ACROMEGALY

Dr Pukar Ghimire
MBBS, MD
• Caused by excess growth hormone secretion
or excess growth hormone releasing hormone
secretion
• Unless GH level is controlled survival is
reduced by an average of 10 years
CAUSES OF ACROMEGALY
• If GH hypersecretion occurs before puberty,
then the presentation is with gigantism.
• More commonly, GH excess occurs in adult life
and presents with acromegaly.
• If hypersecretion starts in adolescence and
persists into adult life, then the two conditions
may be combined.
 PRESENTATION
Acral bone overgrowth:
• frontal bossing,
• mandibular enlargement with proganthism,
• increased hand and foot size
Soft tissue swelling:
• Increased heel pad thickness
• Increased shoes and gloves size
• Ring tightening
• Coarse face with large fleshy nose
Visceromegaly:
• Cardiomegaly
• Macroglossia
• Thyroid enlargement
Other features:
• Deep and hollow voice, oily skin, arthropathy,
kyphosis, carpal tunnel syndrome, proximal
muscle weakness and fatigue, acanthosis
nigricans and skin tags
 COMORBIDITIES
HEART:
• Coronary artery disease
• Cardiomyopathy
• Arrythmias , HTN, LVH, Diastolic dysfunction
Respiratory tract: OSA
GI tract: colorectal carcinoma
Diabetes mellitus: 25% pt
 INVESTIGATIONS
IGF 1:
• elevated , useful screening measure when clinical
feature raises the possibility of acromegaly
GH suppression test:
• since GH is secreted in pulsatile fashion so single
random GH is not useful for diagnosis of acromegaly
• Failure of growth hormone suppression to less than
0.4 ugm/L after 1-2 hr of oral glucose load (75 gm)
• About 20% patient has a paradoxical GH rise
after glucose
Prolactin:
• Should be measured as it is elevated in 25%
patient with acromegaly
TFT, Gonadotrophins and sex steroid: may be
attenuated becz of mass effect
IMAGING: MRI> CT: Tumor size and extent : only
after biochemical analysis
 TREATMENT
• GOAL:
• Control GH and IGF-I hypersecretion
• Ablate or arrest tumor growth
• Ameliorate co morbidities
• Restore mortality rate to normal and preserve
pituitary function
MODALITIES:
• Surgery
• Medical therapy
• Radiation
 SURGERY
• Transsphenoidal resection
• 1st line of treatment
• Primary treatment for both microadenoma (remission
rate 70%) and macroadenoma (remission rate <50% )
• GH returns to normal within an hour and IGF-I returns
to normal within 3-4 days
• More often, surgery serves to debulk the tumour and
further second-line therapy is required, according to
post-operative imaging and glucose tolerance test
results.
 RADIOTHERAPY
• External radiation therapy
• High energy streotactic therapy
• Used as 2nd line treatment if acromegaly
persists after surgery, to stop tumour growth
and lower GH levels.
• However, GH levels fall slowly (over many
years) and there is a risk of hypopituitarism.
 Medical therapy
• If acromegaly persists after surgery, medical
therapy is usually employed to lower GH levels
to below 1.5 μg/L (below approximately 5
mU/L) and to normalise IGF-1 concentrations.
• Medical therapy may be discontinued after
several years in patients who have received
radiotherapy.
 MEDICAL THERAPY
• Somatostatin analogues
• Dopamine agonist
• GH receptor antagonist
INDICATIONS:
• Adjuvant treatment for preoperative shrinkage of
large invasive macroadenoma
• Immediate relief of debilitating symptoms
• When surgery fails to achieve biochemical control
• When surgery is not possible or denied
SOMATOSTATIN ANALOGUES:
• Octreotide
• Lanreotide
• Long acting formulation can be used every 4-6
weekly
• Somatostatin analogues can also be used as primary
therapy for acromegaly either as an alternative or in
advance of surgery, given evidence that they can
induce modest tumour shrinkage in some patients.
DOPAMINE AGONIST:
• Bromocriptine
• Carbegoline

• Are less effective at lowering GH but may sometimes

be helpful, especially with associated prolactin excess.
• If the prolactin is over 5000 mU/L, then the lesion is
likely to be a macroprolactinoma and to respond to a
dopamine agonist with shrinkage of the lesion, making
surgery unnecessary
• GH RECEPTOR ANTAGONIST
• Pegvisomant
• Antagonize GH action by blocking peripheral
GH receptor : so IGF-I is suppressed: reduce
the deleterious effect of excess growth
hormone
• However GH level remain elevated as drug
doesn’t target pituitary adenoma