ACROMEGALY CASE ACROMEGALY CASE STUDIESSTUDIES

Margaret E Roberts

Acromegaly Specialist Nurse

Christie Hospital Manchester

AcromegalyAcromegaly

acros - extremitymegale - great

David & Goliath 7’ 5’’

AcromegalyAcromegaly

Acromegaly is Rare (3 per million per year)

Can be disfiguringLife shortening (average 10 Years)Equally prevalent in both ♂ and ♀ Average age 44 years at diagnosisDelay in diagnosis (8 to 10 years)

CauseCause

Excess of growth hormone Slow growing pituitary tumour (99%) Post puberty Overgrowth soft bone tissue Bone thickening Pre-puberty- gigantism ↑ GH

Hypopituitarism

↓ Testosterone and Oestrogen

Ectopic GHRH McCune-Albright Syndrome

Signs and SymptomsSigns and Symptoms

Enlargement of hands and feetInsidious changes in the faceDeepening of the voiceCoarsening of the skinExcess sweatingHypopituitarism

Signs and SymptomsSigns and Symptoms

Severe headacheSnoringSleep ApnoeaLethargyAches and painsMenstrual cycle disturbance/impotence

Acromegaly Acromegaly “spade-like”“spade-like” hands hands

AcromegalyAcromegalyCo-morbiditiesCo-morbidities

Hypertensionand heart disease

Cerebrovascular eventsand headache

Sleep apnea 50%

Arthritisirreversible

Insulin-resistantdiabetes

40% DM or Glucose

in tolerance

AcromegalyAcromegalyCo-morbiditiesCo-morbidities

Hypertensionand heart disease

Cerebrovascular eventsand headache

Sleep apnea

Arthritis

Insulin-resistantdiabetes

Direct tumour effects

visual problems

compression

hypopituitarism

TreatmentTreatment

SurgeryRadiotherapy (not widely used)Stereotactic Gamma KnifeDopamine Agonists ( Cabergoline)Somatastatin Analogue (Octreotide LAR,

Lanreotide Autogel)GH receptor Antagonist (Pegvisomant)

Objectives of treatmentObjectives of treatment

Reverse the symptoms and signs of acromegaly

Restore circulating GH (<2.5 µg/l) and Insulin-like growth factor (IGF-1) levels to normal

Restore normal life expectancy

Remove the pituitary tumour and preserve residual pituitary function

Prevent recurrence

Case StudyCase Study

Summary18 year old Iady Height 180.5cm (5ft 11½in)diagnosed 2006Acromegaly with hyperprolactinemia

History History

From 6 years age taller than friendsSlept for long periods up to 20hrs at a

time.Headaches and tirednessPins and Needles in arms and handsMum thought depressed and alcoholic

16 years went to GP referred to local hospital

Diagnosed acromegaly commenced cabergoline.

Commenced Menstrual Periods

12 months later referred to Hope Hospital.

Endocrine blood screenBasal pituitary functionIGF-IGHDCOGTT

MRIscan

Assessment (on cabergoline)Assessment (on cabergoline)

GHDC mean 14.92mU/LIGF-I 109 nmol/L (ref range 21.2-

75.9nmol/L) Prolactin 129 mU/L (ref range 50-400)Non-suppression of GH during OGTTMRI scan macroadenomaComfirmed

Acromegaly with Hyperprolactinemia

.2006 referred for surgery had 2

transsphenoidal operations not cured.

Had octreotide LAR injections not controlled.

2007 referred to Christie Hospital for further opinion.

Assessment (off cabergoline)Assessment (off cabergoline)

Prolactin 1810mU/L (ref range 50-400)IGF-I 790 mg/l (ref range 151-528)

Persistent AcromegalyThyroid and Cortisol DeficientDiabetes insipidusMenstrual Periods stopped

MedicationMedication

Thyroxine 75mcgs dailyHydrocortisone 10/5/5mgsOmeprazoleDesmopressin sprayCabergoline

Cabergoline effective in 30% patients Prolactin and GH co-secreting

tumours respond well to dopamine agonist

─ biochemical response

─ tumour shrinkage Non responsive to somatostatin

analogues

Prolactin responds, better than Growth hormone to dopamine agonists.

Cabergoline effect on heart no evidence in Acromegaly patients

Dopamine agonist → ORAL → CHEAP

In this patient Cabergoline had a much more dramatic effect.

She now feels very well on 3.5mgs Cabergoline weekly

I would like to thank

Dr Claire Higham for all her help.