|Year : 2015 | Volume
| Issue : 4 | Page : 265-267
Anaplastic ependymoma: A rare brain tumor in a neonate
Iyer Harohalli Venkatesh1, Shantigrama Ramachandra Kumar1, Sunithi Krishna Mishra2
1 Department of Neonatology, Manipal Hospital, Bengaluru, Karnataka, India
2 Department of Histopathology, Manipal Hospital, Bengaluru, Karnataka, India
|Date of Web Publication||16-Oct-2015|
Iyer Harohalli Venkatesh
Manipal Hospital, Bengaluru, Karnataka
Source of Support: None, Conflict of Interest: None
A term male baby weighing 2.7 kg was brought to emergency with a history of increasing head size. Magnetic resonance imaging of the brain revealed enhancing hemorrhagic lesion with areas of necrosis involving septum pellucidum, predominantly in right frontal horn extending into 3rd ventricle with obstructive hydrocephalus. Tumor was decompressed and biopsy of the tumor revealed anaplastic ependymoma.
Keywords: Anaplastic ependymoma, brain tumor, hydrocephalus, immunohistochemistry, magnetic resonance imaging, neonate
|How to cite this article:|
Venkatesh IH, Kumar SR, Mishra SK. Anaplastic ependymoma: A rare brain tumor in a neonate. J Clin Neonatol 2015;4:265-7
| Introduction|| |
Congenital brain tumors represent 0.5–1.9% of all pediatric brain tumors. The incidence of central nervous system (CNS) tumors in UK is 2–3/100,000 children under 1 year per annum. In Europe, it is 3/100,000. In USA, they account for <5% of all newborn and stillborn children diagnosed with cancer. Although the prognosis is guarded, some successful operative cases have been reported. Surgery can be technically challenging and the sensitivity of the developing nervous system to the side effects of radio- and chemo-therapy has limited their utility as adjunctive treatments.
| Case Report|| |
A term male baby was brought to emergency room on day 4 of life with the history of increasing head size. He was born to Gravida 2 Para 1 mother by lower segment cesarean section (nonconsanguineous union). Baby cried immediately after birth with APGAR score of 7 and 8 at 1 and 5 min respectively. Birth weight was 2.84 kg. Antenatal scans done at 34 and 36 weeks of gestation revealed moderate hydrocephalus. Postnatal period was uneventful. Baby was started on breast feeding immediately after birth. On day 4 of follow-up, he was noticed to have sudden increase in the head circumference to 36.5 cm(birth head circumference was 34 cm). In view of increasing head circumference, baby was referred to our hospital. Baby at admission was active, alert and was hemodynamically stable. His heart rate was 142 beats/min and blood pressure was normal. He was maintaining saturations at room air. Neurologically baby had spontaneous eye opening, moving all four limbs, crying well and anterior fontanel was full but not tense. Magnetic resonance imaging brain [Figure 1] was done which demonstrated enhancing haemorrhagic lesion arising from or involving septum pellucidum, extending into third and fourth ventricle with obstructive hydrocephalus. He was admitted to neonatal intensive care unit and treated as per unit protocol. Septic markers were sent to rule out infection. Neurosurgery team was involved and endoscopic guided left frontal corticostomy and decompression of lesion was done with extra ventricular drainage placement in frontal lobe under general anesthesia. Specimen was sent for frozen section and immunohistochemistry.
|Figure 1: Magnetic resonance imaging demonstrating enhancing hemorrhagic lesion with areas of necrosis arising/involving septum pellucidum, predominantly in right frontal horn extending into 3rd ventricle along with involvement of adjacent frontal white matter and gyri with obstructive hydrocephalus|
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Hematoxylin and eosin stained paraffin sections [Figure 2] showed round to oval cells with hyperchromatic nuclei with pseudorosette formation in a fibrillary background suggesting anaplastic ependymoma. Immunohistochemistry was confirmatory of the same. Medical oncologist opinion was sought who advised to start vincristine after stabilization. Baby was ventilated for 2 days postoperatively during which he had 1 episode of generalized tonic-clonic seizure and was treated with phenobarbitone. He was successfully extubated to continuous positive airway pressure and then to room air after 48 h. Baby was started on feeds gradually increased to full feeds that he accepted and tolerated well. He was shifted to ward and then discharged home.
|Figure 2: H and E stained paraffin section in ×10 (a) and ×40 (b) showing round to oval cells with hyperchromatic nuclei with pseudorosette formation in a fibrillary background|
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- Congenital hydrocephalus
- Space occupying lesion of brain.
| Discussion|| |
Arnstein et al.first reviewed congenital brain tumors presenting in neonates, and defined them as limited to tumors presenting within 60 days after birth. Later in 1964, Solitare and Krigman reviewed 45 cases of neonatal brain tumors and classified into three categories: (a) Definitely congenital, those presenting or producing symptoms at birth (b) probably congenital, those producing symptoms within 1st week of life and (c) possibly congenital presenting within 1st month of life. Wakai et al. modified this category by limiting the last group to neonates presenting within 2 months of life.
Congenital brain tumors represent only 0.5-1.9% of all pediatric brain tumors. The incidence of CNS tumors in UK is 2-3/100,000 children under 1 per annum in 2000. In Europe, it is 3/100,000. In USA, they account for <5% of all newborn and stillborn children diagnosed with cancer.
Brain tumors in infancy have a different biology than tumors presenting later in childhood. In most series, the location of these tumors has been reported as supratentorial and only a few cases infratentorial location have been mentioned. Supratentorial astrocytomas, medulloblastomas (including primitive neuroectodermal tumor) and choroid plexus tumors are most common tumors of infancy, whereas later in childhood, the most common tumors are infratentorial astrocytomas and medulloblastomas. Tumors of infancy are predominantly supratentorial, with a shift to infratentorial predominance at around 2 years of age.
Congenital intracranial tumors manifest clinically in several ways. Sometimes they are detected incidentally on routine prenatal ultrasonography. The most frequent, initial symptom reported in literature, is an enlarging head size as seen in our study. Signs of increased intracranial pressure (ICP) such as vomiting and papilledema were noted infrequently because in neonates freely expanding calvaria can accommodate the increased volume. Focal neurologic changes were absent in most cases of neonatal brain tumor as in our baby despite large head size and hydrocephalus. Thus, neonates presenting with an abnormal increasing head circumference with or without signs of rising ICP should have urgent neuroimaging as it was done in our baby too.
Ependymomas are relatively rare tumors in neonates, accounting for 2-3% of all primary brain tumors. These arise from the ependymal cells of cerebral ventricles, the central canal of the spinal cord and cortical rests. About 30% of pediatric ependymomas are diagnosed in children younger than 3 years of age. According to World Health Organization classification of tumors, there are four types of ependymal tumors; (1) subependymomas, (2) myxopapillary ependymoma, (3) ependymoma, (4) anaplastic ependymomas. Anaplastic ependymomas are high-grade tumors (grade III) and tend to be growing faster than low-grade tumors and has less favorable prognosis.
As most cases present with symptoms of intracranial hypertension or increasing head size, patient management is directed toward reliving hydrocephalus that usually needs urgent CSF drainage. Determining the histopathology of the lesion and removing the tumor mass are the other important steps. In spite of good decompression, it may be prudent to leave a ventricular drain in situ postoperatively for several days to monitor ICP to find whether shunting is required as done in our baby. It is mostly recommended that these tumors should be completely removed surgically, whenever not feasible; it should be followed by adjuvant therapy. Irradiation is generally not recommended in children under 36 months of age because of high-risk of cognitive sequelae and endocrine dysfunction. Chemotherapy remains a much accepted and well-tolerated method of choice in this age group and has been proven beneficial as an adjuvant therapy in many tumors once the mass is resected incompletely. The possible neurotoxicity of chemotherapy, including mental retardation and leukoencephalopathy, in these neonates, concerns the pediatric oncologists. A standard effective and well-accepted treatment protocol for the optimal management of neonatal brain tumor is yet to be defined.
The survival rate of neonates with brain tumors has been overall poor. Most reports have shown a better prognosis for tumors of supratentorial location due to site that offers a more accessible surgical approach and the more benign histopathology of supratentorial tumors. It has been shown that the degree of surgical resection is one of the important treatment parameters with complete resection offering the best prognosis. However, surgical resection has been associated with a higher rate of operative mortality ranging from 26% to 33%. This prognosis is not only related to the surgery but also due to risk in anesthesia (hypothermia, bleeding), the difficulties in postoperative care (lack of voluntary control of water and electrolytes) along with the pessimistic attitude of parents in seeking treatment or during follow-up.
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[Figure 1], [Figure 2]