Adrenal Gland Pathology Flashcards
Adrenal Gland Functional Histology Cortical Zones (3) Medullary Cell with Secretions
Glomerulosa: Aldosterone
Fasciculata: Cortisol
Reticularis: Androgens
Chromaffin cells: Catecholamines, Epi/NE
Cushing Syndrome
Pathogenesis, Presentation (6) Diagnosis (2)
Hypercortisolism: either endogenous or exogenous
Obesity/weight gain Moon facies Abdominal striae Decreased libido Thin skin Muscle atrophy
Elevated cortisol on 24 hour urine test
Loss of diurnal cortisol secretion
Iatrogenic Hypercortisolism
Description, Morphology
Exogenous hypercortisolism caused by long term use of corticosteroid drugs
Bilateral adrenal cortical atrophy
Endogenous Hypercortisolism
Etiologies (3) with Diagnostic Tests (3)
ACTH Dependent vs Independent Morphology (2)
ACTH-secreting pituitary adenomas (most common)
ACTH decreases after high dose dexamethasone
Ectopic corticotropin syndrome (paraneoplastic)
Elevated ACTH unchanged by dexamethasone
Adrenal adenoma or carcinoma (ACTH independent)
Low ACTH and high Cortisol levels
Diffuse Adrenal cortical hyperplasia if dependent
Macronodular hyperplasia if independent
Hyperaldosteronism Pathogenesis (2) Clinical Features (4) Diagnosis (2) and Treatment
Primary: autonomous aldosterone overproduction
Secondary: Aldosterone release in response to RAAS
Hypertension
Hypernatremia
Hypokalemia
Hypomagnesemia
Elevated plasma aldosterone
(+) Aldosterone suppression test
Sprionolactone
Primary Hyperaldosteronism Etiologies
Idiopathic, Neoplasia, Glucocorticoid Mediated
Population, Morphology and Genetics
Bilateral Idiopathic (most common)
Older people
Bilateral Nodular hyperplasia of adrenals
KCNJ5 mutation
Aldosterone-Producing Neoplasia (Conn Syndrome)
Middle aged women
Tumors contain Spironolactone Bodies
KCNJ5 mutation
Glucocorticoid Mediated
Familial syndrome
CYP11B2 under CYP11B1 control
Aldosterone under ACTH control
Secondary Hyperaldosteronism Etiologies (5)
Renal Artery stenosis Diuretic use Arterial hypovolemia/edema Pregnancy Renin-secreting tumors
Adrenogenital Syndrome
Pathogenesis (2) and Etiologies (3)
Excess secretion of DHEA and Androstenedione
Excess androgens causes virulization or feminization
Corticotroph Pituitary Adenomas
Androgen-Secreting Adrenal Carcinomas
Congenital Adrenal Hyperplasia
Congenital Adrenal Hyperplasia
Pathogenesis (4) Diagnosis (2) and Treatment (2)
CYP21A2 mutation causing deficiency of 21-hydroxylase
Without 21-h, Aldosterone and Cortisol production is shunted towards making Testosterone
Decreased levels of Aldosterone and Cortisol feed back to pituitary, causing even more Testosterone production
Sustained elevated ACTH causes bilateral adrenal hyperplasia
Diagnosed via routine neonatal metabolic screening
Elevated serum 17-hydroxyprogesterone
Treated with exogenous glucocorticoids
Mineralcorticoid supplements in Salt wasting variant
Congenital Adrenal Hyperplasia Presentations
Classic (6) Simple Virilizing (2) Non-Classic (5)
**Neonatal ambiguous genitalia is seen in all forms
Classic (Salt Wasting)
Total lack of 21-hydroxylase
Hyponatremia, Hyperkalemia
Hypotension, acidosis and cardiovascular collapse
Simple Virilizing
Partial 21-hydroxylase deficiency
Elevated testosterone causing virilization
Non-Classic (most common)
Partial 21-hydroxylase deficiency
Precocious puberty
Hirsutism, acne, menstrual irregularities
Primary Acute Adrenocortical Insufficiency
Etiologies (3) and Presentation (7)
Adrenal Crisis
Rapid Steroid Withdrawal
Adrenal Hemorrhage: Waterhouse Friederichson Syndrome
Fever N/V Abdominal Pain Hypotension Hyponatremia Hypoglycemia Hyperkalemia
Waterhouse Friederichson Syndrome
Etiologies (3) Presentation (4) Gross Morphology
Sepsis from: Neisseria meningitidis, Pseudomonas, Haemophilus influenzae
Rapid hypotension leading to shock
Disseminated intravascular coagulation
Widespread purpura
Acute Adrenocortical Insufficiency from hemorrhage starting in adrenal medulla
Adrenals turned into sacs of clotted blood
Primary Chronic Adrenocortical Insufficiency
Addison’s Disease
Presentation (4) Etiologies (5) Morphology (2)
Long standing malaise and fatigue
Anorexia/Weight loss
Joint pain
Skin hyperpigmentation
Autoimmune Polyendocrine Syndrome Type 1/2
Tuberculosis
HIV
Malignancy
Adrenoleukodystrophy (congenital, X linked)
Irregularly shrunken adrenals with lymphocytic infiltrate (if autoimmune)
Autoimmune Polyendocrine Syndromes Type 1 (APECED) and Type 2 Clinical Features (7/3)
Type 1: AIRE gene mutation Adrenalitis Parathyroiditis Hypogonadism Pernicious anemia Mucocutaneous Candidiasis Ectodermal dystrophy
Type 2:
Adrenalitis
Thyroiditis
Type 1 Diabetes
Secondary Chronic Adrenocortical Insufficiency
Pathogenesis, Presentation (3) Lab Findings (4) Diagnosis, Morphology (2)
Disorders of the hypothalamus or pituitary gland that decrease ACTH secretion
Malaise/Fatigue
Anorexia/Weight loss
Joint pain
Decreased cortisol
Decreased androgen output
Normal aldosterone
Low ACTH
Cortisol and androgen output responsive to exogenous ACTH
Moderate to severe cortical shrinking
Medulla unaffected
Functional Cortical Neoplasms
Adenomas and Carcinomas
Clinical Features (2/4)
Adenoma:
Cushing syndrome
Found incidentally on radiograph
Carcinomas:
Virulization
Found incidentally on radiograph
Compression/invasion of adjacent structures
Size is most important prognostic factor, often > 4cm
Adrenal Myelolipomas Description (3)
Benign Lesions
Comprised of Mature adipocytes and Hematopoietic cells
Adrenal Incidentalomas
Diagnosis, Prevalence, Prognostic Factors (3)
Found on advanced imaging
4% of the population with increase with age
Clinical Intervention if:
>4 cm
Positive functional assays for hypercortisolism or pheochromocytoma
Shows CT enhancement characteristics
Adrenal Medulla Paraganglion System
Locations (3) with Functions
Bronchiomeric: dilates bronchi
Intravagal: inhibits intestines, increases glycogenolysis
Aorticosympathetic: affects cardiac output and BP
Pheochromocytoma
Origin Cell, Clinical Features (5) Diagnosis, Treatment (2), 10% Rule (5)
Chromaffin cells
Hypertension, can be paroxysmal or chronic Palpitations Headache Sweating Anxiety Chronic: cardiomyopathy
Urine and plasma metanephrine elevation
Anti-Adrenergic drugs
Surgical excision
10% are: Extra-adrenal Bilateral In kids Malignant Not associated with HTN
Common Features of Inherited Endocrine Neoplasias (5)
Tumors occur at young age Tumors involve multiple organ systems Tumors are multifocal Tumors preceded by asymptomatic hyperplasia Aggressive and Recurrent
Multiple Endocrine Neoplasia Type 1
Neoplasias (4) Mutation, Clinical Features (4)
Primary Hyperparathyroidism
Pancreatic endocrine tumors
Pituitary adenomas: Lactotrophs and Somatotrophs
Gastrinomas
Germline MEN1 loss of function, decreased menin
Hypoglycemia
Intractable peptic ulcers
Hypercalcemia
Prolactin excess symptoms
Multiple Endocrine Neoplasia Type 2A
Neoplasias (3) Mutation, Clinical Features (3) Prophylactic Treatment
Pheochromocytoma
Medullary Thyroid Carcinoma (very aggressive)
Parathyroid adenoma
Germline RET gain of function
Hypertension
Hypercalcemia
Elevated calcitonin
Thyroidectomy
Multiple Endocrine Neoplasia Type 2B
Neoplasias (3) Mutation, Clinical Features (4), Prophylactic Treatment
Medullary Thyroid carcinoma (very aggressive)
Pheochromocytoma
Mucosal Neuromas
Germline RET gain of function
Marfanoid body
Elevated calcitonin
Hypertension
Skin/Eye/GI/Oral/Respiratory tumors
Thyroidectomy
