Health is Wealth

History

Neuroblastoma was first described by Sir Rudolf Virchow in 1864 and at that time it was

referred as glioma.

In 1891 Marchand histologically linked Neuroblastoma to sympathetic ganglia.

Herxheimer,In1914 gave the substantial evidence of neural origin by demonstrating the fibrils of the tumor stained positively with special neural silver stains.

In 1927, Cushing and WOlbach first described the transformation of malignant Neuroblatoma

into its benign counterpart , ganglioneuroma.IN 1957, Mason published report of a child with

Neuroblastoma whose urine contained pressor amines which helped to understand the possible

origin from sympathetic neurons.

causes:

The etiology of Neuroblastoma is not well understood.

Several risk factors have been prposed and are the subject of ongoing research.

Due to characteristic early onset , many studies have focused on parenteral factors

around conception and during gestation.Factors investgated have included

occupation , that is exposure to chemicals in specific industries,smoking,

alcohol consumption, use of medicinal drugs during pregnancy.

However results have been inconsistent.

Neuroblastoma can exhibit familial incidence .

It can be associated with Beckwith-wiedemann syndrome,

HIrschprungs, Neurofibromatoses or occur as a complication of fetal hydantoin syndrome.

Neuroblastoma has been called the great mimicker because of its myriad clinical

presentations related to the site of tumor, metastatic disease and its metabolic tumor

byproduct.The most common site of neuroblastoma is abdomen arising either in the

adrenals or less commonly in the para midline sympathetic chains.

Other sites being neck, mediastinum, pelvis.Neuroblastoma is one of the rare human

malignancies known to demonstrate spontaneous regression from undifferentiated to

a completely benign cellular appearance.

The initial symptoms are often vague and often include fatigue,

loss of appetite and fever.Later symptoms depend on tumor location.

The most common finding on physical examination is a non tender ,

firm,irregular abdominal mass.at the time of diagnosis ,the site of neuroblastoma

predictably age dependant.Infants often present with compression of sympathetic

ganglia in thoracic region which might result in Horner’s syndrome

(mioses, anhydrosis and ptosis)or Superior vena cava syndrome.

Older children typically present with abdominal complaints.

More than 50% of patients presenting with neuroblastoma have metastatic disease,

which resulted in syndromes. Pepper Syndrome occurs in infants with

metastatic deposits in liver. it generally confers a better prognosis, however,

may die of massive hepatomegaly,respiratory failure and overwhelming sepsis.

Blue-berry muffin babies are infant in whom neuroblastoma has become metastatic

to random subcutaneous sites.Opsoclonus/myoclonus ,thought to be

paraneoplastic ,perhaps autoimmune.

widespread metastasis of neuroblastoma to bone may result in Hutchinson

syndrome. It results in bone pain ,limping and pathological fractures.


Most neuroblastomas produce catecholamines as metabolic by-products resulting,

in some patients, Kerner-morrison syndrome, causes intractable secretory diarrhea,

leading to hypovolaemia ,hypokalaemia, prostration. It is secondary to vasoactive

intestinal peptide(VIP) tumor secretion.


This syndrome more commonly associated with Ganglioneuroblastoma /

Ganglioneuroma typically resolves with complete removal of the tumor.