Adrenal incidentalomas and Acromegaly Flashcards
Adrenal incidentaloma (AI) (3)
- derivative of more than 1cm found in the adrenal gland incidentally (found by chance)
- most common –> hormone non-secretory adenomas
- goal: differentiate between benign and malignant, evaluate where the tumor is functionally active
Adrenal incidentaloma (AI) -clinical symptoms (5)
- most patients have none
- symptoms of Cushing’s syndrome
- symptoms of Pheochromocytoma
- symptoms of hyperaldosteronism
- symptoms of malignancy
Adrenal incidentaloma (AI)
- physical examination (6)
- radiological examination (1)
- sonoscopy (ultrasound) (2)
Evaluate:
- BP, HR
- waist circumference, assess obesity type
- skin lesions - ecchymoses, stretch marks
- hirsutism, virilization
- muscle strength
- possible changes in appearance - compared with previous pics
- differentiate between adrenal adenoma, carcinoma, pheochromocytoma and metastasis
- sensitivity is lower, inappropriate to differentiate between benign and malignant tumors
Adrenal incidentaloma (AI)
- CT without venous contrast
- CT with venous contrast
- recommended for initial patient examination, diagnosis is based on density.
- adenoma –> density less than 10 HV in non-contrast images and size smaller than 4cm - recommended if tumor density is >10 HV, malignant tumors are usually larger than 4cm
- benign changes –> density less than 80-90 HV
- pheochromocytoma –> density greater than 100 HV
Adrenal incidentaloma (AI)
- MRT
- Adrenal scintigraphy
- effectiveness in differentiating malignant and benign tumors, test of choice for: pregnant women, children patients allergic to contrast agents
- not first choice, not recommend for routine patient
Adrenal incidentaloma (AI)
- Positron emission tomography (PET)
- Aspiration Pussy (AP)
- not recommended for routine examination, useful when CT results with intravenous contrast are questionable or malignancy is suspected in other methods
- not recommended for all patients, useful when suspecting metastases or infection, pheochromocytoma should be ruled out before performing it (life threatening complications)
Adrenal incidentaloma (AI) -Hormonal examination (3)
- should be performed in all patients except when imagining techniques are used to detect a cyst or myelolipoma
- suspected adrenocortical carcinoma –> sex hormones and steroid precursors
- primary aldosteronism –> only hypertension and hypokalemia should be investigated
Suspected hormonal activity –> recommended initial tests
- Pheochromocytoma
- Aldosteroma
- Cortisol secreting tumor
- free metanephrines in blood or fractionated metanephrines in urine
- aldosterone and renin in blood
- 1mg Dexamethasone suppression test
Examination for pheochromocytoma (2)
- should be investigated in all patients with adrenal derivatives
- free blood urinary metanephrine or fractionated urinary metanephrine
Examination for primary hyperaldosteronism
-hypertension and/ or hypokalemia
Examination for subclinical Cushing’s syndrome (SCS) (4)
- 1 mg Dexamethasone suppression sample
- cortisol levels 138 nmol/l (5 ug/dL) confirms the diagnosis
- testing for cortisol in urine is less sensitive and not routinely recommended
- if adrenal metastases are suspected –> test for adrenal insufficiency
Adrenal incidentaloma (AI) -treatment (2)
Surgery
- not recommended for –> asymptomatic, hormonally inactive, unilateral incidental <4cm incidence with benign features
- recommended for –> size >4cm, suspected malignancy, hormone releasing derivatives, if the tumor increased in size
Laparoscopic adrenalectomy vs. Laparotomy
Laparoscopic adrenalectomy - with tumor <6cm
Laparotomy - if >6cm or if malignancy is suspected
Preoperative and postoperative period after detection of Pheochromocytoma
Preoperative
- correct BP - alpha and beta blockers
- correct HR
Postoperative
-glucocorticoid replacement therapy –> surgery for cortical adenoma and if Cushing’s syndrome has been diagnosed before surgery
Acromegaly (5)
- definition
- prevalence
- rare
- increased secretion of growth hormone (GH) or somatotropic hormone (STH)
- patients aged 40-45 years
- often remains undiagnosed
Acromegaly
- etiology
- genetic syndromes associated with it: (2)
- 99% are caused by benign anterior pituitary adenoma
- 95% have benign monoclonal pituitary adenoma developing from somatotropin cells
- McCune-Albright syndrome - multiple fibrous bone dysplasia, early puberty, cafe-au-lait spots
- hyperparathyroidism, neuroendocrine tumors, endocrine and non-endocrine tumors in patients with MEN1
Acromegaly
-pathogenesis (4)
- the release of GH is regulated by the inhibitory effect of somatostatin and the stimulatory hormone
- GH acts on the body through insulin-like growth factors (IGF-1) synthesis
- increased GH secretion –> increased IGF-1 production
- usually hypersecretion from a benign monoclonal pituitary adenoma
Acromegaly
-signs and symptoms (14)
- facial changes and enlargement of limbs
- enlargement of internal organs
- increased sweating
- menstrual disorders
- paresthesia of the extremities
- headache
- fatigue and drowsiness
- galactorrhea
- goiter
- erection disturbances
- ear, nose, throat or dental disease
- thrombosis/ arrhythmias
- arterial hypertension
- carpal tunnel syndrome
Acromegaly
-complications (6)
Endocrine - pituitary failure, diabetes, osteoporosis
Oncological - colon, breast, stomach, prostate cancer
Breathing - upper airway obstruction, pulmonary emphysema, COPD
Cardiovascular - cardiomyopathy, MI, arrhythmias, sudden death
GI - cholelithiasis
Bone changes - bone formation more intense, thickening of bone diaphysis in long bones and widespread joints, spinal enlargement, widespread intervertebral spaces, chest deformities
Acromegaly
-diagnosis (8)
- blood levels of GH and IGF-1
- dynamic glucose tolerance sample (GTM)
- ophthalmologic diagnostics
- MRI (more sensitive), CT
- examination of pituitary function to rule out failure
- a single GH test is not always informative!
- if acromegaly suspected –> IGF-1 should be tested first
- for active acromegaly –> 75g of OGTT remains >1ng/nl –> GH counteracts insulin
Acromegaly
-treatment (3)
- surgical –> first-choice
- medication
- radiation - used after unsuccessful operation, ineffective medical treatment or when the patient is unable to have surgery. 45-50 Gy is given but cause complete pituitary failure
Acromegaly
-medical treatment (only names - 4)
- Somatostatin analogues - ocreotide, lanreotide, pasireotide
- Dopamine receptor agonists (bromocriptine and cabergoline)
- Growth hormone receptor antagonists (pegvisomant)
- Combined treatment - Somatostatin analog 1/month + Pegvisomant weekly
Acromegaly
-Somatostatin analogues (3)
- ocreotide, lanreotide, pasireotide
- when surgical treatment is inappropriate or ineffective
- side effects: nausea, vomiting, abdominal pain/discomfort, diarrhea
Acromegaly
-Dopamine receptor agonists (5)
- bromocriptine and cabergoline
- inhibit GH secretion but efficacy is low
- indirectly inhibits IGF-1 secretion
- quick onset of action, low risk of hypopituitarism, ease of use
- cabergoline is more effective and better tolerated, fewer side effects
Acromegaly
-Growth hormone receptor antagonists (6)
- Pegvisomant
- efficiency is 97%
- blocks GH receptors –> inhibits IGF-1 production
- doest not produce pan-hypopituitarism
- does not cause adenoma regression (MRI once a year)
- inconvenient use - daily subcutaneous injection
Acromegaly
-purpose of treatment (6)
- symptom relief
- reduction of visual impairment and soft tissue lesions
- prevention of skeletal deformities
- normalization of basal AH and IGF-1
- normalization of pituitary function
- prevention of relapse
Acromegaly
-long term monitoring strategy (4)
- MRI is recommended 3-4 months after the surgery
- full pituitary evaluation is recommended 3 months after surgery
- echocardiography - if the patient does not have heart disease, this is recommended at the start of the treatment
- colonoscopy - all acromegaly patients over 40 y.o, recommended colonoscopy at the start of treatment
Receptors that can have an effect in suppression of GH secretion? (2)
- GH receptors
- Somatostatin receptors –> most imp. sstr 2 and 5
Where are the receptors for GH located?
found in cell surfaces of many tissues throughout the body including liver, muscle, adipose, kidney…
When do we have the highest and lowest concentration of growth hormone normally?
highest - at night
lowest - with aging, obesity…
Do we still have pulsatile growth hormone secretion in acromegaly?
No, it is always higher than normal
First choice of treatment:
- Prolactinoma
- Acromegaly
- medication
2. surgery
