24‐Nov‐17 1 Pituitary Disease Resident Tutorial 2017 Sarat Sunthornyothin MD Division of Endocrinology and Metabolism King Chulalongkorn Memorial Hospital Pituitary Anatomy hypophyseal portal system direct arterial supply from int. carotid a. 1. Sup. hypophyseal a. 2. Inf. hypophyseal a. Pituitary Gland JAMA. 2017 Feb 7;317(5):516‐524. Pituita (latin)=phlegm
This document is posted to help you gain knowledge. Please leave a comment to let me know what you think about it! Share it to your friends and learn new things together.
Transcript
24‐Nov‐17
1
Pituitary DiseaseResident Tutorial 2017
Sarat Sunthornyothin MD
Division of Endocrinology and Metabolism
King Chulalongkorn Memorial Hospital
Pituitary Anatomy
hypophyseal portal systemdirect arterial supply from int. carotid a.1. Sup. hypophyseal a.2. Inf. hypophyseal a.
• Others• History of irradiation• History of hypertension
intra‐ or postoperative hypotension, cardiopulmonary bypass, anticoagulation and/or microemboli leading to infarction
imbalance between the increased metabolic demand induced by the stimulation and the ability of increased blood flow at the level of the pituitary adenoma
24‐Nov‐17
4
Clinical Manifestations
• Headache of sudden and severe onset• “like a thunderclap in a clear sky”• Dural traction • meningeal irritation from extravasation of blood and necrotic material into the subarachnoid space
• usually retroorbital but can be bifrontal or diffuse
• Clinical suspicious is very important• Blood draw for hormone measurement
• Supportive hemodynamic treatment
• Glucocorticoid : stress dose (200 mg of hydrocortisone)
• Imaging study after initial treatment and hemodynamically stable
Conservative or Surgery
• Conservative therapy can be used in selected patients • minimal symptoms • improve dramatically after administration of glucocorticoids
• Factors to choose treatment options• Patient’s presentation• clinical stability• initial response to glucocorticoids• availability of an experienced neurosurgeon• Imaging findings
• single large hypodense area within the tumor on CT might be associated with better subsequent tumor shrinkage than are several small hypodense areas
• Simple infarction is less severe than hemorrhagic infarct
24‐Nov‐17
7
Hormonal Management
• Glucocorticoid preoperative
• Thyroid hormone if symptomatic or very low
• DI : not common (10%) and often transient
• Re‐evaluate postoperative 2‐4 weeks• Glucocorticoid may be tested early postop.
• Thyroid, sex hormone
Outcomes after Apoplexy
• Ocular palsy resolved• 75%–100% of patients without surgery,• 31%–57% of patients with surgery
• Visual field defect resolved• 50% normalized, 30% improved after Sx• Similar outcome in conservative treatment• Worse outcome in severe deficit • Better outcome if surgery within first week
• Hormonal deficit• Only 20% does not require hormone replacement after apoplexy
• Similar outcome for surgical and conservative group
Clin Endocrinol (Oxf). 2011 Jan;74(1):9‐20.
UK GuidelineApoplexy
24‐Nov‐17
8
Pituitary Apoplexy : Summary
• Clinical diagnosis: high degree of suspicion
• Hydrocortisone therapy as soon as the diagnosis is suspected
• Conservative treatment is an option
• Surgery if• Severely reduced VA• Severe, persistent, deteriorating VF defect• Deteriorating level of consciousness• Not improved or worsening with conservative treatment
• Surgery should be performed within first week
Pitfalls of Management
• Delay in diagnosis• Delay in hydrocortisone treatment
• Need patient education for self adjustment of glucocorticoid dose
• No testosterone replacement• How to prescribe
• IM, transdermal gel, oral
• How to follow up• Testosterone level, CBC, PSA• Consult urology
• PSA increase > 1.4 in first 12 month• PSA > 4• Abn. digital rectal exam
Hypothalamic Pituiary Control
Neuropeptides are released into the specialized blood supply to the pituitary to regulate its secretion
24‐Nov‐17
9
Pituitary Adenoma
JAMA. 2017 Feb 7;317(5):516‐524.
Prevalence of Pituitary Adenoma
JAMA. 2017 Feb 7;317(5):516‐524.
Prolactinoma
24‐Nov‐17
10
Prolactin Regulation
StimulatoryTRH, E, EGF
InhibitoryDopamine
hypogonadism
Prolactin Regulation
Predominant effect: Inhibitory• Dopaminergic neuron• Dopamine D2 receptorStimulatory : Prl Releasing FactorsPeripheral neural control
Hyperprolactinemia
Nat Rev Endocrinol. 2015 May;11(5):265‐75
• Prl inh. Kisspeptin expression• Kisspeptin stim GnRH neuron
Heterophile Ab are antibodies induced by external antigens (heterophile Ag)
Heterophile Ab Interference
Hook Effect
Reading can be normal or slightly elevated. Happen in very big tumor (Actual PRL is extremely high)Unless using the assay not affected by this condition: 2 steps method
24‐Nov‐17
14
Inaccurate Result of Prolactin
• Falsely high• Macroprolactinemia
• Heterophile antibody
• Physiologic causes
• Falsely low• Hook effect• Heterphile antibody
Bromocriptine VS Cabergoline
N Engl J Med. 1994 Oct 6;331(14):904‐9.
Bromocriptine VS Cabergoline
N Engl J Med. 1994 Oct 6;331(14):904‐9.Presse Med. 1995 Apr 29;24(16):753‐7
24‐Nov‐17
15
Bromocriptine vs Cabergoline in PRL‐secreting macroadenoma
• Therapy may be tapered and perhaps discontinued• treated with dopamine agonists for at least 2 yr(ENDO SOC), 3 yr (Pituitary Society)• Normal serum prolactin• No visible tumor remnant on MRI
• Recurrence rate: 26‐69%• Predicted by initial PRL and tumor size
• Increase 18% per mm of tumor size
• Most likely occur in first year • Monitor q 3 month during 1st year then annually• Repeat MRI if PRL above UNL
Recurrence after DA discontinuation
J Endocrinol Invest. 2016 May 31
Recurrence is related to‐ PRL level at Dx and before D/C‐ Pituitary dysfunction at DxNot related to size, DA doseNot related to longer treatment time
24‐Nov‐17
16
Women with Asymptomatic Microadenoma
YOUNG WOMEN
• If pregnancy is desired: DA
• If pregnancy is not desired: DA or OC• Microadenoma rarely grows
POSTMENOPAUSAL WOMEN
• DA may be discontinued
• Monitoring of tumor size increase periodically
Drug‐induced HyperPRL
• Discontinue medication for 3 days or substitution of an alternative drug, followed by remeasurement of serum prolactin• should not be undertaken without consulting the patient’s physician.
• If drug can’t be stopped: perform MRI to differentiate drug‐induced vs. pituitary/hypothalamic lesion if pretreatment PRL is not available
• Treatment with DA may cause psychosis relapsed!• Not common
• Use estrogen or testosterone in patients with long‐term hypogonadism (hypogonadal symptoms or low bone mass)