**18. Atypical teratoid rhabdoid tumors (ATRT)**

Atypical teratoid rhabdoid tumours (ATRTs) are the most common malignant central nervous system tumours in children ≤1 year of age and represent

**163**

**20. Ependymoma**

**Table 6.**

Non-germinomatous germ cell tumour

logical grading system (**Table 7**).

*Treatment strategies of different pineal tumours.*

*An Overview of Pediatric CNS Malignancies DOI: http://dx.doi.org/10.5772/intechopen.88189*

**19. Pineal tumors**

compression by invading the adjacent cisternal space.

ependymoma and atypical teratoid or rhabdoid tumors [76].

of 30 Gy

response

Pineoblastoma <3 years Radiotherapy is avoided

approximately 1–2% of all pediatric brain tumours [69]. ATRT is a primarily monogenic disease characterized by the bi-allelic loss of the *SMARCB1* gene, which encodes the hSNF5 subunit of the SWI/SNF chromatin remodeling complex [70]. The most common site of ATRT is posterior fossa, mainly cerebellar hemispheres (⅔ cases) [71]. It can also occupy fourth ventricle causing its displacement and

In patients <3 years of age, the most common treatment is high dose chemotherapy with autologous stem cell rescue, so that CSI can be avoided in young patients as poor outcomes are seen due radiotherapy induced neurocognitive impairment [72]. Despite using chemotherapy and radiotherapy as treatment options, ATRT has poor

survival outcomes due to early dissemination and progression of the tumours [73].

Incidence of pineal tumours in children ranges from 2.7 to 11% [74]. Germ cell tumors (GCTs) account for nearly 50–75% of all pineal tumors [75], Pineal parenchymal tumors account for nearly 15–27% of pineal tumors and include pineocytoma, parenchymal tumor of intermediate differentiation, pineoblastoma and papillary tumor of the pineal region. Other described pineal tumors include glioma,

Preferred treatment strategy of different pineal region tumours [77] (**Table 6**).

Pineoblastoma >6 years Full-dose craniospinal irradiation (36 Gy) plus boost (total of 54 Gy) to the

followed by 6 cycles of maintenance chemotherapy Germinoma Four cycles of chemotherapy with carboplatin and etoposide followed by whole

chemotherapy with stem cell rescue Pineoblastoma 3–6 years Induction chemotherapy followed by consolidation myeloablative chemotherapy with stem cell rescue

Induction chemotherapy followed by consolidation myeloablative

primary site along with concomitant daily carboplatin and weekly vincristine

ventricular irradiation to 23.4 Gy plus a boost to the primary site to a total dose

Six cycles of chemotherapy with carboplatin, ifosfamide and etoposide followed by 30 Gy whole ventricular irradiation plus a boost to the primary site to a total dose of 50 Gy in patients with a radiographic and serologic complete

Ependymoma accounts for 6–12% of all brain tumors in childhood. It represents the third most common brain tumor in this age group, following astrocytomas and medulloblastomas [78]. Ependymoma are classified according to the WHO patho-

They are usually located in or adjacent to ventricles within the parenchyma. In pediatric age group majority of intracranial ependymoma are located at infratento-

Prognostic factors include tumor location, size, surrounding anatomical structures, tumor appearance, genotype, comorbidities, clinical symptoms, and patient age [79].

rial region in posterior fossa, usually arising at the floor of fourth ventricle.

*An Overview of Pediatric CNS Malignancies DOI: http://dx.doi.org/10.5772/intechopen.88189*

approximately 1–2% of all pediatric brain tumours [69]. ATRT is a primarily monogenic disease characterized by the bi-allelic loss of the *SMARCB1* gene, which encodes the hSNF5 subunit of the SWI/SNF chromatin remodeling complex [70]. The most common site of ATRT is posterior fossa, mainly cerebellar hemispheres (⅔ cases) [71]. It can also occupy fourth ventricle causing its displacement and compression by invading the adjacent cisternal space.

In patients <3 years of age, the most common treatment is high dose chemotherapy with autologous stem cell rescue, so that CSI can be avoided in young patients as poor outcomes are seen due radiotherapy induced neurocognitive impairment [72].

Despite using chemotherapy and radiotherapy as treatment options, ATRT has poor survival outcomes due to early dissemination and progression of the tumours [73].
