Introduction
QoL in active and controlled acromegaly
A 34-year old man presented with headaches, acral enlargement, oily skin, sleep apnea, fatigue, hyperhidrosis and generalized joint pain |
All these symptoms started 5 years prior to the first consultation |
The patient had no relevant background |
Physical exam revealed |
Thyroid: 30 g |
Typical facial features: lip, nose and tongue enlargement |
Prognathism |
Acral enlargement (finger size: 33) |
No Hypertension |
Endocrinological lab results (Nov 1993) |
Baseline and post-glucose GH 50 μg/L |
IGF-1: 700 μg/L (normal for age ≤500 μg/L) |
Prolactin: 200 μg/L (normal 5–20 μg/L) |
Total T4: 1.2 nmol/L (6.9 μg/dL); TSH: 1.6 mIU/L |
Testosterone: 6.6 nmol/L (190 ng/dL) |
LH: 3 IU/L; FSH: 2.8 IU/L |
Normal glucose metabolism |
MRI at diagnosis (Dec 1994) |
Voluminous sellar mass with sphenoid sinus invasion, extension in the suprasellar cistern causing displacement of the pituitary stalk and invasion into the right cavernous sinus (Fig. 1) |
Complementary studies |
Echocardiogram: Mild dilated left ventricle with appropriate systolic function. Left and right ventricular dilatation |
Treatments |
In 1995 he received intermittently subcutaneous SSAs 300 μg/day and bromocriptine for 6 months with no changes in IGF-1 levels or tumor size |
Due to the lack of drug availability and lack of response, the patient was sent to surgery |
Transsphenoidal surgery (Aug 1996) |
Pathology: GH-Prolactin co-secreting tumor |
GH after surgery: 51 μg/L |
Transcranial surgery (Apr 1997) |
GH after surgery: >30 μg/L |
Hypogonadotrophic hypogonadism |
Normal thyroid and adrenal function |
Radiotherapy 5,000 CGy (May 1998) |
One year after radiotherapy |
IGF-1: >400 μg/L |
GH: 40 μg/L |
Prolactin: 80 μg/L |
Rest of anterior pituitary hormones normal |
Bromocriptine and somatostatin analogs were indicated |
The patient returned (Jan 2008) after 9 years without any treatment |
Joint stiffness, paresthesia, arthropathy (osteoarthritis, needed a walking stick), macroglossia, severe obstructive sleep apnea and headache |
Acral enlargement, increased sweating |
Signs and symptoms of Hypopituitarism |
Hormonal evaluation after 9 years without treatments |
GH: 19.9 μg/L; IGF-1: 640 μg/L (normal for age 101–303 μg/L) |
Prolactin: 860 μg/L |
LH: 0.2 IU/L; FSH: 0.5 IU/L; Testosterone: 0.3 nmol/L (0.1 ng/mL) |
Cortisol: 28 nmol/L (1 μg/dL) |
Free T4: 8.4 pmol/L (0.65 ng/dL); TSH: 2.7 mIU/L |
Active disease plus complete Hypopituitarism
|
For MRI scan in Jan 2008, 9 years after surgery and radiotherapy without medical treatments, see Fig. 1
|
Treatment indications |
Hydrocortisone 15 mg/day |
L-T4 100 μg/day |
Testosterone Transdermic testosterone 5 g, one sachet/day |
Cabergoline 1 mg/week |
SSA |
Follow-up |
The patient didn’t return to the hospital again after February 2008, in spite of attempts to contact him for follow-up |
The patient died suddenly in January 2010 at age of 49 |
At the time of death, he had been on regular L-T4, hydrocortisone and cabergoline, as well as irregular testosterone replacement |
He never received somatostatin analogs due to difficulties obtaining the drug |