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anaplastic_ependymoma

Anaplastic ependymoma

Anaplastic ependymomas (WHO grade III ependymoma) are characterised by a higher proliferative rate and a greater tendency to infiltrate surrounding brain or disseminate into cerebrospinal fluid causing drop metastases.

Epidemiology

About 25% of ependymomas exhibit features of anaplasia with a high mitotic rate, cellular pleomorphism, and intratumoral necrosis; these are considered WHO grade III

Pathology

These tumours have a greater propensity to infiltrate surrounding brain or spinal cord parenchyma and have a higher proliferative rate.

Radiographic features

An anaplastic ependymoma typically presents as a calcified cystic tumour in the supratentorial parenchyma or transependyma

Treatment and prognosis

Complete surgical removal resulting in cure is unlikely for anaplastic ependymoma. Even if post-operative imaging demonstrates no residual tumour, the median time to first recurrence is approximately 18 months and to second recurrence is an additional 12 months

Anaplastic ependymomas, are far more common in the pediatric age group, frequently arising as supratentorial ependymoma.

see also Spinal anaplastic ependymoma.

The benefit of postoperative chemotherapy for anaplastic ependymoma remains unknown.

Temozolomide (TMZ) may be effective for pediatric refractory anaplastic ependymoma with low MGMT protein expression 1).

Case report

2015

A 12-year-old girl with an anaplastic ependymoma of the left temporal lobe. She underwent initial image-guided resection following biopsy. A postoperative MRI showed a macroscopic resection. She subsequently relapsed and indeed had 11 local and distant relapses managed by 12 separate craniotomies and tumour resection, 4 courses of radiotherapy and chemotherapy.

For patients with multiple relapses, surgery should be considered primarily to re-resect any symptomatic lesion. This case demonstrates that multiple tumour resections can be undertaken with limited morbidity for the patient and with maintenance of quality of life 2).

2014

A young child presented to the emergency department of a tertiary care hospital with on and off headache, focal seizures involving the left side of the body, weakness of left upper and lower limbs and vomiting for 2 weeks. Examination showed an alert child with grade 4/5 powers in left upper and lower limbs. Blood investigations were normal. An urgent CT of the brain showed intra-axial mass in the right frontal cerebral cortex, superolateral to the right lateral ventricle. MRI of the brain showed supratentorial extraventricular mass of 5.20×3.70×3.80 cm, in the right frontal cortex, emitting heterogeneous signals on T1, T2 and fluid-attenuated inversion recovery sequences and impression of astrocytoma, ependymoma or choroid plexus papilloma was made. Complete surgical resection of mass was performed. Histopathology of the mass proved it as WHO grade III anaplastic ependymoma. The child made an uneventful postoperative recovery and radiotherapy was followed 3).

1)
Komori K, Yanagisawa R, Miyairi Y, Sakashita K, Shiohara M, Fujihara I, Morita D, Nakamura T, Ogiso Y, Sano K, Shirahata M, Fukuoka K, Ichimura K, Shigeta H. Temozolomide treatment for pediatric refractory anaplastic ependymoma with low MGMT protein expression. Pediatr Blood Cancer. 2015 Aug 25. doi: 10.1002/pbc.25696. [Epub ahead of print] PubMed PMID: 26305586.
2)
Kitchen WJ, Pizer B, Pettorini B, Husband D, Mallucci C, Jenkinson MD. Paediatric Intracranial Anaplastic Ependymoma: The Role of Multiple Surgical Resections for Disease Relapse in Maintaining Quality of Life and Prolonged Survival. Pediatr Neurosurg. 2015 Apr 10. [Epub ahead of print] PubMed PMID: 25871983.
3)
Khilji MF, Hamid RS, Qureshi A. Supratentorial extraventricular anaplastic ependymoma in a child. BMJ Case Rep. 2014 Mar 12;2014. pii: bcr2014203750. doi: 10.1136/bcr-2014-203750. PubMed PMID: 24623545.
anaplastic_ependymoma.txt · Last modified: 2017/11/22 18:31 by administrador