Case study: 9 years of uncontrolled disease without appropriate medical therapy: a case for poor QoL (Mirtha Guitelman, MD)
| 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 |