pediatric brain tumors. brain tumors in children “oh, my god, my child/patient has a brain...
TRANSCRIPT
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
אזהרה סימני
גולגלתי • תוך לחץ יתרמתקדמים • שינוייםפוקאליים • שינוייםבאבחון- • איחור תינוקות
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
קליניקהאטקסיה: , • צרבלום
' וכו דיסמטריה•: רביעי חדר על לחץ
גולגלתי תוך לחץ יתרעצבים • עירוב עצבים אין עירוב אין
קרניאליםקרניאלים
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
ראשון- שלב טיפול
גליומה בדרגת
ממאירות נמוכה
אחיםאפנדימומה מדולובלסטומה
אפנדימומה
PNET
ATRT
...ועוד
אחרים
כריתה ריפוי= מלאהלעתים אךמספיקה
חלקית כריתה
עם כריתה 1.5שארית <מ" ס
סיכון= רבועגבוה
שארית כלפרוגנוזה=
טובה פחות
CPC
Role of Irradiation
• Effective
• Toxic
כריתה לאחר משלים טיפול
אפנדימומה מדולובלסטומה
אפנדימומה
קרינה למוח ולחוט
(36 גריי(
כמותרפי .stה )
Jude)
אין אםפיזור: קרינה מקומית
קרינה למוח
24וחוט ) גריי(
כמותרפי COGה )
vs. st. Jude)
SR
HR
60-85% 5yr EFS
40-70%EFS
0.0
0.2
0.4
0.6
0.8
1.0
0 20 40 60 80 100
120
140
160
180
Outcome Radiation + ChemotherapyOutcome Radiation + Chemotherapy
CP1150826-1
0.0
0.2
0.4
0.6
0.8
1.0
0 20 40 60 80 100
120
140
160
180
Pro
bab
ility
Pro
bab
ility
Months post on studyMonths post on study
Pro
bab
ility
Pro
bab
ility
Months post on studyMonths post on study
HistoricalHistorical
Study groupStudy group
HistoricalHistorical
Study groupStudy group
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
כריתה לאחר משלים טיפול
אפנדימומה מדולובלסטומה
אפנדימומה
קרינה למוח ולחוט
(36 גריי(
כמותרפי .stה )
Jude)
אין אםפיזור: קרינה מקומית
קרינה למוח
24וחוט ) גריי(
כמותרפי COGה )
vs. st. Jude)
SR
HR
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
קליניקהחדר • חסימת
לחץ- יתר רביעיגולגלתי תוך
על • לחץסימני- הצרבלום
מוחוןמסלולים • על לחץ
צד- סימני יורדיםעצבים • על לחץ
קרניאלים- ספציפיים חסרים
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.