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J Child Neurol. Author manuscript; available in PMC 2022 January 01.
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Published in final edited form as:

J Child Neurol. 2021 January ; 36(1): 79–82. doi:10.1177/0883073820955101.

Neuro-Oncology Training for the Child Neurology Resident

Fatema Malbari, MD1, Sonia Partap, MD2, Juliane Gust, MD PhD3, Elizabeth Duke, MD4,
Aimee Sato, MD3, Yasmin Khakoo, MD5, Nicole J Ullrich, MD, PhD6
1Texas Children’s Hospital, Baylor College of Medicine, Houston, TX
2Lucile Packard Children’s Hospital, Stanford University, Palo Alto, CA
3Seattle Children’s, University of Washington, Seattle, WA
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4Children’s National Medical Center, Washington, DC

5Memorial Sloan Kettering Cancer Center, Weill Cornell Medical College New York, NY
6Boston Children’s Hospital, Harvard Medical School, Boston, MA

Introduction/Background
Cancer is the leading cause of non-accidental death in children. In the United States, about 1
to 2 children per 10,000 are diagnosed with cancer each year.1 The two most common types
are leukemia and central nervous system (CNS) tumors, the latter being the most common
solid tumor and the largest cause of childhood cancer-related mortality.2 Over time, cure
of pediatric cancers has been improving and now approaches 80%.3 With improvements
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in therapeutics and overall survival, both short and long-term neurologic adverse effects
resulting from underlying disease and its treatments are better appreciated.4 Neuro-oncology
is a rapidly evolving subspecialty that involves the management of patients with primary or
metastatic central and peripheral nervous system neoplasms, as well as any other disorders
or complications affecting the nervous system that result either directly or indirectly from
CNS or systemic malignancies and related treatment (Table 1). Neurologists serve a critical
role in the multidisciplinary management of these complex patients. As leaders of the Child
Neurology Society Special Interest Group in Neuro-Oncology, we propose the following
considerations to promote sufficient exposure, minimize knowledge gaps and optimize
training experiences in neuro-oncology for child neurology residency programs.
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Current state of training

Current training models vary widely depending on whether the home institution has
a dedicated pediatric neuro-oncology program and/or a dedicated child neurology
subspecialist in neuro-oncology. At present, several training models exist. Child neurology
residents can 1) rotate with a child neurology-trained neuro-oncologist at an institution
with a neuro-oncology program, 2) rotate with different subspecialists at a hospital
with a dedicated neuro-oncology program, 3) evaluate neuro-oncology patients through

Corresponding author: Nicole Ullrich, MD, PhD, Boston Children’s Hospital, 300 Longwood Ave, Boston, MA 02115, Tel:
617-355-2751, Fax: 617-730-0282, [email protected].
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the neurology inpatient and outpatient services at a center without a dedicated neuro­
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oncology program. Hospitals with dedicated neuro-oncology programs generally have an

infrastructure for residents to receive didactic lectures as well as attend multidisciplinary
team meetings to discuss patient care. These tumor board meetings provide valuable
education in neuroradiology, neuropathology and overall management in neuro-oncology.
There are advantages to having the learning experience led by a child neurologist with
expertise in neuro-oncology and to being at a hospital with a dedicated neuro-oncology
program. These include education and experience provided from a neurological perspective
with higher patient volumes and more diverse and complex cases that are often seen at these
centers. A trainee’s experience may not be as integrated and comprehensive at institutions
without a dedicated neuro-oncology program, but we propose ways to improve this learning
experience.
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Accreditation Council for Graduate Medical Education (ACGME) guidelines

As per the updated ACGME guidelines for child neurology (ACGME-approved focused
revision: February 3, 2020; effective July 1, 2020), residents should receive teaching in
neuro-oncology by faculty members or consultants with expertise in this field. In addition, as
part of the general educational program, residents must have a broad range of structured
didactic activities. ACGME competencies must be incorporated into the curriculum,
specifically: professionalism, patient care and procedural skills, medical knowledge,
practice based learning and improvement, interpersonal and communication skills and
systems based practice. Residents are also required to receive instruction and experience
in pain management, demonstrate knowledge in neuropathology, neuroimaging and
neuropsychology, as well as demonstrate competence in the appropriate and compassionate
use of palliative care. Exposure to neuro-oncology would fulfill many of the ACGME
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guidelines for child neurology.

The core competencies in Child Neurology related to training in Neuro­

oncology
Patient care:
The possibility of a CNS tumor challenges skills in interviewing, developing and carrying
forth patient management plans and counseling and education of families. This is
particularly true in developing skills in approaching patients and parents with the discussion
of a CNS tumor. The resident will be able to gather accurate and essential information
from all sources, including medical interviews, physical examinations, medical records, and
diagnostic/therapeutic experiences.
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Medical knowledge:
This is a fertile area for residents to practice localization, pathologic investigation and
diagnostic skills for primary and secondary brain and spinal cord tumors as well as treatment
and the early and late neurologic sequelae.

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Interpersonal and communication skills:

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Residents will have the opportunity to develop and maintain compassionate therapeutic
relationships that can help to guide families through diagnosis and treatment. In addition,
they will learn to participate within the multidisciplinary team to improve and optimize care.

Professional issues:
Residents will learn to deal ethically and professionally with patients and families who have
a wide range of understanding of neurologic issues and prognosis and with families who
need additional information to make diagnostic decisions regarding the care of children
whose prognoses may be poor. Training also includes the discussion of complex medical
issues, treatment of disease, supporting children and their families during an acute medical
crisis, and how to support them when there is a terminal diagnosis or a need for end-of-life
care.
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Systems-based practice:
Residents will recognize that the effective diagnosis and treatment of CNS tumors is based
on collaboration with pathology, oncology, neurosurgery, rehabilitation medicine and other
professionals. Increasing the interactions with and understanding of the complex system will
allow residents to be effective advocates for their patients.

Proposed approaches to incorporate into current training

The child neurology resident should receive training and exposure to neuro-oncology
which encompasses a common set of training experiences. This training should include
principles of diagnosis and management of primary and secondary central and peripheral
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nervous system neoplasms, acute and long-term neurologic complications of cancer and
related disorders, side effects of treatments and supportive care measures. Trainees should
be familiar with hereditary disorders that predispose to CNS tumors.5–8 These can be
accomplished at all types of training programs by incorporating a didactic lecture series as
well as expanding the residents’ clinical experiences by providing increased exposure to this
patient population.

Medical Knowledge/Learning Objectives:

The core didactic and practical learning objectives for the child neurology trainee are
delineated in Table 2. The didactic learning experience should consist of a core lecture
series covering: 1) Overview of neuro-oncology including clinical presentation, diagnostic
imaging, pathology, treatment, neurologic complications from tumor and treatment, 2)
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Neurocutaneous syndromes, 3) CNS tumors in familial cancer predisposition syndromes, 4)

Cancer therapy induced neurotoxicity, 5) Long term neurologic sequelae from CNS tumors
and associated therapy, 6) Goals of care, Delivering difficult news, end of life care. This
lecture series could be integrated in the dedicated neuro-oncology rotation or as part of a
broader lecture series provided throughout the year for child neurology residents.

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Patient care:
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The clinical experience must include practice in both the inpatient and outpatient areas.
These experiences include the management of patients with primary neuro-oncologic
problems and consultations requested by other services (ie oncology, pediatrics, neurology,
ophthalmology, physical medicine and rehabilitation, neurosurgery, radiation oncology and
neuropsychology). The ideal experience is one in which the resident provides supervised
consultations of patients with primary CNS tumors and neurologic complications in children
with systemic cancer, as well as those of stem cell transplant and cellular therapies, although
the latter may not be feasible at all training sites. The neurologic issues can be divided
roughly into two groups: direct cancer related toxicity and indirect neurologic effects of
treatment (Table 1). At presentation, the most common symptoms of CNS tumors include
headache, seizures, changes in vision, ataxia and altered mental status.9 Treatment related
toxicities can occur from surgery, chemotherapy, radiation and immunotherapy and can
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occur at any time during or even many years after completion of therapy. The most common
reasons for inpatient neurologic consultation in pediatric patients with cancer are altered
mental status, seizures, pain and headache management and peripheral nervous system
issues (sensory and motor).9 The spectrum of neurologic symptoms also includes diagnosis
and treatment of toxic, nutritional or metabolic encephalopathy, chimeric antigen receptor
T cell (CAR-T) therapy, CNS and systemic infections, cerebrovascular disease, increased
intracranial pressure and paraneoplastic disease. As many novel therapies have only recently
been used in the pediatric setting, the toxicities in children are not well known.

Residents could rotate through different specialties such as neuropathology, neuroradiology,

radiation oncology, oncology, and neurosurgery to enhance their education in neuro­
oncology. A rotation through neuropathology will supplement the teaching of basic
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histopathology, immunohistochemistry and molecular biology. Trainees will be expected

to have an understanding of the classification and histopathology of CNS neoplasms.
This should include a basic understanding of the molecular mechanisms that underlie
the most common tumors, especially low and high grade gliomas, embryonal tumors
and ependymomas. Many institutions have a tumor board patient review session for
the multidisciplinary management of these patients which child neurology residents
should attend. Through a rotation in neuroradiology, residents will be able to recognize
the appropriate neurodiagnostic imaging needed for evaluation of CNS tumors. By
rotating through neurosurgery, oncology and radiation oncology, trainees will gain basic
knowledge in the general approach to the standard and experimental treatment of primary
CNS tumors and a general overview of medical neuro-oncological therapies, including
traditional treatments: surgery, radiation, chemotherapy, as well as novel therapies, among
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them molecularly targeted agents and immunotherapy. This will include participation in
interdisciplinary management of patients with neuro-oncologic disorders. When feasible,
the resident will gain exposure to clinical trial development and its relevance to the use of
targeted and novel therapeutics.

Survivors of childhood cancer should be followed into adulthood and monitored for
multisystemic effects of treatment. Long term neurologic sequelae include, but are not
limited to, difficulties with cognitive function (in particular, difficulties with memory,

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processing speed and attention), hearing loss, visual field deficits, motor deficits,
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peripheral neuropathy, and sleep disorders (http://www.survivorshipguidelines.org/pdf/2018/

COG_LTFU_Guidelines_v5.pdf).

Conclusions
The field of Neuro-oncology is a diverse and complex one. Neurologists serve a critical
role in the multidisciplinary management of these complex patients, and it is important to
optimize their specialized training to care for this population whether as a neuro-oncology
subspecialist or general child neurologist. Training programs should use the opportunity
to give residents exposure to these patients following a core content and common set of
training experiences; potential approaches could be as described above. Neurology residents
are often the first subspecialist a family may meet after presenting to the emergency room
with an undiagnosed brain or spinal cord tumor tumor. Therefore, astute clinical acumen
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in localization and diagnosis, as well as ability to compassionately disclose findings on

neuroimaging is critical to their training. Prompt recognition and diagnosis of neurologic
symptoms in a patient with cancer may prevent permanent disability or death. No matter
the timing, neurologic complications of cancer can be distressing and difficult for patients,
families and caregivers and can substantially diminish the quality of life. The aim is to
prepare the child neurology resident to provide compassionate care that optimizes patient
function and quality of life.

References
1. Ullrich NJ. P SL. Neurologic Complications of Pediatric Systemic Cancer. In: Schiff DAI, Wen P
(eds) ed. Cancer Neurology in Clinical Practice. Totowa, NJ: Springer, Cham; 2018:607–619.
2. Dang M, Phillips PC. Pediatric Brain Tumors. Continuum (Minneapolis, Minn.). 2017; 23(6,
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Neuro-oncology):1727–1757.
3. Shapiro CL. Cancer Survivorship. The New England journal of medicine. 2018; 379(25):2438–
2450. [PubMed: 30575480]
4. Waber DP, Pomeroy SL. Introduction: survivors of childhood cancer: the new face of developmental
disabilities. Dev Disabil Res Rev. 2008; 14(3):183–184. [PubMed: 18924153]
5. Ranger AM, Patel YK, Chaudhary N, Anantha RV. Familial syndromes associated with intracranial
tumours: a review. Child’s nervous system : ChNS : official journal of the International Society for
Pediatric Neurosurgery. 2014; 30(1):47–64.
6. Ullrich NJ. Neurocutaneous Syndromes and Brain Tumors. J Child Neurol. 2016; 31(12):1399–
1411. [PubMed: 26459515]
7. Hottinger AF, Khakoo Y. Neurooncology of familial cancer syndromes. J Child Neurol. 2009;
24(12):1526–1535. [PubMed: 19955345]
8. Ullrich NJ. Inherited disorders as a risk factor and predictor of neurodevelopmental outcome in
pediatric cancer. Dev Disabil Res Rev. 2008; 14(3):229–237. [PubMed: 18924162]
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9. Armstrong C, Sun LR. Neurological complications of pediatric cancer. Cancer Metastasis Rev.
2020.

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Table 1.

Neurologic complications of cancer in children

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Direct, Cancer-related toxicities Indirect/Treatment related toxicities

Focal motor/sensory symptoms Chemotherapy induced peripheral neuropathy

Seizure/Epilepsy Seizure/Epilepsy

Headache Headache

Hydrocephalus (obstructive/non-obstructive) Vasculopathy

Movement disorder Radiation necrosis

Visual acuity/visual fields changes Drug specific toxicities (ie retinal changes)

Neuroendocrine dysfunction Neuroendocrine dysfunction

Cognitive changes Cognitive changes

Local compression/mass effect Central nervous system infection

Metastatic/leptomeningeal disease Paraneoplastic syndromes

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Hearing loss Hearing loss

Posterior fossa syndrome

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Table 2.

Learning Objectives related to Neuro-oncology for the Child Neurology Trainee.

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1. Residents will demonstrate an understanding of the epidemiology of childhood CNS tumors and prognosis of the subtypes

2. Residents will understand the typical presenting signs and symptoms of a primary or secondary CNS tumor

3. Residents will obtain and document the history and physical examination of a child/adolescent with a primary or secondary CNS tumor,
including neuroimaging and pathology

4. Residents will demonstrate an understanding of the World Health Organization classification’s system, histopathology of CNS tumors,
and will participate in neuropathologic review

5. Residents will understand the application of neuroimaging

6. Residents will learn to distinguish CNS tumors from potential mimickers such as demyelinating disease, vascular malformations and
infections

7. Residents will recognize the findings and associated tumor-related complications of neurocutaneous disorders, specifically
neurofibromatosis type 1, neurofibromatosis type 2, tuberous sclerosis complex, von Hippel-Lindau disease

8. Residents will attend outpatient neuro-oncology clinic and neurocutaneous clinic, if feasible. Other options include attending general
neurology clinic, genetics clinic, and neurosurgery clinic
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9. Residents will provide consultation for neurologic issues, including seizures, raised intracranial pressure, headache, infection, peripheral
neuropathy, and vasculopathy

10. Residents will use a developmental framework to evaluation of children treated for a primary or secondary CNS tumor and refer when
appropriate for hearing assessment, visual acuity/visual field testing, neuroendocrine testing, neurocognitive assessment, school-based
assessments

11. Residents will learn to diagnose and manage acute and long-term neurologic complications of the tumor and treatment

12. Residents will gain competency in identifying patient populations at risk of specific long-term complications based on treatment
intervention and age of treatment

13. Residents will develop an understanding of issues related to families in crisis and end of life
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