1. Pediatric Brain Tumors

2. Brain Tumors in Children
“Oh, my God, my child/patient has a brain tumor!”
Depression!

3. Brain Tumors in Children
What are the symptoms and signs of brain tumors in children?
What kinds of tumors are there and how are they treated?
Is the outcome always so bad?

4. Location specific symptoms

5. סימני אזהרה יתר לחץ תוך גולגלתי שינויים מתקדמים שינויים פוקאליים
תינוקות-איחור באבחון

6. Imaging Studies
MRI
Emergency CT.

7. Types of Tumors
Brain tumors is children are not common, about 3 cases per 100,000 children less than 15 years of age per year or about 1500 new cases per year.
Second only to leukemia in both incidence and mortality.

8. CP

9. CP

10. קליניקה צרבלום: אטקסיה , דיסמטריה וכו'
לחץ על חדר רביעי: יתר לחץ תוך גולגלתי
אין עירוב עצבים קרניאלים

11. Medulloblastoma 20% of pediatric brain tumors
40% of posterior fossa tumors

12. Medulloblastoma
Symptoms often those of hydrocephalus/raised ICP--headache, vomiting, diplopia
Falling, incoordination
Signs: papilledema, EOM paresis, ataxia

13. טיפול- שלב ראשון אחרים אפנדימומה מדולובלסטומה
גליומה בדרגת ממאירות נמוכה
אחים
אפנדימומה
ATRT
PNET
CPC
ועוד...
כל שארית =פרוגנוזה פחות טובה
כריתה מלאה=ריפוי אך לעתים מספיקה כריתה חלקית
כריתה עם שארית >1.5 ס"מ רבוע=סיכון גבוה

14. Role of Irradiation
Effective
Toxic

15. אם אין פיזור: קרינה מקומית כמותרפיה (COG vs. st. Jude)
טיפול משלים לאחר כריתה
אפנדימומה
מדולובלסטומה
אפנדימומה HR SR
אם אין פיזור: קרינה מקומית
קרינה למוח ולחוט (36
גריי)
כמותרפיה (st. Jude)
קרינה למוח וחוט (24
גריי)
כמותרפיה (COG vs. st. Jude)
60-85% 5yr EFS
40-70%
EFS

16. Outcome Radiation + Chemotherapy
Study group
Historical
Probability
Probability
Study group
Historical
Months post on study
Months post on study CP

17. Prognostic Factors Age ‹2 years, poor prognosis
CSF dissemination, poor prognosis
Radical resection, good prognosis

18. CSF Dissemination

19. Ependymoma 6% of pediatric brain tumors
70% occur in the posterior fossa
Hallmark on imaging is extension out of the foramina of the fourth ventricle into the CPA or cervical canal

20. Medulloblastoma-Survival
Current best 5-year survival rates are 70%
Not too bad!
Survival continues to fall after 5 years.
We need radical, new treatments that are effective and eliminate use of radiation

21. Ependymoma

22. Ependymoma

23. Ependymoma

24. Ependymoma Signs and symptoms of hydrocephalus
May be prominent vomiting from invasion of floor of fourth ventricle

25. אם אין פיזור: קרינה מקומית כמותרפיה (COG vs. st. Jude)
טיפול משלים לאחר כריתה
אפנדימומה
מדולובלסטומה
אפנדימומה HR SR
אם אין פיזור: קרינה מקומית
קרינה למוח ולחוט (36
גריי)
כמותרפיה (st. Jude)
קרינה למוח וחוט (24
גריי)
כמותרפיה (COG vs. st. Jude)
60-85% 5yr EFS
40-70%
EFS

26. Ependymoma Treatment consists of radical resection
No question that prognosis is greatly influenced by extent of resection
Patients with radiographically confirmed GTR have greater than 80% five-year survival; 20% or less for less than GTR

27. Astrocytoma
Symptoms and signs depend on location. Posterior fossa-symptoms and signs for hydrocephalus. Cerebral hemispheres-focal deficit, seizures.
Tumor behavior depends on histology
Tumor treatment depends on histology and location

28. Cerebellar Astrocytoma
Pilocytic astrocytoma
Diffuse, grade II astrocytoma
Grade III or IV astrocytoma in the cerebellum is rare in children

29. Pilocytic Astrocytoma

30. Pilocytic Astrocytoma

31. Pilocytic Astrocytoma
Surgical disease
We try to remove all tumor
But we do not chase tumor into cerebellar peduncle, brainstem

32. Pilocytic Astrocytoma
Post op scan clean, follow
6 month scan clean, may not need any further studies

33. Cerebellar Astrocytoma
Treatment for pilocytic astrocytoma is resection. A gross total resection is goal
Controversy: Immediate reoperation for residual tumor?

34. Cerebellar Astrocytoma

35. Cerebellar Astrocytoma
If postoperative scan shows residual tumor, only about 1/3 will show growth over next 10 years
Reasonable to follow for symptoms and with scans, reoperate for progression

36. Diffuse Pontine Astrocytoma

37. Brainstem Tumors
Called “brainstem glioma”, but not all tumors in the brainstem are the same.
Symptoms and signs of brainstem dysfunction: diplopia, swallowing problems, facial weakness, long track signs

38. קליניקה חסימת חדר רביעי- יתר לחץ תוך גולגלתי
לחץ על הצרבלום- סימני מוחון
לחץ על מסלולים יורדים- סימני צד
לחץ על עצבים קרניאלים- חסרים ספציפיים

39. Focal Brainstem Astrocytoma
No rush to treat. Many are slow growing and cause few symptoms
Stereotactic biopsy may be used to establish histology, if needed
Treatment for documented growth and/or symptom progression

40. Diffuse Pontine Astrocytomas
Characteristic image, diffuse infiltration of the pons
Unresectable
No role for biopsy, as patients do poorly regardless of histology

41. Diffuse Pontine Astrocytomas
No controversy, 2 year survival is less than 5%.
What is needed is radical, new, effective therapy

42. Diffuse Pontine Astrocytomas
No effective treatment
Conventional or hyperfractionated radiation are palliative
No effective chemotherapy

43. Conclusions
Brain tumors in children are not common, but must be kept in mind for the child with headache or neurologic symptoms or signs. Subtle findings may be important.
“One perceives only what one actively seeks.”

44. Conclusions Outcome is not as bad as generally thought
Pilocytic astrocytomas, grade II astrocytomas, gangliogliomas, choroid plexus papillomas, dermoid tumors all can be treated effectively with surgery alone
Medulloblastoma, ependymoma, malignant germ cell tumors have greater than 70% long term survival with surgical resection, radiation therapy and chemotherapy

45. Conclusions Irradiation is bad for the brain
Current research directed at finding focused therapy based on what is known about the molecular biology of the different tumor types.