The Cribsiders podcast

#81: Pediatric Brain Tumors – Let’s Think About It!

March 29, 2023 | By

Audio

Summary:

Brain tumors can be intimidating, for families and providers alike! Tune into this episode as Dr. Trent Hummel, Pediatric Neuro-Oncologist, guides us through the red flags to look for, when to escalate care and how to approach discussions with families. This conversation will blow your mind!

 

Pediatric Brain Tumor Pearls

  1. When a patient presents with headache, be on the lookout for red-flag signs and symptoms, including severe and sudden onset, persistent and progressive pain, pain upon waking in the morning, alleviation of pain with vomiting or new-onset neurologic symptoms (frequent falls, dizziness, lack of use of an extremity, etc.). 
  2. In addition to obtaining a symptom history, be sure to take a thorough past medical history, medication reconciliation and social history, looking for pertinent factors such as smoking or drug use.
  3. Complete a thorough neurologic and fundoscopic exam in patients with red flag symptoms. 
  4. If you have concern for a potential mass-like process, you should have a low threshold for obtaining imaging. CT scans are good for screening, and MRIs are more definitive. Take into consideration your patient’s individual factors (ability to tolerate longer scans, ability to undergo sedation, etc.) and your level of concern in deciding which imaging is best. 
  5. Obtain lab work judiciously – consider a complete blood count and a renal panel to assess for anemia/leukocytosis and electrolyte abnormalities, respectively.
  6. In cases in which you have high concern for a brain mass, consider referring to an Emergency Department to expedite work up. 
  7. The majority of pediatric brain tumors are in the posterior fossa (60%). The most common types, in decreasing frequency, include medulloblastoma, juvenile pilocytic astrocytoma (JPA), ependymoma, diffuse intrinsic pontine glioma (DIPG), and atypical teratoid rhabdoid tumor (ATRT). The other 40% of pediatric brain tumors are in the cerebral hemispheres of the brain, and include astrocytomas, gangliogliomas, craniopharyngiomas and meningiomas, amongst others. [1]
  8. Depending on the type of tumor, treatment can range from various combinations of surgical resection, chemotherapy, radiation and/or immunotherapy.
  9. Minority groups are traditionally under-represented in clinical trials, and often have difficulty accessing quality care for their child’s tumor.


Pediatric Brain Tumor Notes 

Patient History

  • Key history components include: location of pain, characteristics, onset, timing, duration, alleviating and aggravating factors, and associated symptoms (e.g., focal weakness, dizziness, loss of consciousness, increased falls, vomiting that alleviates pain, waking from sleep, phono/photophobia). 
  • In younger kids who may have more difficulty verbalizing their symptoms, parents may notice things like not eating as well, increased vomiting, poor growth/development, fussiness or increased clumsiness (i.e., running into walls). 
  • Expert Opinion: Oftentimes, a parent will have concern that something is different than before, though they may be unable to specify or verbalize what exactly is different. Dr. Hummel recommends taking this “parent’s intuition” into account. 
  • Other important history questions include: family history of migraines or headaches (strong predictor of benign headaches), a thorough medication reconciliation (e.g., contraceptive pill or acne medications, which could make other benign differential diagnoses such as idiopathic intracranial hypertension more likely), and a thorough social history (e.g., smoking, caffeine, alcohol or other drug use, stress, trauma). 

History Red Flags

  • Systemic symptoms (e.g., fever, signs of meningitis, myalgia, malaise)
  • Neurological deficits/dysfunction (e.g., altered mental status, weakness, seizures)
  • Severity of headache is constant and progressive
  • Pattern changes of headache or recent onset (pearl: symptom onset in primary brain tumors is usually insidious, while symptom onset in patients with brain metastases is typically acute or subacute). 
  • Positional headache
  • Papilledema and other signs of increased ICP
  • Visual deficits
  • Pain on waking in the morning

Physical Exam

  • Complete neurologic exam, including mental status, cranial nerves, sensation, motor function, reflexes, coordination, cerebellar function and gait assessment. Be on the lookout for nystagmus, focal neurologic deficits or signs of cerebellar dysfunction, which can be present in patients with brain tumors. 
  • Fundoscopic exam, assessing for papilledema (sign of increased intracranial pressure). Tips include: In a dark room, have the patient fixate on a target as far as possible in the room to best dilate the eye. Angle the fundoscope 15 degrees lateral to the patient’s line of sight, and identify the red reflex by slowly approaching the patient until the instrument is 2-4 cm from the eye. Angle the ophthalmoscope to visualize the structure of the eye, starting with branching blood vessels. Once you have identified a blood vessel, follow it in towards the optic disc. Assess for papilledema (a blurred, swollen disc). Other tips and abnormal findings can be found at this site. [2]

Diagnostic Work Up

  • In a patient with concern for a brain mass, Dr. Hummel recommends having a low threshold for imaging. You can refer to your nearby Emergency Department to expedite the process. 
  • CT scan: Good for screening in patients who are unstable, cannot tolerate long scans or sedation. Order without contrast, as this will be sufficient to identify a mass. If you have concern for other metabolically active etiologies like infection, you can consider a CT scan with/without contrast. 
  • MRI with/without contrast: Preferred method of imaging in patients who are stable, can tolerate long scans and/or can tolerate sedation. Lookout for kids with decreased renal function and consider omitting contrast. 
  • Labs: Labs have little function in diagnosing brain tumors outside of ruling out other etiologies, such as infection. That said, Dr. Hummel recognizes the utility of a CBC to assess for anemia/leukocytosis and a renal panel to assess for electrolyte abnormalities. 
  • Pearl: In patients with hydrocephalus, there can be pituitary involvement leading to precocious puberty, short stature or panhypopituitarism.  
  • Consults: If imaging shows a mass, consult your friendly neighborhood neurosurgeon and oncologist. 

Treatment

  • If you are in an outpatient setting, and have concern for a brain mass, Dr. Hummel says to consider deferring dexamethasone until your patient is evaluated by neurosurgery unless you have concern for acute increased intracranial pressure.
  • Consult neurosurgery and oncology to help formulate a treatment plan. 
  • Surgical Resection: Gold standard treatment; goal is total resection & pathology. If a patient has a subtotal resection, can consider surveillance with intermittent MRI’s and/or adjuvant treatment with chemotherapy or radiation (low grade gliomas tend not to grow back). 
  • Immunotherapy:  Genomic alterations identified within pediatric brain tumors can provide definitive diagnosis and offer potential therapeutic interventions with less side effects than traditional chemotherapy. 
  • Chemotherapy: Typically a carboplatin based regimen.
  • Radiation: Kids have developing brains, so not typically used unless last line. Be aware of endocrinopathies as a result of radiation – growth hormone deficiency, thyroid abnormalities, reproductive potential (radiation and chemotherapy).
  •  

Communicating Bad News

  • Find tips on breaking bad news at this American Academy of Family Physicians article. [3]
  • Dr. Hummel recommends using the phrasing, “mass”, in place of definitive words like “malignant” or “cancer”. This is often unsettling to parents and can be premature. 
  • Dr. Hummel emphasizes the importance of defining benign, malignant and metastatic.  “Benign” refers to well-differentiated tumors that have slow growth, and often do not recur after resection. “Malignant” refers to poorly-differentiated tumors with unpredictable growth and metastatic potential, and often recur after resection. “Metastatic” refers to the ability of cancer to disseminate from the primary site of cancer to other parts of the body. While all tumors will have an impact on the life of a child, it is important to be accurate when discussing brain masses with families.

Disparities

  • Multiple studies have shown disparities in childhood brain tumors when stratified for race and ethnicity. Racial disparity among children likely reflects a lack of access to treatment and low enrollment/representation in clinical trials. 
  • Study 1: In a statistical review published in 2021 in “CA: A Cancer Journal for Clinicians”, the 5-year survival in children with malignant brain tumors was found to be lowest in non-Hispanic Black patients (70%) and highest in non-Hispanic White patients (79%). This persisted after adjustment for tumor stage, location and histology. [4]
  • Study 2: Another study using the National Program of Cancer Registries analyzed the racial and ethnic differences in survival rates in pediatric brain tumors. Using data from 11,302 patients, the 5-year survival rate was “77.6% for non-Hispanic white patients, 69.8% for non-Hispanic black patients, and 72.9% for Hispanic patients”. Additionally, “Based on multivariable analysis, non-Hispanic black patients had a higher risk of death at 5 years after diagnosis compared to non-Hispanic white patients (adjusted hazard ratio = 1.2, 95% confidence interval, 1.1–1.4).” [5]
  • Study 3: A third study analyzed 9577 children & adolescents diagnosed with primary malignant CNS tumors, and found race/ethnicity to be strongly associated with survival (p<0.001), with Black and Hispanic children having higher hazards of death than White children. This disparity likely exists due to lack of access to care and lack of representation of minority groups in clinical trials. [6]

Citations

  1. Storm PB. Pediatric Brain Tumors. Children’s Hospital of Philadelphia. https://www.chop.edu/conditions-diseases/pediatric-brain-tumors. Published January 28, 2014. Accessed February 27, 2023. 
  2. Stanford Medicine 25. Fundoscopic Exam (Ophthalmoscopy). Stanford Medicine 25. https://stanfordmedicine25.stanford.edu/the25/fundoscopic.html. Accessed February 27, 2023. 
  3. Vandekieft GK. Breaking Bad News. American Family Physician. 2001;64(12):1975-1978. https://www.aafp.org/dam/brand/aafp/pubs/afp/issues/2001/1215/p1975.pdf. Accessed February 27, 2023. 
  4. Miller, KD, Ostrom, QT, Kruchko, C, Patil, N, Tihan, T, Cioffi, G, Fuchs, HE, Waite, KA, Jemal, A, Siegel, RL, Barnholtz-Sloan, JS. Brain and other central nervous system tumor statistics, 2021. CA Cancer J Clin. 2021. https://doi.org/10.3322/caac.21693
  5. Siegel DA, Li J, Ding H, Singh SD, King JB, Pollack LA. Racial and ethnic differences in survival of pediatric patients with brain and central nervous system cancer in the United States. Pediatr Blood Cancer. 2019;66(2):e27501. doi:10.1002/pbc.27501
  6. Mitchell HK, Morris M, Ellis L, Abrahão R, Bonaventure A. Racial/ethnic and socioeconomic survival disparities for children and adolescents with central nervous system tumours in the United States, 2000-2015. Cancer Epidemiol. 2020;64:101644. doi:10.1016/j.canep.2019.101644

Goal

Listeners will explain headache red flag symptoms, basic evaluation and work up, and management of brain tumors in pediatric patients.  

Learning objectives

After listening to this episode listeners will…  

  1. Recall the red-flag signs and symptoms of pediatric headaches. 
  2. Identify the historical questions and physical exam maneuvers that are essential to diagnosing pediatric brain tumors. 
  3. Describe the imaging and lab work up of pediatric brain tumors.
  4. Recognize when it is appropriate to escalate care for further management of pediatric brain tumors.
  5. Feel comfortable relaying bad news and communicating with families about their child’s brain tumor.
  6. Recognize racial disparities in diagnosis and treatment of pediatric brain tumors.

Disclosures

Dr. Hummel reports no relevant financial disclosures. The Cribsiders report no relevant financial disclosures. 

Citation

Holloway R, Hummel T, Lee N, Mao C, Masur S, Chiu C, Berk J. “#81: Pediatric Brain Tumors –  Let’s Think About It!”. The Cribsiders Pediatric Podcast. https:/www.thecribsiders.com/ March 29, 2023.

Episode Credits

  • Producer, Writer and Infographic: Rachel Holloway
  • Executive Producer: Nick Lee MD
  • Showrunner: Sam Masur MD
  • Cover Art: Chris Chiu MD
  • Hosts: Chris Chiu MD, Clara Mao MD and Rachel Holloway
  • Editor: Justin Berk MD; Clair Morgan of nodderly.com
  • Guest(s): Trent Hummel, MD

CME Partner

vcuhealth

The Cribsiders are partnering with VCU Health Continuing Education to offer FREE continuing education credits for physicians and other healthcare professionals. Visit cribsiders.vcuhealth.org and search for this episode to claim credit.

Contact Us

Got feedback? Suggest a Cribsiders topic. Recommend a guest. Tell us what you think.

Contact Us

We love hearing from you.

Notice

We and selected third parties use cookies or similar technologies for technical purposes and, with your consent, for other purposes as specified in the cookie policy. Denying consent may make related features unavailable.

Close this notice to consent.