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Presentation on theme: "NEUROBLASTOMA TA OGUNLESI (FWACP)."— Presentation transcript:


2 Neuroblastoma is an embryonic tumor
Derived from primordial neural crest cells in the adrenal medulla and sympathetic ganglia. Some tumors may spontaneously regress or mature even without therapy. It is one of the small, blue, round cell tumors of childhood. [Rhabdomyosarcoma, Ewing sarcoma, non-Hodgkin lymphoma] TA OGUNLESI (FWACP)

3 EPIDEMIOLOGY Clinical frequency is approximately 1 case per ,000 children in the US. Neuroblastoma is more common in whites It is slightly more prevalent in boys than in girls. The male-to-female ratio is 1.3:1. It forms 8% of all cancers in children in US but 5% of childhood cancers in Nigeria TA OGUNLESI (FWACP)

4 EPIDEMIOLOGY The typical age at diagnosis is younger than that for Wilms tumor. Most common tumor in infancy 33% are diagnosed in children younger than 1 year. 80% of cases are diagnosed before age 4 Median age at presentation is 2 years TA OGUNLESI (FWACP)

5 AETIOLOGY This is unknown.
Fact is these tumors arise from primordial neural crest cells, which ultimately populate the sympathetic chain and the adrenal medulla. Most neuroblastomas produce catecholamines [Vanillymandelic acid (VMA) and Homovanillic acid (HVA)] & vaso-active intestinal peptides, as metabolic by-products TA OGUNLESI (FWACP)

6 CLINICAL FEATURES The tumor may arise anywhere along the sympathetic nerve chain and ganglia Frequently multiple General symptomatology Fever Malaise Anorexia Weight loss Toxic-look TA OGUNLESI (FWACP)

7 CLINICAL FEATURES These are related to the site of the primary tumor, metastases and the chemical effects of the metabolites Most cases (65%) occur in the abdomen, mostly in the adrenal glands and paraspinal sympathetic ganglion Most common sites of metastases are the regional lymph nodes, bone marrow, bone, liver and subcutaneous tissue TA OGUNLESI (FWACP)

8 CLINICAL FEATURES Presentations include: Craggy, hard abdominal mass
Massive hepatomegaly (from metastasis) Paraspinal masses extending into the chest (dyspnea) Paraplegia Large intra-thoracic masses causing respiratory obstruction Bone pain and limp TA OGUNLESI (FWACP)

9 CLINICAL FEATURES Blanching bluish, non-tender mobile skin nodules
Bumps on the scalp (metastasis to the skull bone) Severe anaemia and bleeding following bone marrow infiltration Intractable diarrhoea TA OGUNLESI (FWACP)

10 CLINICAL FEATURES Other presentations are:
Proptosis (retro-orbital deposits) Peri-orbital ecchymosis (raccoon eyes) from sphenoid bone involvement Bladder or bowel obstruction (either from pressure effect OR cord infiltration) Oedema of the lower limbs resulting from Inferior Vena Cava obstruction by retrospinal mass TA OGUNLESI (FWACP)

11 INVESTIGATIONS Full blood count to determine pancytopenia
Coagulation profile: Prothrombin time [PT], activated partial thromboplastin time [aPTT]) are deranged in liver involvement. The ESR is elevated. Urinary HVA and VMA are elevated (X3). CT scan & MRI Skeletal survey with bone scintigraphy Tumor Biopsy for Histology TA OGUNLESI (FWACP)

12 TREATMENT Localized disease can be totally excised
Surgery is not helpful in advanced disease Combination cytotoxic therapy is useful in advanced neuroblastoma cases using: Vincristine Cyclophosphamide Cisplatin Doxorubicin TA OGUNLESI (FWACP)

13 OTHER TREATMENTS The tumour is radio-sensitive but clinical trials are not yet conclusive. Autologous bone marrow transplantation for high risk cases. TA OGUNLESI (FWACP)

14 PROGNOSIS Poor long-term (5-years) survival rate (55%) is the rule
50% to 60% have metastasis at the time of diagnosis Poor prognostic factors include: Bone disease Age > 2years Abdominal primaries Advanced disease at diagnosis TA OGUNLESI (FWACP)


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