Pediatric Brain Tumors

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

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?

Location specific symptoms

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

Imaging Studies MRI Emergency CT.

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.

CP1150826-4

CP1150826-3

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

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

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

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

Role of Irradiation Effective Toxic

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

Outcome Radiation + Chemotherapy Study group Historical Probability Probability Study group Historical Months post on study Months post on study CP1150826-1

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

CSF Dissemination

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

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

Ependymoma

Ependymoma

Ependymoma

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

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

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

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

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

Pilocytic Astrocytoma

Pilocytic Astrocytoma

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

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

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

Cerebellar Astrocytoma

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

Diffuse Pontine Astrocytoma

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

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

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

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

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

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

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.”

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

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.