**19. Pineal tumors**

*Current Cancer Treatment*

**Table 5.**

in lactic acid and lipid peaks [64].

*Risk stratification of medulloblastoma.*

or absence of disseminated disease (**Table 5**).

they appear iso- to hyperintense to grey matter and can seem heterogeneous due to cyst formation, calcification and necrosis. MR spectroscopy shows elevated choline peaks and decreased creatine and N-acetyl acetate peaks, with occasional elevation

Residual postoperative tumour volume <1.5 cm2 ≥1.5 CSF cytology/evidence of disease dissemination on MRI in brain and spine Absent Present

**Average risk**

**High risk**

Maximal safe resection is recommended in all medulloblastoma patients. Apart from surgical resection, the current standards of radiation therapy and medical management vary by extent of disease and age of the patient. Radiation therapy can be used to decrease the risk of recurrence but neurocognitive effects of radiation

Patients who are 3 years of age or older are stratified as either "average-risk" or "high-risk" depending upon postoperative residual tumor volume and the presence

Patients who are younger than 3 years of age, are treated without upfront radiation therapy due to the unacceptably high risk of severe neurocognitive impairment [65]. In the postoperative setting, average-risk patients >3 years old were previously treated with 36 Gy craniospinal irradiation (CSI) but now a boost to the posterior fossa is given for a total dose of 54 Gy due to the high rate of relapse within the posterior fossa. CSI dose of 23.4–24 Gy can be given with the addition of chemotherapy as supported by Studies conducted by the International Society of Pediatric

For high risk medulloblastoma cases in children 3 years or older, the treatment is surgical resection followed by post-operative "standard dose" RT (36 Gy CSI with a boost to both the posterior fossa and focal sites of metastatic disease to 55.8 Gy) as

The most common adverse effect of craniospinal irradiation in children <3 years age is neurocognitive deficit. Therefore radiotherapy is either delayed or omitted in this subset of patients. There is evidence that regimens consisting of surgery and chemotherapy without RT can be successful in specific subsets of medulloblastoma patients. Outcomes in patients with relapsed disease are generally poor, with reported 5-year survival rates of approximately 25% [68]. Unfavorable prognostic factors include large tumor, csf dissemination, age <4 years, subtotal tumour resec-

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

Current recommendations for post-radiation chemotherapy in averagerisk patients include approximately 1 year of therapy consisting of 8 cycles at 6-week intervals of cisplatin, lomustine (CCNU), and vincristine. The St. Jude Medulloblastoma-96 trial has demonstrated a similar event-free survival of 83% when an alkylator-based, dose-intensive chemotherapy regimen consisting of four 4-week cycles of cyclophosphamide, cisplatin, and vincristine with autologous stem

therapy have to be considered by weighing the risk benefit ratio.

Oncology (SIOP) and the Children's Oncology Group [66].

cell rescue was employed following each cycle [67].

tion (<90%), chromosome deletion 17p, c-*MYC* amplification.

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

well as adjuvant chemotherapy.

**162**

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, ependymoma and atypical teratoid or rhabdoid tumors [76].

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


#### **Table 6.**

*Treatment strategies of different pineal tumours.*

#### **20. Ependymoma**

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

They are usually located in or adjacent to ventricles within the parenchyma. In pediatric age group majority of intracranial ependymoma are located at infratentorial region in posterior fossa, usually arising at the floor of fourth ventricle.

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


**Table 7.**

*WHO pathological grades of ependymoma.*

The current treatment of choice for pediatric patients with cranial ependymoma is resection, if possible, followed by radiation therapy alone [80].
