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NeuroblastomaTUMOR BORD

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History

10 month old male.

Previously healthy.

The history started 1 month before admission. Patient developed fever ,on-off, documented 39 C

relieved by antipyretics, with no history of sweating, norigor.

Hi

story of poor feedi

ng and decreased acti

vi

ty. 2 weeks before admission,the mother noticed that her

baby had left upper eyelid swelling.

The swelling increased in size and then the baby couldnot open his left eye.

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She seek medical advise at many peripheral centersin west bank, then she was referred to al-makassedhospital.

In the hospital the baby was found to had abdominalmass.

Abdominal u/s and then CT scan was done whichshowed abdominal mass originated from suprarenal

gland suspected to be neuroblastoma. FNA biopsy was taken from the mass and the

diagnosis was neuroblastoma.

The patientHB was 6 and he received PRBC.

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No history of vomitting,no history of diarrhea,no skin rash, no change in urine amount or

colour,no abnormal movement, no weightloss.

Developmental history: up to age.

Vaccination history : up to date according to

the Palestinian national vaccination program. Past- medical history and family are not

significant.

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Examination Vital signs:

-Temp.:37.1C axillary.- HR: 140 beat/m.

- RR: 25 breath/m.- B.P. 150/110.-O2 : 98%.

Growth parameters:- Wt.:8.5 kg- Length: 71CM-Head circumference: 46 CM.

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Growth chart: Wt./age:10th.percentile Length/age: 25th percentile. Head circumference/age: 50th percentile.

General appearance: Conscious,allert,pale,active,left eyelid swelling which cover eye and eye ball

cannot seen. No palpable LAP. No skin rash.

ENT: free.

Chest and heart: free.

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Abdomen

Distended, inverted umbilicus, visible veins.

Palpable mass can be felt on the left lumbar area, notattached to skin, not mobile,hard,smooth,ill definededges ,10x5cm ,upper border cannot be palpatedliver and spleen are not palpable.

The mass is dull by percussion.

Neurological examination: free.

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Investigations

CBC: -HB:7.5 - WBC:9.7 -N.63% - L.25%

CRP:164 -ESR:64 -LDH:3500 KFT : NL

LFT :NL

24 hour urine collection:

-Catecholamines:55 - VMA:3.5 -metanephr

ine:0.2(all are NL)

bone marrow aspiration and biopsy:

Metastatic neuroblastoma.

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Bone scan:

Increased uptake on skull, sternum and right greater

trochanter and distal end of femur.

MIBG scan:

- Multiple active lesions on skull, chest and abdomenconsistent with neuroblastoma.

- Left upper abdomen diffuse mass.

CT scan chest and brain.

CT scan abdomen which was done in al-makassidhospital was reviewed in our hospital.

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Treatment

Tumor lyses precaution:

-IV fluid 1.5 maintenance+NAHCO340meq/litre.-Allopurinol po.

Antihypertensive medications: according to

the advi

se of pedi

atri

c nephrologi

st)- Propronolol po.- Furosemide IV.

Chemotherapy according to protocol.

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Neuroblastoma Embryonic cancer of the peripheral sympathetic

nervous system with heterogeneous clinicalpresentation and course.

Epidemilogy: Third most common pediatric cancer.

8% of childhood malignancies.

Most common neoplasmi

ni

nfants.. 28%-39% of neonatal malignancies.

90% of cases are diagnosed by 5 yr. of age.

Incidence is slightly higher in boys and in whites.

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Pathology

Variable degrees of neural differentiation, ranging fromundifferentiated small round cells to mature ganglion cells

(ganglioneuroblastoma or ganglioneuroma) The tumor may resemble other small round cell tumors such as

rhabdomyosarcoma ,Ewing sarcoma, and non-Hodgkinlymphoma.

Genetics: Prognostic importance. Amplification of MYCN is strongly associated with advanced

tumor stage and poor outcome independent of stage and age. Hyperdiploidy confers better prognosis if the child is younger than 1

year of age at diagnosis.

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Clinical manifestations

May develop at any site of sympathetic nervoussystem tissue.

Most cases arise in the abdomen either in the adrenalgland or in retroperitoneal sympathetic ganglia.

Firm nodular mass palpable in the flank or midlinecausing abdominal discomfort.

Originates from cervical ,thoracic, or pelvic ganglia in

30% of cases. Metastatic disease can be associated with

fever,irritability,FTT,bone pain, orbital proptosis ,andperiorbital echymosis.

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Metastasis: long bones and skull ,BM ,liver,lymph nodes and skin. lung mets are rare.

Horner syndrome if location in sup. Cervicalganglion.

Spinal cord or nerve root compression ifinvade the neural foramina.

Opsomyoclonus. Increased sweating and hypertension by

producing catecholamines.

Secretory diarrhea by VIP.

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Diagnosis

Mass or multiple masses on plain radiograph ,CT or

MRI. HVA and VMA are elevated in 95% of cases.

Pathological diagnosis by biopsy.

Cytogenetic.

NB can be diagnosed without tumor biopsy ifneuroblasts are observed in BM and elevated HVAand VMA in urine.

Evaluation of metastatic disease should include BMaspirate and biopsies ,bone scan and MIBG scan.

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Treatment

Depends on age of patient at diagnosis ,stage ofdisease ,MYCN STATUS and shimada histology.

Low risk NB : surgery for stage1 and 2 andobservation for stage4S .

Intermediate risk NB : surgery , chemotherapy and insome cases radiation therapy.

High risk NB :induction chemotherapy to achieve

complete response or very good partial response ,with partial response ,resection of the primary tumorfollowed by focal radiation to the residual tumor.induction is then followed by high dosechemotherapy and autologous stem celltransplantation.