Katelyn Fernando Presentation Medulloblastoma

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Medulloblastoma

Katelyn Fernando

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• Can occur in adults and children
• More common in young children
• Most common type of primary brain cancer in
children.
• 20% of all childhood brain tumors
Epidemiology • ~250-500 children diagnosed each year in
United States
• Less than 15 years old
• Male predominant
• The cause mostly unknown.
• Associated with some genetic abnormalities
Etiology • Turcot syndrome
• Gorlin syndrome
Anatomy
• Embryonic tumor of the
brain
• Starts in the cerebellum
• Muscle coordination,
balance, and movement
• Arises in the midline of the
cerebellum
• Can invade the fourth
ventricle and brainstem
• Spreads through the
cerebrospinal fluid
Histology
• Arises from cerebellar stem
cells
• Histologically appear as small,
round blue cells
• Four Main Histological Types
• Classic
• Desmoplastic/nodular
• Medulloblastoma with
extensive nodularity
• Large-cell anaplastic
Classic
Medulloblastoma
• Most common
• Highly cellular
• Composed of small round
cells
• Form Homer Wright rosettes
Demoplastic/Nodular

• Less cellular nodules


surrounded by densely
packed small cells
• More common in infants
• Better prognosis than Classic
Medulloblastoma
with Extensive
Nodularity
• Very similar to
Desmoplasmic/nodular
• Nodules are expanded
• Also occurs in infants with a
good prognosis
Large cell-
anaplastic
• Large anaplastic nuleli
• High mitotic rate
• Poor prognosis
Present over a period of a few weeks to a few
months
• Morning headaches
• Nausea/vomiting
• Poor coordination
Symptoms • Visual difficulties (rare)
• Back pain
• Difficulty walking
• Bladder and bowl incontinence
Diagnosis
• MRI with contrast
• Entire CNS
• Presents as a well-
defined central cerebral
mass.
• Hydrocephalus often
present
• Spinal tap
• Detect tumor cells in CSF
• Grade IV tumors
• Fast growing
• Classified as standard or high risk.
• Standard-risk
• No spread to CSF or other parts of brain
Classification • Less than 1.5𝑐𝑚2 of the tumor left after
surgery
• High-risk
• Spread to other parts of brain or spine.
• No spread but more than 1.5𝑐𝑚2 of the
tumor remains after surgery
• Combination of Surgery, Chemotherapy and
Radiation therapy
Treatment • Surgery is used as first line of treatment
Surgery
• To relieve fluid buildup
• To remove the tumor
• Residual tumor less than 1.5𝑐𝑚2
gives better outcomes
• Tumor infiltration into brainstem
or cerebellar peduncles reduces
resectability
• Posterior occipital craniotomy
• Surgery alone is rarely curative
• Can lead to posterior fossa
syndrome (PFS)
• Speech, communication, motor
control, and emotional difficulties
• Improved with rehabilitation
• Adjuvant chemotherapy
• Cisplatin, vincristine and CCNU
Chemotherapy • Allows for lower radiation doses or delayed
radiation
• Important to reduce long term side effects
Radiation
Therapy
Craniospinal
• Lateral-opposed fields for
brain
• Collimator angled to match
spine field divergence
• 1 or 2 posterior fields for the
spine (depending on patient
height)
• Gap junctions
Simulation
• Patient positioning is critical and
difficult because of the long fields.
• Prone position is preferred
• May not be possible due to
sedation.
• Aquaplast mask
• Head rest
• VacLoc bag
Immobilization has to accommodate
anesthesia equipment.
Critical Structures TD 5/5 (cGy)

Parotid 3200
Lens of Eye 1000
Ear mid/external 3000
Larynx 7000
Oral Cavity 6000
Heart 4000
Lungs 1750
Esophagus 5500
Liver 3000
Kidneys 2300
Treatment • Crainospinal
• 150 to 180 cGy/day
Doses • Total dose of 2340cGy
• Boost to posterior fossa
• Total dose to 5400cGy
Proton
Therapy
• Reduces normal tissue
exposure to radiation
• Bragg Peak
• Important in pediatrics to
reduce secondary
malignancies and late side
effects
Dose distributions for an 11-year-old male patient from CSI
technique applying electrons, photons, DS protons and spot
scanning IMPT.
Radiation Side Effects
Acute Late
• Radiation necrosis
• Fatigue
• Secondary malignancy
• Nausea/vomiting
• Hearing loss
• Headaches
• Cataracts
• Alopecia
• Decreased cognitive ability
• Short term memory loss
• Dependent on patient age and tumor spread
• No CSF spread: 70-80%

Survival Rate CSF spread: ~60%
• Patients younger than 3 years old have a lower
survival rate
• more aggressive tumor.
1. Washington CM, Leaver DT. Principles and Practice of Radiation Therapy.; 2016.
2. 2017 Lupdated: 30 O. These painted masks are helping children to get through their radiotherapy.
BT.com. http://home.bt.com/news/news-extra/these-painted-masks-are-helping-children-
to-get-through-their-radiotherapy-11364224812319. Accessed March 31, 2020.
3. Agamanolis D. Chapter 7. Medulloblastoma. http://neuropathology-
web.org/chapter7/chapter7eMedulloblastoma.html. Accessed March 31, 2020.
4. Brain tumor. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/medulloblastoma/cdc-
20363524. Published April 27, 2019. Accessed March 31, 2020.
5. Medulloblastoma. St. Jude Children's Research Hospital.

References: https://www.stjude.org/disease/medulloblastoma.html. Accessed April 2, 2020.


6. Medulloblastoma - Childhood - Stages. Cancer.Net. https://www.cancer.net/cancer-
types/medulloblastoma-childhood/stages. Published May 2, 2019. Accessed March 31, 2020.
7. Proton Therapy: Proven Cancer Treatment. Provision Healthcare. https://provisionhealthcare.com/.
Published March 13, 1970. Accessed March 31, 2020.
8. Research. All About Medulloblastoma. OncoLink.
https://www.oncolink.org/cancers/pediatric/information/brain-tumors-in-pediatric-
patients/all-about-medulloblastoma. Accessed March 31, 2020.
9. The experts in childhood cancer. Children's Cancer and Leukaemia Group. https://www.cclg.org.uk/.
Accessed March 31, 2020.

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