Acromegaly

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• Acromegaly

METIB SAFAR AL-OTAIBI
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Acromegaly

• Acromegaly is a hormonal disorder that results
  when the pituitary gland produces excess growth
  hormone (GH). After puberty
• The name acromegaly comes from the Greek words
  for "extremities" and "enlargement.
• It most commonly affects middle-aged adults
  (occurs in about 6 of every 100,000 adults).

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What Causes Acromegaly?

• caused by prolonged overproduction of GH by the
  pituitary gland.
• Pituitary Tumors
• Non-pituitary Tumors

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Pituitary Tumors

• In over 90 percent of acromegaly patients, the
  overproduction of GH is caused by a benign tumor
  of the pituitary gland, called an adenoma.
• These tumors produce excess GH and, as they
  expand, compress surrounding brain tissues, such
  as the optic nerves.

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Pituitary Tumors

• In addition, compression of the surrounding
  normal pituitary tissue can alter production of
  other hormones, leading to changes in
  menstruation and breast discharge in women and
  impotence in men.
• Some adenomas grow slowly Other adenomas grow
  rapidly

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Pituitary Macroadenoma (MRI)
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Non-pituitary Tumors

• In a few patients, acromegaly is caused not by
  pituitary tumors but by tumors of the pancreas,
  lungs, and adrenal glands.
• These tumors also lead to an excess of GH, either
  because they produce GH themselves or, more
  frequently, because they produce GHRH, the
  hormone that stimulates the pituitary to make GH
  .
• In these patients, the excess GHRH can be
  measured in the blood .

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Symptoms   

• swelling of the hands and feet
• facial features become coarse as bones grow
• body hair becomes coarse as the skin thickens
  and/or darkens
• increased perspiration accompanied with body odor
  
• protruding jaw
• voice deepening
• enlarged lip, nose, and tongue
• degenerative arthritis
• thickened ribs (creating a barrel chest)
• joint pain
• enlarged heart

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Symptoms cont.

• enlargement of other organs
• impotence in men
• strange sensations and weakness in arms and legs
• snoring
• fatigue and weakness
• headaches
• loss of vision
• irregular menstrual cycles in women
• breast milk production in women

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Diagnosis

• serial photos taken over the years?
• to observe physical changes in the patient
• x-rays?
• to detect bone thickening
• blood tests?
• to check the growth hormone level

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• Typical Skull RX (Thickening of the Calvarium) of
  an Acromegalic patient

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Treatment

• Surgical therapy
• This is the gold standard of GH-secreting tumors
  because of its aggressive behavior
• And must be done as the primary choice in all
  patients who are otherwise acceptable surgical
  risks
• The surgical modality can be transsphenoidal (the
  better) or craniotomy.
• operation of pituitary tumors no larger than 10
  mm in diameter

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Treatment cont.

• Radiation therapy
• The main disadvantage of this therapy is the
  long-term duration to reach the goals of
  therapy
• About 5 years are required for reduction of GH
  levels to normal in 50 of the patients.

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Treatment cont.

• Clinical Therapy
• Dopaminergic Analogues.
• Bromocriptine (Parlodel)
• Somatostatin analogues .
• octreotide (Sandostatin).
• Pegvisomant, a new drug that directly blocks the
  effects of growth hormone.

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• untreated, acromegaly can lead to diabetes
  mellitus and hypertension.
• also increases a patient's risk for
  cardiovascular disease and colon polyps that may
  lead to cancer.

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•   monitored for years for possible recurrence.

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summary

• Acromegaly is an increase in size of the hands,
  feet, and the face.
• due to excessive production of growth hormone
  (somatotrophin) usually by a tumor of the
  anterior pituitary gland.
• The tumor can be treated with X-rays, or
  surgically removed.

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2.39 M
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