suprarenal tumor

Suprarenal tumor

تسلسل 20
المرحلة الرابعة
جراحة
د.ليث
العدد4
3\4\2018

Non - f unctioning tumoursof the suprarenal cortex

Small non - secreting adenomas of the suprarenal cortex are common post-mortem findings which
are of no significance, and which are increasingly
detected by modern cross - sectional imaging techniques
such as CT and MR.

Adrenomedullary tumoursClassificationPrimary

• Neuroblastoma.
• Phaeochromocytoma.
• Ganglioneuroma.


Neuroblastoma
A highly malignant tumour of sympathetic cells
occurring in children under the age of 5 years, and
the most common malignant tumour in neonates
and infants under 1 year old. It may be bilateral,
and up to 80% are associated with chromosomal
abnormalities.
Macroscopically, it varies from a small nodular
tumour to a large retroperitoneal mass, containing
areas of haemorrhage and necrosis.
Microscopically, it arises from neuroblasts of the
suprarenal medulla, or within any cells of neuroectodermal
origin along the spine.
Capsular invasion occurs early with spread to
adjacent tissues, the regional nodes and by the
blood to bones and the liver

Special i nvestigations

• CT, MR, ultrasound and bone scan are all used
to stage the disease.
• Neurone - specifi c enolase is a sensitive marker
of disseminated disease, and can be used as a
tumour marker following treatment.
Treatment
A combined approach with surgical removal of
local disease together with chemotherapy and/or
radiotherapy is necessary.
Prognosis
Early disease, localized to the area of origin and in
the absence of distant or lymph node spread,
carries a favourable prognosis, as do absence of
chromosome abnormalities, and age under 1 year
together with histologically well - differentiated
tumor


Phaeochromocytoma
A physiologically active tumour of chromaffi n
cells, which secretes adrenaline and noradrenaline
in varying proportions. Ten per cent are
malignant and 10% are multiple; 10% occur
outside the suprarenal gland in the sympathetic chain or the organ of Zuckerkandl near the aortic
bifurcation; 10% are familial (the ‘ 10% tumour ’ ).
Any age may be affected, but the tumour is particularly
found in young adults. The sexes are
equally affected

Clinical f eatures

These are produced by excess circulating adrenaline
and noradrenaline.
There is hypertension, which is paroxysmal or
sustained, and which may be accompanied by
palpitations, headache, blurred vision, fi ts, papilloedema
and episodes of pallor and sweating.
There may be hyperglycaemia with glycosuria.
Attacks may be infrequent, or occur several times
a day.
The diagnostic triad, with high specifi city and
sensitivity is as follows:
• headache , sudden in onset, and pounding;
• tachycardia and/or palpitations;
• sweating.
Occasionally, the tumour may coexist with neurofi
bromas and caf é - au - lait spots, medullary carcinoma
of the thyroid or a parathyroid adenoma
as part of a multiple endocrine neoplasia syndrome


Special investigations
Identifying the presence of a
phaeochromocytoma
• Urinary catecholamines , particularly
vanillylmandelic acid collected over a 24 hour
period, are usually raised, particularly during
an acute hypertensive attack.
• Fasting plasma catecholamine concentrations
are raised.
Locating a phaeochromocytoma
• CT or MR may demonstrate the site and size of
the tumour.
• 131 I - MIBG scan : meta - iodobenzylguanidine
(MIBG) is a structural analogue of