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Virtual Pediatric Hospital: Paediapaedia: Neuroblastoma Paediapaedia: Genitourinary Diseases

Neuroblastoma

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
May present with compression or involvement of adjacent structures, metastases, or become hormonally active. Most produce excess catecholamines which can cause three recognized clinical syndromes: 1) paroxysmal episodes of profuse sweating and flushing, headache, tachycardia, hypertension, 2) diarrhea refractory to conventional therapy, and 3) acute cerebellar encephalopathy (opsoclonus-myoclonus, ataxia). It is one of the most common malignant tumors of childhood, having the same frequency as Wilms. It incidence peaks in the first 5 years of life (85% ), with 50% of patients less than 2 years old..

Etiology/Pathophysiology:
Arises from neuroblasts anywhere within the sympathetic nervous system and adrenal medulla. Most common site is abdomen (60% ) and 70% of these are adrenal, 13% are in the thorax, 5% are in the neck and 4% are in the pelvis and 2% are in the posterior fossa and olfactory bulb. Spreads via direct invasion, blood, and lymphatics. Many have early bone marrow involvement. Skeletal metastases can be isolated foci or can be diffuse metaphyseal infiltration of the long bones. Hepatic metastases are also common.

Pathology:
Not applicable

Imaging Findings:
The abdominal plain film shows a calcified mass in 66%.

Ultrasound shows a solid mass that displaces the kidney, but does not distort it.

CT and MRI show the extent of the mass, with MRI demonstrating if there is any extension into the spinal canal.

Nuclear medicine bone scan to displays bone metastases.

DDX:

References:

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