Pituitary Tumor & Acromegaly

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Pituitary tumor & Acrom

egaly
Case report
Patient profile
• 張 OO
• 58 years old male
• Past history
• 1. OP history:
• - External hemorrhoid s/p total hemorrhoidectomy on 2006-5-29.
• - Hernia operation in 1982
• 2. Disease history:
• - Hyperthyroidism in 2010, irregularly on medicine
• - BPH in 2018
• - acute pancreatitis on 2005-05-23, alcoholism related
• Family history
• Unknown
• Occupation: 水電工
Present History

2020-02 2020-04-04 2020-04-27


Blurred vision At 台安醫院 At VGH-TPE Dr. 黃文成’ s
Hand tremor MRI: OPD
1.85X2.02cm Dx:
Past history: w/ optic n. compression Spinal stenosis,
Hyperthyroidism in 2010, cervical disc displacement
irregularly taking medicine C3/C4 severe stenosis pituitary macroadenoma
C4/C5/C6/C7/T1 stenosis Transferred to Dr. 顏玉樹
Physical Examination
✖ proptosis ✖ fatigue
✖ galactorrhea ✖ myxedema ✖ erection dysfunction
◯ acromegaly ✖ goiter ✖ muscle mass decrease
✖ polyuria ✖ hyperthermia ◯ secondary sex
✖ feeling hot characteristic
✖ clubbing finger
◯ hand tremor
✖ fatigue ✖ headache
✖ Diplopia
✖ striae ✖ nausea/vomiting
◯ blurry vision
✖ buffalo hump ✖ facial sensation/motor
✖ decreased acuity
✖ moon face dysfunction
✖ ptosis
✖ easy bruising ◯ normal DTR
✖ oculomotor disorder
✖ central obesity ◯ normal muscle power
◯ light reflex
Facial
features
16 v.s 52

Facial line
Big nose

Thick lip

prognathism
IGF-1
Diagnosis of acromegaly
GH after OGTT
Limitations of random serum G
H measurements :
• pulsatile, diurnal
Pituitary MRI
• Factor: >2mm
short-term fasting, exercise, st Body CT
ress, and sleep GH-secreting
• exercise or at night : 20 to 30 arteriovenous pituitary
before meal: 2 to 5 ng/mL GH or GHRH
• GH clearance is rapid
adenoma
(half-life 20 minutes) Ectopic GHRH
Melmed S, Anterior pituitary. In: Current tumor 0.5%
Practice of Medicine, Korenman S (Ed), 1996. J Clin Endocrinol Metab. 2012;97(6):2093.
Lab data
Prolactin GH TSH ACTH FSH LH
• Pituitary
14.2 ng/mL 3.81 ng/ml <0.005 uIU/ml 40.9 pg/ml 9.6 mIU/ml 8.61 mIU/ml

IGF-1 T3 T4 Free T4 Cortisol


• Endocrine 396 ng/ml 207 ng/dL 10.40 ug/dL 3.11 ng/dL 11.9 ug/dL

• GH after OGTT(oral glucose tolerance test) within 2 hrs:


0’ 30’ 60‘ 90’ 120’
4.39/3.15/2.51/2.79/2.93 all >1ng/mL
• Glucose after OGTT
0’ 30’ 60‘ 90’ 120’
107/240/254/193/146
After Admission
• Ophthalmologist: compressive optic neuropathy, OD
• MRI on 2020-05-05 :
2.4x1.9x2.3cm w/o cavernous sinuses or sphenoid sinuses invasion
• Dx: GH-secreting pituitary macroadenomas
• Transferred to META for primary hyperthyroidism
• Sono on 2020-05-08 :
Multiple nodules at bil. side. Largest one about 1.8 cm at the left side, s
uspected nodular goiters. Suggest fine needle aspiration
• Transsphenoidal surgery on about 5/26
Epidemiology
• 15% primary brain tumor
Epidemiology of pituitary mass
(3rd common)
• 25% primary benign brain tumor
• Glioblastoma& 20.50%

astrocytoma
37.00%

12%
• meningioma
• malignant tumor
7%

• Other non-
8% 16.00% • Pituitary tumor
malignant tumor • Nerve sheath
Endocrinol Metab Clin North Am. 1987; 16(3), 529-551.
Epidemiology of pituitary mass
• 18% GH 18.0%

• 1.0%TSH 1.0% 40.5% • 40.5% non-


functioning
9.60%

• 9.60% ACTH

30.9%
• 30.9% prolactin

The Lancet. 2014; 383(9927), 1503-1516.


Epidemiology of acromegaly
• 18% of pituitary mass The Lancet. 2014; 383(9927), 1503-1516.
• Progression is usually very slow.
Few patients seek care because their appearance had changed or for
other symptoms related to acral enlargement (eg, only 13 percent of
256 patients in one series) Endocrinol Metab Clin North Am. 1992;21(3):597.
• The interval from the onset of symptoms until diagnosis is approximat
ely 12 years Endocr Rev. 2004;25(1):102.

• Approximately 75 percent of patients have macroadenomas


Endocrinol Metab Clin North Am. 1992;21(3):597.
Symptoms
Secreting tumor Symptoms Non-secreting tumor

Visual loss, visual acuity


。 GH ptosis
→ Acromegaly or gigantism diplopia
。 Prolactin
→ Amenorrhea-galactorrhea

。 TSH
→ Secondary hyperthyroidism
。 ACTH facial
→ Cushing’s disease paresthesias
。 FSH, LH
→ PCOS, infertility, OHSS diaphragma sellae: Headache
menstrual irregularity 。 stalk effect→interruption of dopamine
<96 ng/mL (v.s >200prolactinoma) BMC research notes,
2014; 7(1), 555.
Symptoms in acromegaly
13% of
256
seek 40-50%
help
Facial features Finger acromegaly Sleep apnea
Endocrinol Metab Clin North Am. 1992;21(3):597 Eur J Endocrinol. 2008;159(5):533

HTN
IGF-1 LVH 50%
effect impaired glucose
colon cancer Cardiovascular abnormalities tolerance
Cancer Res. 1996;56(3):523.  Medicine (Baltimore). 1994;73(5):233. 
Facial
features
16 v.s 52

Facial line
Big nose

Thick lip

prognathism
Cephalometrics:
• The nose width, length, height and d
epth were longer;
• The upper and lower lips were
thicker;
• The face length, face width and
gonion-gnathion distances were
longer;
• The nasofrontal and columella-labial
angles were smaller
Frontiers in endocrinology, 2020; 9, 722.
Cephalometrics:
• IGF-1 were linearly and positively corr
elated with the nose width and gonio
n-gnathion distance
• Linearly and negatively correlated with
the nasofrontal angle.

Frontiers in endocrinology, 2020; 9, 722.


Management
Indications of Surgery
• Mass effect
• Excess hormone secretion not controlled by medical mana
gement
• Pituitary apoplexy
Surgery
OP: optic
protuberance

CP: carotid
protuberance
(parasellar&
paraclival)

Pg: pituitary gland

C: clivus

Sphenoid stage
w/ sella opening
Cappabianca, P, etc. (2005). Endoscopic
Pituitary and Skull Base Surgery. P. 15
Postoperative Care and Follow-up
• New-onset Diabetes Insipidus treatment
• Normal : 60kg 60cc/hr ; DI: >250cc/hr ; gravity<1.005 , two times
• urine output >3 L/24 h and urine osmolality < 300 mOsm/kg
• Record urine output and fluid intake, urine specific gravity daily (<1.005), and
serum sodium every 6–12 h until discharge
• Subcutaneous vasopressin or desmopressin (DDAVP)
• Adrenal insufficiency prevention
• Hydrocortisone
• Evaluate ACTH one week later
Complications
• Sinonasal Complications (anosmia, congestion, minor or major epistaxis, sens
e of difficulty breathing or the empty-nose syndrome)
• The incidence of major postoperative complications was 8% (254 patients):
• 2% permanent diabetes insipidus
• 2% cerebrospinal fluid leaks requiring surgical repair
• 2% meningitis
• 2% hypopituitarism

J Clin Endocrinol Metab. 1998;83(10):3411. 


Prognosis
Recurrence rate:
(115 patients s/p TSS from 1981 to 1
995)
• The recurrence rate in our series was 5.4% for all tumors.
• 7.7% for macroadenomas and 0% for microadenomas.
J Neurosurg 89:353-358, 1998
Post-op (17 people)
• serum GH concentrations typi
cally fall to normal within one
to two hours
• Serum IGF-1 concentrations fall
more slowly, from 7 to 10 days
to several months

The Journal of Clinical Endocrinology & Metabolism, 90(12), 6480-6489.


Morbidity in successfully treated ac
romegaly
• 118 patients (age of 58.6 ± 12.9 yr) were sent an explorative questio
nnaire.
• Joint-related complaints were reported by 77% of the patients.
J Clin Endocrinol Metab. 2005;90(5):2731. 
Obstructive sleep apnea syndrome

• 74.1%(20/27) had OSAS


• Of the 16 patients with repeated polys
omnography and OSAS at baseline, 11
(68.8%) were cured of OSAS.
18 人
7人 2.5Y
w/o dz w/o dz
J Clin Endocrinol Metab. 2020;105(1) 
3D cephalometry
M • Age: 56.0 ± 10.7 years.
• Remission of acromegaly for 8.5
± 8.6 years (range 2–36).
Less More • 3D cephalometry shows that
many typical soft tissue
F deformities persist
• A wider face at the level of the
zygoma and longer maxilla

Pituitary, 2015;18(1), 126-134.

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