Neuroblastoma

Powerpoint presentation for Neuroblastoma.

1. Dr Ranjeet Patil
(MS)
Paediatric Urology

2. •Most common extracranialsolid tumorof
childhood
•Over half of the children present with metastatic
disease
•Arise from cells of the neural crest that form the
adrenal medulla and sympathetic ganglia

3. •Adrenal gland
•Sympathetic chain
•Neck
•Thorax
•Retroperitoneum
•Pelvis

4. •8% to 10% of all childhood cancers
•10 cases per 1 million live births
•Most common malignant tumorof infancy
•Median age at diagnosis of 19 months
•*(Brodeurand Maris, 2006)
•There are no geographic or racial variations

5. •Autosomal dominant pattern of inheritance
•*(Knudson and Strong, 1972a; Robertson et al, 1991)
•In familial cases median age decresesto 9 months
•Hereditary neuroblastomapredisposition gene
chromosome 16p12-13
•* (Maris et al, 2002)
•Amplification of the N-MYC oncogene seen in roughly
20%
•*(Look et al, 1991; Muraji et al, 1993)
•Deletion of the short arm of chromosome 1
•*(Brodeur et al, 1992; Caron et al, 1996)

6. •Homer-Wright
pseudorosettes-consist of
eosinophilicneutrophils
surrounded by neuroblasts
•Schwann cell-Other type

7. HistopathologicFeatures and
Ages
Shimada Favourable Shimada Unfavourable Joshi
Stromarich, all ages, no
nodular pattern
Stromapoor, age <1.5 yr, MKI
>200
Grade 1-low mitotic rate and
calcification present
Stromarich, all ages, nodular
pattern
Stromapoor, age >5 yr Grade 2-either low mitotic
rate or calcification present
Stromapoor, age 1.5-5 yr,
differentiated,
MKI <100
Stromapoor, age 1.5-5 yr,
undifferentiated
Grade 3-neither low mitotic
rate nor calcification present
Stromapoor, age <1.5 yr, MKI
<200
Stromapoor, age 1.5-5 yr,
differentiated,
MKI >100
(MKI-mitosis-karyorrhexis
index)

8. •Abdominal pain
•Palpable mass
•fixed, hard abdominal mass
•Bone or joint pain
•Periorbitalecchymosis
•Cough
•Dyspnea
•Neurologic deficits
•Urinary retention
•Constipation
•Paraneoplasticsyndromes
•Paroxysmal hypertension
•Palpitations
•Flushing
•Headache
•Severe watery diarrhea
•Hypokalemia
•Acute myoclonic
encephalopathy

9. •Laboratory Evaluation
•Routine Investigations
•Haemoglobin-Anemiain bone mets
•Vanillylmandelicacid (VMA)-24 hour Urinary and Serum
•Homovanillicacid (HVA)
•Two bone marrow aspirates and two biopsies

10. •Imaging
•Ultrasound-First line, Detects incidentaloma
•Plain radiographs-calcified abdominal or posterior
mediastinalmass
•Computed Tomography-local extent of the primary
tumors, Invasion of the renal parenchyma
•Magnetic resonance imaging-evaluation of intraspinal
tumorextension, demonstrating the relationship
between the major vessels and the tumor
•Radionuclide bone scan
•Meta-iodobenzylguanidinescan

15. Evans et al PediatricOncology Group International NeuroblastomaStaging System
Stage I—tumorconfined to
organ orstructure of origin
Stage II—tumorextending in
continuitybeyond organ or
structure of origin, but not
crossing midline. Regional lymph
nodes onipsilateralside may be
involved.
Stage III—tumorextending in
continuitybeyond midline.
Regional lymph nodes maybe
involved bilaterally
Stage IV—remote disease
involvingskeleton,bone
marrow, soft tissue, andDistant
lymph node groups
Stage IVS—patients who would
otherwisebe stage I or II, but
who have remote disease
confined to liver, skin, or bone
marrow
Stage A—complete gross resection
ofprimary tumor, with or without
microscopicresidual. Intracavitary
lymph nodes not adheredto and
removed with primary tumor,
histologically freeof tumor. If
primary tumorin abdomen or
pelvis, liver histologically free of
tumor
Stage B—grossly unresected
primary tumor.
Nodes and liver same as stage A
Stage C—complete or incomplete
resectionofprimary tumor.
Intracavitarynodes not
adhered to primary tumor
histologicallypositivefor tumor.
Liver as in stage A
Stage D—any dissemination of
diseasebeyondintracavitarynotes
(i.e., extracavitarynodes, liver, skin,
bone marrow, bone)
Stage DS—infants <1 yrwith stage
IVSdisease
Stage I—localized tumorwith completegross excision
without microscopic residualdisease; representative
ipsilaterallymphnodes negative for tumormicroscopically
Stage IIA—localized tumorwith incompletegross excision;
representative ipsilateralnonadherentlymph nodes
negativefortumormicroscopically
Stage IIB—localized tumorwith or withoutcomplete gross
excision, with ipsilateralnonadherentlymph nodes
positive for tumor. Enlarged contralateral lymph nodes
must be negative microscopically
Stage III—unresectableunilateral tumorinfiltrating across
midline, with or withoutregional lymph node
involvement; orlocalized unilateral tumorwith
contralateralregional lymph node involvement; ormidline
tumorwith bilateral extension byinfiltration (unresectable)
or by lymph nodeInvolvement
Stage IV—any primary tumorwith dissemination
to distant lymph nodes, bone, bonemarrow, liver, skin, or
other organs
Stage IVS—localized primary tumorasdefinedfor stage I,
2IIA, or IIB with disseminationlimited to skin, liver, or
bone marrow

17. •Surgery
•Chemotherapy
•Radiation therapy

18. •Goals
•Establish the diagnosis
•Stage the tumor
•Excise the tumor(if localized)
•Provide tissue for biologic studies

19. •Surgical excision
•Children with stage I neuroblastomahave a disease-free
survival rate of greater than 90% after exicision
•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.

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

21. •Thoracic tumors-posterior-lateral thoracotomy
•Dumbbell-shaped tumorsthat enter the neural
foramina are generally treated initially with
chemotherapy

22. •Atelectasis
•Infection
•Ileus
•Haemorrhage

23. •Local control
•stage IV or bulky stage III tumors*
•*(Matthayet al, 1989; Castleberry 1991; Evans et al,
1996)
•Dose-15 and 30 Gy
•Intraoperative radiation therapy-unresectable
disease

24. •Chemotherapy
•Reserve laminectomy for children with progressive
neurologic deterioration (Katzensteinet al, 2001).
•Radiotherapy-avoided, because of its adverse effect
on growth of the spine.

25. THANK
YOU