Nutrition Support in

Pediatric Neuroblastoma
Stephanie Rodriguez, Dietetic Intern
Preceptor: Amy Hood, MPH, RD, CNSC

Learning Objectives
By the end of this presentation, you will be able to:
Describe neuroblastoma along with prevalent signs/symptoms.
Correctly perform a mid-upper arm circumference measurement.
Explain what the mid-upper arm circumference measurement can
indicate in the pediatric population.
Understand basic medical nutrition therapy interventions commonly
used in the pediatric oncology patient.
Interpret the results from a metabolic cart study and explain how these
studies can aid in feeding the critically ill child.

Introduction to Patient
3 year old M (JC)
Medical hx: premature birth at 32 wks gestational age
Lives at home with father, step-mom and 2 siblings.
Initial complaint: vomiting and stomach distention
Patient had been sluggish for 1 week PTA. Admitted to OSH after 1
episode of vomiting and father noticed large abdomen. Transferred to
UWHC.
Diagnosis differential: Wilms tumor vs. neuroblastoma
Final diagnosis: stage IV neuroblastoma with L pleural lung metastases

Stage IV Neuroblastoma
Cancerous tumor from immature nerve
cells (neuroblastoma).
Nerve tissue in adrenal glands, near
spine/neck, chest, abdomen, or pelvis
Stage IV - cancer has spread to other
parts of the body.
Signs/symptoms: lump in abdomen,
chest or neck, difficulty breathing,
bulging eyes, bone pain, darkness under
the eyes, weakness and paralysis
Diagnosis: physical/neurological exam,
urine/blood studies, bone marrow
biopsy, MRI/CT/US, tissue biopsy
http://www.medpagetoday.com/CelebrityDiagnosis/40510

Prevalence
1 every 7,000 live births - most common type of extracranial solid tumor in children
Abdominal tumors most common.
90% diagnosed under age 5.
70% of patients have metastatic disease at diagnosis.
5 year survival rate increased from 34 to 68% [in
children age 1-14] between 1975 and 2010.
National Cancer Institute. Neuroblastoma treatmentfor healthcare professionals. Dec. 1, 2015.

Factors of Prognosis
Age of diagnosis
Site of tumor
Tumor histology
Lymph node
involvement/metastases
How fast tumor is growing
How tumor responds to
treatment
National Cancer Institute. Neuroblastoma Treatment-for
healthcare professionals. Dec 1, 2015.

imgbuddy.com; http://www.cancernetwork.com/reviewarticle/neuroblastoma-biology-and-therapy/page/0/2

Initial Assessment (Day 2)

Anthropometrics
Admission weight: 14.6 kg (29 % ile, z-score= -0.55) measured day 0, bed scale, trunk edema
Weight day 2: 16.1 kg - standing scale, trunk edema, 1 foot
swollen
Tumor size: (CT from OSH) 18.9 cm x 15.7 cm x 13.2 cm
Height: 96.4 cm (20 % ile, z-score -0.84)
Mid-upper arm circumference: 14 cm (5 %ile, z-score -1.67)

Diet History
Typical Intake:
Breakfast: cereal or 2 eggs and 1 slice toast + 1 cup 1% milk
Lunch: PB&J (1 - 1.5 sandwich) or 1 can ravioli + water or juice
Snack: might be fruit snacks or fruit
Dinner: 1-2 chicken drumsticks or "good size" spaghetti + 1 cup
1% milk
Snack: occasional ice cream
Father reports that JC typically has a good appetite and is not a
picky eater.
Normal meal schedule: 3 meals per day plus snacks
Drinks 2 cups 2% milk daily.
Takes a daily gummy multivitamin.

Estimated Needs
Estimated nutrient needs (based on 15 kg):
Energy: 1085-1335 kcals (72-89 kcals/kg) using BMR x 1.3-1.6
Protein: 1.5 - 2 g/kg/d
Fluid: 1250 mL/d
Diagnosis: Inadequate oral intake related to nausea and early satiety as
evidenced by report of poor intake x 2 days and reported unintentional
weight loss over last few months.

*BMR (WHO) for males age 3-10= (22.7 x wt in kg) + 495

Interventions
1) NG tube to be placed during MRI day 4. Plan to start
EN in addition to oral intake. If unable to tolerate
gastric feeds, advance to post-pyloric feeds. If unable to
tolerate, consult for initiation of PN.
2) Start calorie counts.
3) Obtain twice weekly weights.
Bauer J, et al. Adv Nutr. 2: 67-77

Hospital Course

Day 2 - 8
NPO/Oral diet

Intermittently NPO.
No calorie data recorded. Appeared pt vomited after ~50% of
food/liquid intake.
Day 4: NG tube placed during MRI.
Day 5: JC pulled tube out. Father felt that he was eating better so
it did not need to be replaced. No EN feeds given.
Day 8: Admitted to PICU d/t hemodynamic and respiratory
monitoring during chemotherapy and enlarging pleural effusion.

Day 9 - 12

PN + NPO except medications

Day 9: Diagnosed with malnutrition (mild, acute) related to nausea, early satiety, and
emesis with large abdominal mass as evidenced by reported unintentional weight loss
(~6.5%), estimated intake meeting <25% of needs and MUAC z-score of -1.77.
Day 9: PN started at 50 kcal/kg/d (plan to advance to 70 kcal/kg/d).
Day 9-10: Received chemo regimen of vincristine, doxorubicin and cyclophosphamide.
Hematuria- concern for bleeding w/i tumor.
Day 10: Left IJ subcutaneous port removed. Double lumen Hickman catheter and chest
tube (for pleural effusion) placed.
Day 11: Small pneumothorax noted. PN adjusted to 69 kcal/kg/d.
Day 12: Patient developed fever. Started on empiric antibiotics (received cetriaxone &
vancomycine through day 14). PN adjusted to 68 kcal/kg/d.

Day 13-16

PN + NPO and sips of water

Day 14: Bridled NG replaced. PN adjusted to 70 kcal/kg/d.
Day 15: MRI showed mass increasing in size, despite recent
chemotherapy.
Day 15-16: Patient remained intubated/sedated with
pentobarbital and propofol post-MRI.
Day 15: Intubated energy needs estimated to be 57-69
kcal/kg (WHO 1.1 - 1.2) [Extubated needs 70-86 kcal/kg
(REE x 1.2 - 1.6)]. PN adjusted to provide ~60 kcal/kg while
intubated.

Day 17-19
PN + EN

Day 17: Patient sedated with propofol, dexmedetomidine, ketamine

and pentobarbital.
Day 18: EN initiated with Pedialyte at 5 mL/hr for 4 hours.
Day 18: EN advanced to Pediasure Peptide 1 Cal (no fiber) initiated at 5
mL/hr.
Plan to continue PN until EN can meet 75-80% of needs.
Day 19: Concern for typhlitis; U/S showed colitis; EN stopped.
Patient received an average of 80 kcal per day (x 2 days) from EN plus
an additional 900 kcals/d from PN

Day 20-29
PN

Day 21: sedated with pentobarbital, precedex and diluaded

Day 22: CT confirms typhlitis, small amount of shrinkage of abdominal
tumor
Day 23: diluaded D/C, start fentanyl
Day 28: MRI of abdomen/pelvis showing improving typhlitis, bilateral
hip effusions (cause expected to be infection) and possible pneumonia.
Patient with fevers and stable respiratory status but difficulty weaning
from ventilator. Worsening renal function noted day 28.
Day 29: PN adjusted to 55 kcal/kg/d while sedated and paralyzed.

Day 30-32
PN + Trophic EN

Day 30: Chest tube removed (renal status stabilized, mixed respiratory and
metabolic acidosis)
Day 30: Trophic feeds re-initiated- Pediasure Peptide 1 Cal (no fiber) at 5 mL/hr
Day 31: Patient had 1 bout of emesis. EN stopped until feeding tube confirmed to
be post-pyloric. Patient off pentobarbital. Now sedated with precedex, loraz,
ketamine, morphine, and phenobarbital.
Day 32: Patient spiked fever. Had been afebrile x 3 days and planned to start
round 2 of chemo. Chemo postponed.
Patient received a total of 121.1 kcal from EN. Receiving 825 kcal/d from PN (PN
at 55 kcal/kg/d while sedated and paralyzed).
Day 32: Metabolic cart study to assess patient needs.

Metabolic Cart Study

Results:
REE= 642 kcals
RQ= 0.91
Respiratory quotient= ratio of carbon
dioxide production/oxygen
consumption
Underfeeding decreases RQ
Overfeeding increases RQ
RQ >1 = reduced tolerance to feeding,
mild respiratory compromise

https://en.wikipedia.org/wiki/Indirect_calorimetry

Mclave SA. JPEN. 2003. 27(1):21-6.

Indirect Calorimetry
Determines energy expenditure by measuring CO2 production and
oxygen consumption.
Gold standard with metabolic carts being the standardized
equipment for determining resting energy expenditure in the
clinical setting.
Proven most reliable within various disease states/critical illness,
age and body size/distribution.
Psota & Chen:
Most commonly used in clinical practice: Harris-Benedict, Mifflin
St. Jeor, Owen, WHO
Predictive equations best for groups vs individuals.
Flancbaum, et al:
Predictive equations including the Fick method, Harris-Benedict,
Ireton-Jones, Frankenfield and Fusco did not correlate
(Flancbaum, et al).
http://health-and-you-life.com/monitoring-during-mechanical-venti
lation-indirect-calorimetry-1.html

Mid Upper Arm Circumference in

Pediatric Population
Assessment of nutritional status and predictor of mortality
Inexpensive, easy-to-use, less affected by fluid status
In children 6-59 months:
<110-115 mm= severe malnutrition
110/115 - 120/125 = moderate malnutrition
Not sensitive enough for routine monitoring.
Dairo, et al. Int J Biomed Sci. 2012. 8(2): 140-143

Mid-Upper Arm Circumference

Day 2: 14 cm (5 % ile, Z-score
-1.67)
Day 9: 13.9 cm (4 % ile, Z-score
-1.77)

Circumference of left upper arm

Measured halfway between tip
of shoulder and tip of elbow
http://www.gwh.nhs.uk/media/140852/community_nutriti
on_screening_tool___must__.pdf

Patient Update
Day 33 - Present

Plan Moving Forward

Keep patient comfortable.
Adjust sedation. Wean off ventilator.
Round 2 of chemotherapy.
Resume EN and advance to goal rate.
Tracheostomy 4-6 weeks.
Expected 4-6 month stay.

Palliative/Hospice Care and the

Dietitian
Palliative: approach that improves quality of life of patients and their
familiesthrough the prevention and relief of suffering (WHO)
Neither hasten or prolong dying, incorporate psychosocial and spiritual
aspects of care, provide a support system, enhance QoL
Hospice: special concept of care designed to provide comfort and support
to patients and their families when a life-limiting illness no longer responds
to cure-oriented treatments (Hospice Foundation of America)
Improve QoL, preserve dignity, provide comfort, control pain
Primary team: MD, NP, nursing, social work, spiritual services, pharmacy,
child life services

Palliative/Hospice Care and the

Dietitian
Malnutrition can contribute to poor performance status and
functional decline.
An RD in this setting is able to help:
Provide MNT to manage symptoms and improve QoL for patient
Facilitate discussion and education about nutrition care (with
families and primary team) and evidence-based interventions
Assist with decision-making regarding artificial nutrition and
hydration

In Conclusion
Stage IV neuroblastoma is a cancer of the immature nerve cells that
has developed in a primary location and spread to a distant
location.
Indirect calorimetry is considered the gold standard for estimated
energy needs in individuals.
Mid-upper arm circumference can indicate severe/chronic
malnutrition, as well as predict mortality risk. Not validated for
routine monitoring.
Interventions do not always produce the intended effect, but should
be continuously monitored and changed as needed.

National Cancer Institute. General information about neuroblastoma.

http://www.cancer.gov/types/neuroblastoma/patient/neuroblastoma-treatment-pdq. Updated December 4, 2015. Accessed
December 5, 2015.
National Cancer Institute. Neuroblastoma treatment- for health professionals.
http://www.cancer.gov/types/neuroblastoma/hp/neuroblastoma-treatment-pdq/#link/_571. Updated December 1, 2015.
Accessed December 5, 2015.
Exercise and Physical Activity Resource Center. Indirect calorimetry. http://www.ucsdeparc.org/index.php?
option=com_content&view=article&id=104&Itemid=82. Accessed December 6, 2015.
Flancbaum L, Choban PS, Sambucco S, Verducci J, Burge JC. Comparison of indirect calorimetry, the Fick method, and
prediction equations in estimating the energy requirements of critically ill patients. Am Society Clin Nutr. 1999. 69(3): 461-466.
Psota T, Chen KY. Measuring energy expenditure in clinical populations: rewards and challenges. Eur J Clin Nutr. 2013. 67(5):
436-442
American Society of Clinical Oncology. Neuroblastoma- childhood. http://www.cancer.net/cancer-types/neuroblastomachildhood/overview. Approved 3/2015. Accessed December 5, 2015.
Collins S, Duffield A, Myatt M. Mid-upper arm circumference (MUAC).
http://www.unsystem.org/scn/archives/adults/ch06.htm. Published July, 2000. Accessed December 6, 2015.
Dairo MD, Fatokun ME, Kuti M. Reliability of the mid-upper arm circumference for the assessment of wasting among children
among 12-59 months in Urban Ibadan, Nigeria. Int J Biomed Sci. 2012. 8(2): 140-143.
Trentham, K. Nutrition management of oncology patients in palliative and hospice settings. Onc Nutr Clin Prac. 2010. 241-245.
Bauer J, Jurgens H, Fruhwald MC. Important Aspects of Nutrition in Children with Cancer. Adv Nutr. 2011. 2: 67-77.
Mclave SA, Lowen CC, Kleber MJ, McConnell JW, Jung LY, Goldsmith LJ. Clinical use of respiratory quotient obtained from
indirect calorimetry. JPEN. 2003. 27(1):21-6.