Good Afternoon

Case report

Central nervous system neuroblastoma

with infiltration to nasal cavum
By
Luh Witari Indrayani
Scientific advisor
dr. I Gde Ardika Nuaba, Sp.T.H.T-K.L(K)FICS

Introduction
Neuroblastoma is a neoplasma derived
from embryonic neural cells
One of the most common extracranial
solid tumors are found in children and
are rarely found in adults

Prevalence : 8-10%of all children cancer, 90%

were diagnosed before age 5

Introduction....
Neuronerve cells and blastoma a cancer that
affects the immature or developing cells
Tumor can regress spontaneously or very
aggressive and unresponsive to intensive
multimodal therapy
Spontaneus regression and transform to benign
tumor especially in children under 1 year old

`
Introduction...
The outcome of patients
neuroblastoma remains ugly

with

aggressive

Clinical manifestations of the neuroblastoma is

related to the location of tumors and metastases
Survival rate of infants with low stadium neuroblastoma
exceeds 90% and the infant with metastatic disease have
a number of long-term survival of 50%

Children with low stage disease generally

have an excellent prognosis, regardless of
age
The older age of the patient and further
spread the disease poor prognosis

Nose Anatomy

Anatomy...

Septum blood supply

Etiology
Unknown
Cytogenetic abnormality 80% of cases
including the deletion of partial short arm of
chromosome 1,anomalies of chromosome
17

Epidemiology
Neuroblastoma representing 8-10% of
all cancers in children
About 600 new cases are diagnosed each
year in the United States

15% of cancer deaths in children

Epidemiology...
The average age of children which
diagnosed with neuroblastoma is 22 months
and 90% of cases are diagnosed at the age
of 5 years

Patophysiology
Neuroblastoma arises from primordial neural
crest cells that migrate during embryogenesis
to form the adrenal medulla and sympathetic
ganglia
Neuroblastoma occur in the adrenal medulla
and the sympathetic ganglia, especially in
the
retroperitoneum
and
posterior
mediastinum

Histology
Neuroblastoma is composed of small
neuroblasts mature, solid uniform cells, the
nucleus and cytoplasm slightly hyperchromatic

Cell differentiation has the appearance of a

more mature ganglion cells and nucleoli
eosinophilic sitoplasma
The number of neutrophils is also a hallmark of
tumor differentiation

Biologic marker

Potential prognostic marker including:

1.

BIRC (associated with apoptosis)

2.

CDKN2D (related to the cell cycle)

3.

SMARCD3 (related to the activation

of transcription)

Symptom

About half of neuroblastoma tumor arising in

the adrenal gland and most of the rest
coming from paraspinal sympathetic ganglia

Metastases going through local invasion,

haematogenous or lymphogen.

The organ most commonly addressed by

the process of metastasis are the regional
or distant lymph nodes, long bones and
skull, bone marrow, liver and skin

Symptom...
According to Willie (2008):

Retroperitoneal neuroblastoma

Mediastinal neuroblastoma

Neck neuroblastoma

Pelvic neuroblastoma

CNS neuroblastoma

Neuroblastoma in the central nervous

system

A rare case
Horten and Rubinstein stated that the
incidence of neuroblastoma in the central
nervous system is only one case every
decade
Neuroblastoma in primary central nervous
system mostly occurred in the first decade

Primary neuroblastoma is characterized by

symptoms and signs of increased
intracranial pressure
Tumors can spread rapidly and these
tumors are often large

(International Neuroblastoma Staging

System/INSS)

Stage 1
Tumor
limited
to
the
primary
organ,
macroscopically complete resection, with or
without recurrent microscopically. No ipsilateral
regional lymph nodes enlargement
Stage 2A
Limited tumor surgery, no ipsilateral regional
lymph nodes enlargement
Stage 2B
Limited tumor surgery with ipsilateral regional
lymph nodes enlargement

Stage 3
Non operable tumor, tumor already spread
to surrounding organs

Stage 4
Primary tumors already spread to distant
lymph nodes, bone, bone marrow, liver, skin
or other organs

Stage 4S
Age <1 year, tumor metastasis to the skin,
liver, bone marrow but without bone
metastases

Neuroblastoma in adult patient

Rare in adults
World wide 100 cases reported in the
literature
Adult patients with metastatic neuroblastoma
usually has a very bad prognosis
Patients with age older than 18 months at
the time of first diagnosis usually have a
poor outcome
Slower disease progression

Neuroblastoma in adult...

Some cases progress with slow progressive

or recurrent relapses over the years

N-myc amplification is rare in adults

There is no consensus on the treatment of

neuroblastoma in adult patients

Surgery and radiation therapy may be

sufficient for local disease

For
metastatic
disease,
combination
chemotherapy with or without radiation
therapy may be quite effective

In a study of 25 adult patients with

neuroblastoma, 16% occur in the pelvis,
68% in the retroperitoneal or adrenal gland

Diagnosis
Anamnesis, physical examination and ancilliary
examination.
Ancilliary examination :

Laboratorium

1.

Lactate dehydrogenase

2.

Ferritin

3.

Neuron spesific enolase

4.

Cathecolamin

Diagnosis...

Radiography
Chest X-raythorax neuroblastoma or
metastasis
Ultrasonography
CT scan
MRI
Scintigraphy
Bone marrow examination

Management

Children with good prognosis generally does

not require treatment or only minimal
resection

Stage I tumor resection

Stage II surgery, chemotherapy and

sometimes with local radiotherapy

Stage IVS high number of spontaneous

regression, low-dose chemotherapy and
strict observation

Management...

Stage III and IVintensive therapy,

including chemotherapy, radiation therapy,
surgery, bone marrow transplantation or
allogeneic
bone
marrow
rescue,
metaiodobenzilquainid
(MIBG)
and
immunotherapy with monoclonal antibodies
specific to neuroblastoma

Complication

Complicationsearly tumor metastases to

various organs limfogen through the lymph
nodes and hematogenous to the bone
marrow, bone, liver, brain, lung and others

Bone metastases cranial bone or the

long bone extremities

Metastases to the bone marrow

anemia, bleeding and trombositopenia

Prognosis
5 years survival rate is 60%
Sometimes reported spontaneus regretion
Age an stage of the disease
The older age of the patient and further
spread of the diseasepoor prognosis

Case report
Patient identity

Name

: NNS

Age

: 42 y.o

Sex

: Female

Address

: Ungasan, Jimbaran, Bali

Occupation : Housewife

Anamnesis

On 19th April 2016 patient come to ENT

clinic Sanglah hospital with chief complaint
headache and stuffy nose on the left side
since 2 months ago

Patients previously treated at the Sanglah

Hospital April 2016 because of bleeding
from the left nostril

History of fever, cough, runny nose was

denied

Anamnesis...

Patients also
headache

Patients went to the ENT specialist :

complained

of

frequent

Biopsy suspected neuroblastoma

Immunohistochemistry positive S100 and
NSE 8.94

Physical examination

Vital sign : within normal limit

Ear

: within normal limit

Nose

: visible mass in the left nostril

Throat

: within normal limit

Neck

: no enlarged lymphnodes of
the neck

Nasoendoscopy

CT-scan

MRI

Laboratory and chest X-ray

within normal limit
Consulted to Internal medicine
and Anesthesiology department
ASA 1

Joint operation with Neurosurgery department

ENT departmenttumor extirpation with FESS
approach
Neurosurgery departmentTumor resection

May

th
20 ,2016

May

th
20 ,

2016

Post operation

Post operation in ICU :

Ceftriaxone 2x1 gram intravena

Phentanyl via syringe pump
Phenytoin 3x100 mg intravena

Dexamethasone 3x10 mg intravena

On 31st May 2016patient was allowed to

go home with oral therapy paracetamol
3x500 mg and levofloxacine 1x500 mg

Histopatology :
CNS Neuroblastoma with
infiltration to nasal cavum

Discussion
literature

case

Neuroblastomasolid
tumor in children
Very rare in adult, worldwide
less than 100 cases
Male > female

Female
Adult
42 y.o

Discussion
literature

case

CNS
Neuroblastomamostly occured in the first
decade

42 y.o

Discussion...

literature

Case

Mostly from adrenal gland

CNS neuroblastomavery
rare only 1 case per one
decade

CNS Neuroblastoma with

infiltration to nasal cavum

Discussion...

literature

Case

The symptom often simillar

with other disease according
to the site of the tumor and
the metastase

Headache and stuffy nose

since 2 months ago
Nose bleeding
No sign and symptom of
metastasis

Discussion...
literature
Anamnesis

Physical
examination

Ancilliary
examination

case
Headache, stuffy
nose and
nosebleed

ENT examination

Nasoendoscopy,
CT-scan, MRI
and biopsy

Discussion...
literature

case

Stage Itumor resection

Stage IIsurgery with or
without chemotherapy and
local radiotheraphy

Tumor resection
joint operation with
neurosurgery
department

Stage III and

IVsurgery,chemotherapy,
radiotherapy,bone marrow
transplantation,etc.

No symptom and
sign of
metastaseno need
chemotherapy and
radiotheraphy

Conclusion
Conclusion
Neuroblastoma is a neoplasm derived from
embryonic neural cells with unknown etiology
One of the most common solid tumors are found in
children and rarely found in adults
CNS Neuroblastomamostly occured in the first
decade
It can be managed with tumor resection,
chemotherapy, radiotherapy and bone marrow
transplantation

Conclusion...
It

was reported one case female, 42 y.o with

CNS neuroblastoma which infiltrated to nasal
cavum, no sign and symptom of metastase
and managed with tumor resection