PITUITARY TUMOR SYNDROME

DIABETES INSIPIDUS
Case report Practical activity

General Data
Initials: B.L. Age: 45 years-old Gender: male Race: caucasian Address: Vlcea County

hospitalized in Endocrinology Clinic Tg.Mure on January 2006

Chief Complains (CC)

polyuria about 10 L/day polydipsia about 10 L/day sexual dysfunction (erectile dysfunction) muscular pain, fatigue nausea, vomiting, alimentary intolerance reduction of vision

Medical History
Past Medical History (PMH):
grade 2 obesity, hyperlipidemia, grade 1 hypertension, steatosis hepatis

Family History (FHx):

father: Parkinsons disease mother: hypertension, stroke

Smoking and Alcohol: smoker 25-30 cigarettes/day for 30 years

Medications: fenofibrate, perindopril

Social/Work History (SocHx):

occupation: lorry driver marital status: married, father of 2 children

Medical History
History of Present Illness (HPI)
- polyuro-polydipsic syndrome appeared insidious about 1 year ago, with progressive character, reaching to 10-12 liter/day, increased thirst - without any investigations, the patient have received Minirin tb. 0,1mg 2x1 tb/day with improvement of the symptoms; then he ceased the drug - progressive vision deterioration: blurred vision, reduction of vision on temporal visual fields

- erectile dysfunction from about 1 year

- December 2005: nausea, vomiting, alimentary intolerance January 2006 hospitalized to Clinic of Internal Medicine No.3 Tg.Mure transfered to Endocrinology Clinic Tg.Mure for polyuria- polydipsia

PHYSICAL EXAMINATION (PE)

General status: influenced Constitution: hyperstenic Skin & Mucosa: pale, dry, moon-like face, macrocheilia, dry mouth and tongue decrease in facial hair growth (beard and mustache), reduced density and thickness of other sexual hairs Adipose tissue: well represented on face, neck, trunk, adipomastia, body weight: 108 kg, BMI: 34 kg/m2, W/H-ratio: 105/104 = 1,01,

PHYSICAL EXAMINATION (PE)

Musculoskeletal system: normal aspect, muscle weekness Cardiovascular apparatus: BP: 140/100 mmHg, HR: 60/min, Gastrointestinal app.: polydipsia, epigastric pain, nausea, Genitourinary app.: polyuria (10 l/day), nicturia, both testes in scrotum, normal size, normal primary sexual characters Nervous system: agitation, insomnia, vision disturbances, reduced vision on temporal fields bilateral

PHYSICAL EXAMINATION (PE) Endocrine system

Hypothalamo-pituitary system: Pituitary tumor syndrome:
1. neurological syndrome: no signs or symptoms 2. opto-chiasmatic syndrome: blurred vision, temporal vision field defect 3. imagistic findings: discussed at paraclinical findings 4. endocrine syndrome: signs of pituitary hormone deficiencies:
erectile dysfunction + reduced secondary sexual signs (GnRH and/or gonadotropine deficiency ? ) dry skin, constipation, obesity (TRH and/or TSH deficiency?) polyuria-polydipsia, nocturia (AVP deficiency ?)

Thyroid gland: normal size and consistency Parathyroid glands: no sings or symptoms Adrenal glands: muscle weakness, pale, dry skin (? if pituitary cause) Pancreas: obesity, polydipsia-polyuria Gonads: sexual dysfunction, reduced sexual hair

LABORATORY AND IMAGING FINDINGS

Basal glucose 101mg/dl Total cholesterol: 163 mg/dL (N<199) HDL-C 30.8mg/dl Triglycerides 253mg/dl Na 154.8 mmol/l (NV 135-155) K 4.16mmol/l (NV 3.5-5.5) Uric acid 10.27mg/dl Urea - 14.7mg/dl OGTT basal 125; 2hs after 75g glucose 105mg/dl Plasma osmolality 318 mOsm/l

Abdominal US: Steatosis Hepatis Upper gastrointestinal endoscopy: Erythematous gastritis Urology: normal, PSA normal Cardiology: Chronic Ischemic Cardiopathy. Dysmetabolic cardiopathy.
Congestive Cardiac Failure NYHA 2.

LABORATORY FINDINGS
Urine output (ml) 580 660 770 560 660 710 1,210 510 USG 1.000 1.001 1000 1.000 1.000 1.000 1.000 1.000

Fluid balance/24 h:
- fluid intake: 11 L - urine output: 10.63 L

560
710 1,010

1.000
1.000 1.000

Confirmed polyuro-polydipsic syndrome, diluted urine and hemoconcentration

WATER DEPRIVATION TEST

Start at 7:00 am:
BP 130/80 mmHg, HR: 84 bpm,

Body weight: 94.5 kg

Draw blood for serum osmolality, Hb, HTC

Measure hourly
urine output, weight, blood pressure

Stop the test, if weight decreases by 3% from baseline or symptoms of dehydration !

WATER DEPRIVATION TEST

Time (hour) 08:00 09:00 10:00 11:00 12:00 13:00 Weight (kg) 94.5 94.5 94.2 94 93.4 93.1 Urine output (ml) 160 106 200 204 280 320 USG 1.005 1.000 1.000 1.000 1.000 1.000

14:00
15:00 16:00

92.5
92 91.6

380
335 390 50 35 30

1.000
1.000 1.000 1.006 1.020 1.020

DESMOPRESSIN solution, 2 drops intranasal (20 g)

WATER DEPRIVATION TEST INTERPRETATION

Confirmed Central Diabetes Insipidus signal disease: may indicate an organic lesion

(Miller& ass., Ann Intern Med 1970;73:72 modified)

HORMONAL ASSESSMENT
Pituitary hormone level at baseline
GH: 0.08 ng/mL LH: 0.62 mIU/mL (N: 5-25) FSH: 1.33 mIU/mL (N:2.0-10.0) TSH: 1.70 mIU/L (N: 0.44-3.45) ACTH

Appropriate peripheral hormone(s) or parameter

IGF-I not determined Total Testosterone: 0.27 ng/mL (N: 2.4-12) Inhibin, sperm analysis not determined FT4:1.02 ng/dL (N: 0.8-2) Cortisol at 8 am

Interpretation
GH stimulation test not effectuated gonadotropines testosterone central hypogonadism

Normal Not determined

PRL: 9.86 ng/mL

Normal

Confirmed secondary hypogonadism

IMAGING FINDINGS Sella turcica X-Ray (January 2006):

Sella turcica with normal size, aspect of interclinoid bridge Cranian CT scan, focused on pituitary: not available

Cranian MRI focused on hypothalamic-pituitary system (Feb 2006):

Intrasellar tumor mass with suprasellar extension: cystic, multilobed aspect, 21x14x15 mm

EVALUATION FOR THE PITUITARY TUMOR SYNDROME

Ophthalmologic evaluationbitemporal hemianopia

Neurological evaluation: no signs or symptoms Neurosurgical consultation: Intra- and suprasellar chistic tumor. Recommendation: Transcranial approach.

DIAGNOSIS
1. 2. 3. 4. 5. 6. 7. 8. 9. Intra- and Suprasellar Cystic Tumor Hypogonadotropic Hypogonadism Central Diabetes Insipidus Metabolic syndrome Grade 2 obesity Hypertriglyceridemia Grade 2 arterial hypertension Hyperuricemia Liver steatosis

DIFFERENTIAL DIAGNOSIS
1. Diabetes Mellitus 2. Renal diseases in polyuric phase 3. Primary Aldosteronism 4. Primary Hyperparatiroidism 5. Potomania (psychogenic polydipsia)

MANAGEMENT
January-February 2006:
- General measures: reduced salt intake; fluid intake 2-3 L/day - Medication: Desmopressin Adiuretin drops in. 2x1/day - Monitoring Fluid balance

February 2006
total ablation of the tumor, right frontal craniotomy approach Adiuretin + Medrol 16mg tb --0/day, than Medrol ceased

Pathology result: Craniopharyngioma (embryonic squamous remnants of Rathke's pouch)

EVOLUTION
2 weeks after surgery
TSH: 0.79 mIU/L, FT4: 0.73 ng/dL (N: 0.8-2) CENTRAL HYPOTHROIDISM Euthyrox 25g 1-0-0 tb/day

6 weeks after surgery

- fluid balance: 1.850 mL/1.800 mL, USG 1.005-1.011 Minirin tb 0.2mg

2x1/day
- Na: 147 mmol/L, K: 4.1 mmol/L, HR: 60 bpm, BP: 135/90 mmHg - Plasma cortisol at 8:00 am: 0.86 g/dL (N: 5-25) Prednison 5 mg 1-0-0 tb/day

EVOLUTION
March 2007
- Aggravated visual field defect, body weight increase with 30kg, constipation, fatigue at minimal physical activity, anosmia - Normal urine output, USG 1.010, - Se-Na: 153 mmol/L, K: 4.4 mmol/L, - Hgb 14.4 g%, Hct: 43.7% - TSH: 1.67 mIU/L, FT4: 0.75 ng/dL, PRL: 20.3 ng/mL (N:2.5-17) - MRI: no tumor recurrence - Visual field examination: greately reduced at both eyes

EVOLUTION
March 2008
- Progressive aggravation of pituitary tumor syndrome, severe ophthalmologic syndrome - Cranial MRI: Intra- and suprasellar cystic mass, 19x16x20 mm

May 2008
- right frontal craniotomy, total ablation of the tumor adherent to the optic chiasm

March 2009
- no tumor recurrence

EVOLUTION
February 2011
- MRI: no tumor recurrence - Glycemia: 156-207-176 mg/dl - Hgb, Hct, Na, K, fluid balance, USG, PSA - normal - FT4, PRL, Testosteron normal - under treatment with
Rp/ Metformin 500mg D.S. Oral 1-0-1 tb/day Rp/Prednison, 5mg D.S. Oral 1-0-0/day Rp/Euthyrox, 25mcg D.S. Oral 2-0-0/day Rp/ Nebido, 1000 mg/4 ml D.S. IM 1 v every 3 month

Rp/Minirin Melt 120 mcg D.S .Sublingual 3X1 tb/day

FINAL DIAGNOSIS
1. SURGICALLY REMOVED CRANYOPHARYNGIOMA 2. PANHYPOPYTUITARISM: ADRENAL, THYROID, GONADAL DEFICIENCY 3. DIABETES INSIPIDUS 4. SEVERE OPTO-CHIASMATIC SYNDROME: ATROPHY OF RIGHT OPTIC NERVE; PARTIAL ATROPHY OF LEFT OPTIC NERVE
5. 6. 7. 8. 9. 10. 11. 12. GRADE 3 ANDROID OBESITY METABOLIC SYNDROME TYPE 2 DIABETES MELLITUS HYPERLIPIDEMIA HYPERURICEMIA LIVER STEATOSIS CHRONIC ISCHEMIC CARDIOPATHY. GRADE 2 HYPERTENSION