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Acromegaly

Background

Acromegaly happens when a pituitary tumor produces too much growth hormone.

The excess growth hormone can cause dramatic and disfiguring bone and soft tissue changes and serious cardiovascular problems.

In addition, the tumor itself can cause blindness by pushing on the visual nerve pathways.

 

Evaluation

Symptoms of acromegaly include enlargment of the hands and feet, leading to surprising increases in ring and shoe size, and jaw and forehead prominence. These symptoms can come on slowly so that they become advanced before anyone notices them.

Carpal tunnel syndrome and excessive sweating can occur. More subtle problems include diabetes and hypertension. Patients with untreated acromegaly are also more susceptible to colon cancer. Blurred vision, headache, fatigue, depression and impotence are often noted. Breasts can start to produce milk spontaneously.

Work-up

Blood tests confirm excess hormone levels (Growth Hormone and Insulin Growth Factor). Simply comparing old pictures to current ones can confirm the characteristic physical Findings. MRI will show the tumor and pituitary, and CT scan can document and destruction of bone around the tumor.

Treatment

  • Endonasal Endoscopic Surgery:  The first-line treatment for most pituitary adenomas is endonasal transsphenoidal surgery. The Endonasal endoscopic approach the preferred method for removal of the great majority of pituitary adenomas, RCCs, most craniopharyngiomas and chordomas as well as many midline meningiomas. Rates of cure are in the 85% range for microadenomas, and in the 50% range for larger or invasive tumors. Complications can include added visual impairment,  bleeding, stroke, cerebrospinal fluid leak and meningitis, but these risks are low when performed by experienced board-certified neurosurgeons.
  • Cyberknife Radiosurgery: While radiosurgery is traditionally reserved for patients who are not operative candidates, or in whom surgery has been ineffective, results of radiosurgery are excellent, with control rates of 90% in a recent review of the literature. The risk of complications is lower than with surgery, and the risk of visual damage is less than with traditional radiation.
  • Medical Therapy: For patients with persistent GH elevation after endonasal surgery or radiosurgery medical treatment with octreotide, lanreotide or pegvisomant can be helpful. Octreotide injections once a month, or lanreotide injections every four weeks achieve long-term suppression of GH in about 70% of patients. Tumor shrinkage is only achieved in about 40% of patients, but this can relieve headache and in rare cases improve vision.