Neuroblastoma is the most common cancer in babies and the third-most common cancer in children after leukemia and brain cancer, proper diagnosis, treatment must be done in appropriate time. As it a fatal condition psychosocial support is most important for patient and family.

1. Presented by
BHARATI SAHU
MSC.(N) 2ND YEAR
SNC,BBSR
NEUROBLASTOMA

2. Introduction
 Neuroblastoma is a malignant (cancerous) tumor of
the peripheral sympathetic nervous system that
develops from immature nerve cells (neural crest )
found in several parts of the body.
 Neuroblastoma is a pediatric neoplasm that is the most
common cancer diagnosed during infancy.
 It most commonly arises in and around the adrenal
glands and sits atop the kidneys. However, it can also
develop in other areas.

3. Incidence
 Neuroblastoma is the most common extra cranial solid
tumor in children, accounting for 7% to 8% of all
childhood cancers. Most common malignant tumor of
infancy.
 It is disease of early childhood, with two-thirds of cases
developing before the age of 5 years.
 The prevalence is about 1 case per 7,000 live births.
 Neuroblastoma is slightly more common in boys than in
girls.
 There are no geographic or racial variations

4. Sites of Neuroblastoma
Sympathetic chain
Neck
Thorax
Retro-peritoneum
Pelvis
Adrenal gland
 75% arise in the retro-peritoneum, 50% in
the adrenal, and 25% in the par-vertebral
ganglia.

5. Sites of Neuroblastoma

6. CAUSES
Neuroblastoma begins in neuroblasts - immature
nerve cells that a fetus makes as part of its
development process. In most cases these neuroblasts
mature or disappear. Others, however, form a tumor
(neuroblastoma).
Associated with mutation in
MYCN oncogene
Tumor suppressor gene-PHOX2B,ALK,others
on chromosome 1 & 11
The cause of the tumor is unknown, though children
with a family history of neuroblastoma are more
likely to develop the disease.

7. CLINICAL FEATURES
 Most children present
with abdominal pain or
a palpable mass.
 Enlarged abdomen
(from a large tumor or
excess fluid)
 Fever
 Malaise/fatigue
 Flushed, red skin

8. Cont…
 Bone or joint pain
 Respiratory symptoms of cough or Dyspnea.
 Neurologic deficits as a result of cord compression.
 Periorbital ecchymosis
or Raccoon eyes
(Dark circles, similar to
bruises, around the eyes)

9. Other symptoms
 Swelling in the legs
 Eyeballs that seem to protrude
 Unexplained weight loss
 Profuse sweating
 Rapid pulse
 Muscle weakness
 Bladder weakness

10. Cont……
Other syndromes:
 Blueberry muffin baby

11. Cont….
 Pepper syndrome
(Neuroblastoma of adrenal gland with
metastases in the liver)
 Hutchinson syndrome(limping and irritability
due to skeletal metastases
from neuroblastoma.)
 Horner syndrome( miosis ,ptosis, anhidrosis )

12. Cont…
 Paraneo-plastic syndromes
 Paroxysmal hypertension
Palpitations
Flushing
Headache
Severe watery diarrhea
Hypokalemia
Acute myoclonic encephalopathy

13. Diagnostic evaluation
Laboratory Evaluation
 Routine Investigations
 Haemoglobin-Anemiain bone mets
 Vanillylmandelicacid (VMA) & Homovanillicacid (HVA)
-24 hour Urinary and Serum
 Two bone marrow aspirates and two biopsies

14. Cont…..
Imaging
•Ultrasound-First line, Detects incidentaloma
•Plain radiographs-calcified abdominal or posterior
mediastinal mass
•Computed Tomography-local extent of the primary
tumors, Invasion of the renal parenchyma
•Magnetic resonance imaging-evaluation of intraspinal
tumor extension, demonstrating the relationship
between the major vessels and the tumor
•Radionuclide bone scan
•Meta-iodobenzylguanidine scan

16. staging
International Neuroblastoma Staging System
 Stage 1: Localized tumor with complete gross excision,
with or without microscopic residual disease;
representative ipsilateral lymph nodes negative for
tumor microscopically (nodes attached and removed
with the primary tumor may be positive)
 Stage 2A: Localized tumor with incomplete gross
excision; representative ipsilateral non-adherent lymph
nodes negative for tumor microscopically

17. Cont…
 Stage 2B: Localized tumor with or without complete gross
excision, with ipsilateral non-adherent lymph nodes positive
for tumor. Enlarged contra lateral lymph nodes must be
negative microscopically.
 Stage 3: Unrespectable unilateral tumor infiltrating across
the midline, with or without regional lymph node
involvement; or localized unilateral tumor with contra lateral
regional lymph node involvement; or midline tumor with
bilateral extensity by infiltration (unrespectable) or by lymph
node involvement

18. Cont…
 Stage 4: Any primary tumor with dissemination to
distant lymph nodes, bone, bone marrow, liver, skin,
and/or other organs (except as defined for stage 4S)
 Stage 4S: Localized primary tumor (as defined for stage
1, 2A, or2B), with dissemination limited to skin, liver
and/or bone marrow (limited to infants <1 year of age)

19. Treatment
 Surgery
 Chemotherapy
 Radiation therapy
 High-dose chemotherapy/radiation therapy and
stem cell transplant
 Retinoid therapy
 Immunotherapy

20. surgery
Surgical excision
•Children with stage I neuroblastoma have a disease-free
survival rate of greater than 90% after excisions
•Low-Risk Disease (Stages I, II, and IV-S)
•Complete excision should be undertaken only when
there is not a concern for undue morbidity to vital
organs or the patient
•Sacrifice of major organs such as the kidney or spleen
should be avoided, especially in children less than one
year of age.

21. Abdominal tumors-generous transverse incision
•Ligation of feeding vessels, Tumor excised
•Lymph node sampling
•noncontiguous nodes above and below the tumor
•Liver biopsy indicated if Stage 4S
•Patients with incomplete resection initially-
delayed attempt at resection of residual tumors
undertaken at the end of induction chemotherapy
•Surgery is not indicated for those patients who have
progressive disease at this time

22.  Thoracic tumors-posterior-lateral thoracotomy
•Dumbbell-shaped tumors that enter the neural
foramina are generally treated initially with
chemotherapy.
 complication
•Atelectasis
•Infection
•Ileus
•Haemorrhage

23. RADIO THERAPY
 For local control in neuroblastoma
 Stage IV or bulky stage III tumors*
 Doses of external beam irradiation used have
ranged between 15 and 30 Gy (gray).
(depending on the patient’s age, location, and
extent of residual disease)
 Intraoperative radiation therapy- unresectable
disease

24. Chemotherapy
Usually includes a combination of drugs
 Cyclophosphamide or ifosfamide
 Cisplatin or carboplatin
 Vincristine
 Doxorubicin (Adriamycin)
 Etoposide
 Topotecan
 Busulfan and melphalan
The most common combination of drugsincludes
carboplatin (or cisplatin), cyclophosphamide,
doxorubicin, and etoposide

25. Retinoids therapy
 Retinoids are chemicals that are related to
vitamin A. They are known as differentiating
agents because they are thought to help some
cancer cells mature (differentiate) into normal
cells.
 In children with high-risk neuroblastoma,
treatment with a retinoid called 13-cis-retinoic
acid(isotretinoin) reduces the risk of the cancer
coming back after high-dose chemotherapy and
stem cell transplant. Most doctors now
recommend 6 months of 13-cis-retinoic acid after
the transplant. This drug is taken as a capsule,
twice a day for 2 weeks, followed by 2 weeks off.

26. Immunotherapy
 Immunotherapy is the use of medicines to help a
patient’s own immune system recognize and
destroy cancer cells more effectively.
 Monoclonal antibodies called dinutuximab
(Unituxin) are man-made versions of immune
system proteins that can be made to attack a very
specific target. They can be injected into the body
to seek out and attach to cancer cells.
 This antibody can be given together with
cytokines (immune system hormones) such as
GM-CSF and interleukin-2 (IL-2) to help the
child’s immune system recognize and destroy
neuroblastoma cells.

27. Nursing Care of a Child with Neuroblastoma
Understanding the implications of nursing care and what is actually
going to be beneficial to the patient is going to be completely
dependent on each patient's case.
Protect Against Infection
 Monitor blood pressure, temperature, heart rate, respiration rate,
oxygen saturation and pain
 Monitor lab values (electrolytes, blood cell counts, etc.)
 Teach patient and family about the importance of frequent hand
washing
Promote Adequate Nutrition
 Assess and document weight daily; record intake and output as
necessary
 As appropriate, meals should include food that the child enjoys
 Be present during mealtimes so that child does not have to eat alone

28. Prognosis
 Between 20% and 50% of high-risk cases do not
respond adequately to induction high-dose
chemotherapy and are progressive.
 The majority of survivors have long-term effects from
the treatment.
 Survivors of intermediate and high-risk treatment
often experience hearing loss.
 Growth reduction, thyroid function disorders, learning
difficulties, and greater risk of secondary cancers
affect survivors of high-risk disease.
 An estimated two of three survivors of childhood
cancer will ultimately develop at least one chronic and
sometimes life-threatening health problem within 20 to
30 years after the cancer diagnosis.

29. SUMMARY

30. CONCLUSION
Neuroblastoma is the most common cancer in
babies and the third-most common cancer in
children after leukemia and brain cancer,
proper diagnosis, treatment must be done in
appropriate time. As it a fatal condition
psychosocial support is most important for
patient and family.