHENOTYPE:
- GFAP: very focally positive in perivascular cell procesesses
- EMA: prominent apical staining on surface of true papillae as well as focal dot-like cytoplasmic staining
- CK8/18: extensive positivity, especially in perivascular cell processes
- Chromogranin: negative
Cyclin D1: weak to moderate nuclear staining in 5-10% of tumor cells
-Ki67: mostly 10% labelling, but focally up to 20% (estimated)
Specimen D: Fragments of tumor with severe crush artefact and small fragments of gliotic grey matter.
DIAGNOSIS
EPENDYMOMA, PAPILLARY VARIANT , WITH INCREASED PROLIFERATIVE ACTIVITY
Our patient went on to receive adjuvant therapy with focal radiation in the form of proton beam therapy, which is recommended, compared with photon beam therapy, for its property of sparing normal tissue.
Our patient’s papillary ependymoma did not involve its spinal cord. Ependymoma's grading is reported to be less useful, but as per our pathologists, “increase proliferative activity” lesions have been associated with worse outcome.
So far, one year-year post resection (see Images)-resection and adjuvant proton radiotherapy, our patient remains free of disease recurrence.
-<p>Our patient was found to have signs of raised intracranial pressure, an unusual feature for a supratentorial ependymoma, which usually presents with headache, seizures or/and focal neurological deficits.</p><p>The imaging findings were typical for an ependymoma: On CT the mass was isodense relative to gray matter, contained both (micro)calcifications and cystic components. On MRI the mass's solid component demonstrated isointensity relative to gray matter on T1WI (not shown) and hyperintensity on T2WI with heterogeneity of enhancement post gadolinium. </p><p>Supratentorial ependymomas - rare primary tumours of the central nervous system - are thought to arise from the lining of cerebral ventricles or the spinal cord central canal. However, recent studies suggest that radial glial stem cells are the cell of origin. Surgical resection and radiotheray are the top main treatment modalities. </p><p>Prior to resection our patient's mass was endoscopically biopsied; results came back inconclusive between an ependymoma and a papillary tumor of the pinal region (PTPR). Subsequently, the mass was partly excised and histopathologically then confirmed it to be an “ependymoma. papillary variant, with increased proliferative activity”. </p><p> </p><p>HISTOLOGY</p><p>MICROSCOPIC DESCRIPTION:</p><p>Specimen A: Tiny fragments of grey matter. No tumor.</p><p>Specimen B and C: Moderately cellular tumor composed of isomorphic elongated tumor cells with oval euchromatic nuclei arranged in sheets, with perivascular pseudorosettes, occasional true ependymal rosettes, and true papillae covered by polarized ciliated tumor cells. No microvascular proliferation, no necrosis. Approx.2 mitoses/10 HPF.</p><p>IMMUNOPHENOTYPE:</p><p>- GFAP: very focally positive in perivascular cell procesesses</p><p>- EMA: prominent apical staining on surface of true papillae as well as focal dot-like cytoplasmic staining</p><p>- CK8/18: extensive positivity, especially in perivascular cell processes</p><p>- Chromogranin: negative</p><p>Cyclin D1: weak to moderate nuclear staining in 5-10% of tumor cells</p><p>-Ki67: mostly 10% labelling, but focally up to 20% (estimated) </p><p>Specimen D: Fragments of tumor with severe crush artefact and small fragments of gliotic grey matter.</p><p> </p><p>DIAGNOSIS</p><p>EPENDYMOMA, PAPILLARY VARIANT , WITH INCREASED PROLIFERATIVE ACTIVITY</p><p> </p><p>Our patient went on to receive adjuvant therapy with focal radiation in the form of proton beam therapy, which is recommended, compared with photon beam therapy, for its property of sparing normal tissue.</p><p>Our patient’s papillary ependymoma did not involve its spinal cord. Ependymoma's grading is reported to be less useful, but as per our pathologists, “increase proliferative activity” lesions have been associated with worse outcome.</p><p>So far, one year post resection (see Images) and adjuvant proton radiotherapy, our patient remains free of disease recurrence.</p>- +<p>Our patient was found to have signs of raised intracranial pressure, an unusual feature for a supratentorial ependymoma, which usually presents with headache, seizures or/and focal neurological deficits.</p><p>The imaging findings were typical for an ependymoma: On CT the mass was isodense relative to gray matter, contained both (micro)calcifications and cystic components. On MRI the mass's solid component demonstrated isointensity relative to gray matter on T1WI (not shown) and hyperintensity on T2WI with the heterogeneity of enhancement post gadolinium. </p><p>This patient's histologically confirmed papillary ependymoma did not involve its spinal cord. Ependymoma's grading is reported to be less useful, but as per our pathologists, “increase proliferative activity” lesions have been associated with worse outcome.</p><p>Supratentorial ependymomas - rare primary tumours of the central nervous system - are thought to arise from the lining of cerebral ventricles or the spinal cord central canal. However, recent studies suggest that radial glial stem cells are the cell of origin. Surgical resection and radiotherapy are the top main treatment modalities. </p><p>So far, one-year post-resection and adjuvant proton radiotherapy, our patient remains free of disease recurrence.</p>
Updates to Link Attributes
Updates to Primarylink Attributes
Updates to Study Attributes
MRI performed one day later:
Redemonstration of a solid-cystic mass in the region of the pineal gland, which is not individualized from the lesion. The lesion measures approximately 2.3 x 1.7 x 1.7 cm, coming in close contact with the confluence of the bilateral internal cerebral veins. The solid component of the lesion follows cortical signal with faint and irregular post contrast enhancement and restricted diffusion. The lesion blunts the posterior aspect of the third ventricle and extends through the aqueduct, causing obstruction and posterosuperior displacement of the tectal plate.
Moderate secondary dilatation of the ventricular system with mild periventricular transependymal flow. The fourth ventricle is of normal caliber. No significant effacement of the extra-axial CSF spaces.
No midline shift.
No evidence of leptomeningeal nodular enhancement.
No other intra or extra axial-axial focal lesion.
Updates to Study Attributes
One year later post resection and endoscopic third ventriculostomy:
Expected postsurgical changes related to right parieto-occipital craniotomy.
The surgical bed is clear.
No abnormal signal change or enhancement to suggest recurrence.
Updates to Freetext Attributes
Prior to resection, the patient's mass was endoscopically biopsied; results came back inconclusive between an ependymoma and a papillary tumor of the pineal region (PTPR). Subsequently, the mass was partly excised and histopathologically then confirmed it to be an “ependymoma. papillary variant, with increased proliferative activity”.
HISTOLOGY
MICROSCOPIC DESCRIPTION:
Specimen A: Tiny fragments of grey matter. No tumor.
Specimen B and C: Moderately cellular tumor composed of isomorphic elongated tumor cells with oval euchromatic nuclei arranged in sheets, with perivascular pseudorosettes, occasional true ependymal rosettes, and true papillae covered by polarized ciliated tumor cells. No microvascular proliferation, no necrosis. Approx.2 mitoses/10 HPF.
IMMUNOPHENOTYPE:
- GFAP: very focally positive in perivascular cell procesesses
- EMA: prominent apical staining on surface of true papillae as well as focal dot-like cytoplasmic staining
- CK8/18: extensive positivity, especially in perivascular cell processes
- Chromogranin: negative
- Cyclin D1: weak to moderate nuclear staining in 5-10% of tumor cells
- Ki67: mostly 10% labelling, but focally up to 20% (estimated)
Specimen D: Fragments of tumor with severe crush artefact and small fragments of gliotic grey matter.
DIAGNOSIS
EPENDYMOMA, PAPILLARY VARIANT, WITH INCREASED PROLIFERATIVE ACTIVITY
The patient went on to receive adjuvant therapy with focal radiation in the form of proton beam therapy, which is recommended, compared with photon beam therapy, for its property of sparing normal tissue.
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