Adrenal Pathology Flashcards
What is shape of right adrenal? What is shape of left adrenal?
Right is pyramidal in shape. Left is more crescent in shape
What do layers of cortex look like?
Glomerulosa is more purple
Fasciculata is largest layer; most clear (lipids for steroid synthesis)
Reticularis is pinker
What is pathology of Addison’s disease (5)?
Destruction of cortical cells Chronic inflammatory cells (if autoimmune) Infectious agents (i.e granulomatous inflammation) Metastatic tumors Amyloid deposition
What are clinical features of Addison’s disease? (8)
Fatigue, malaise, weakness, anorexia, vomiting, weight loss, hyper pigmentation, postural hypotension
What are causes of Addison’s disease (5 categories)
Congenital: Agenesis/dysgenesis Autoimmune Infections: TB, Strep Pneumo Metastases to adrenals Amyloid deposition
Causes of Cushing’s syndrome: (3)
Bilateral hyperplasia: Pituitary ACTH secreting adenoma, ectopic ACTH
Adrenal cortical adenoma
Adrenal cortical carcinoma
Which tumor is most often responsible for ectopic ACTH?
Small cell lung carcinoma
What is most common cause of Cushing’s syndrome? Is it different for adults vs. children?
Adults: Hyperplasia
Children: Carcinoma
What is breakdown of appearance of adrenal cortical hyperplasia?
More common in men or women? Weight?
Simple diffuse: 65%, women, 6-12g
Bilateral nodular: 20%, women, 10-15
Ectopic hyperplasia: 15%, males, 20g
What is gross pathology of adrenal cortical adenoma? (3)
Unilateral and solitary
Well circumscribed
Yellow tan surface
Describe the microscopic pathology of Cushing’s Syndrome (3)
Mixture of growth patterns: nests, cords, solid
Cells have clear cytoplasm– resemble zona fasciculate
No mitoses or atypical cells
Describe the gross pathology of Adrenal cortical carcinoma (3)
Large bulky tumor
Hemorrhage and necrosis
Invasion into adjacent structures
Describe microscopic pathology of adrenal cortical carcinoma (6)
Invasion of nearby organs Necrosis Pleomorphism Capsule invasion Vascular invasion Metastases
What are the causes of hyperaldosteronism? (2)
Adenoma: 65%
Bilateral hyperplasia: 35%
Describe pathology of aldosterone excess causing adrenal cortical adenoma
Gross: unilateral, solitary, golden-yellow cut surface
Microscopic: Multiple cell types: mostly ZF cells but some have ZG cells
What are clinical features of aldosterone excess? (4)
Hypertension
Muscle weakness, fatigue, polyuria
Periodic paralysis
Cardiac arrhythmias
What are causes of androgen excess?
Congenital adrenal hyperplasia
Adrenal cortical adenoma/carcinoma (rare)
What are clinical features of Adrogenital syndrome? Describe for men and women
Women: male hair pattern with facial hair
Men: gynecomastia, decreased libido, feminized hair pattern, testicular atrophy
What is pathophysiology of congenital adrenal hyperplasia?
Deficiency of enzymes required for biosynthesis of MCs/GCs–>lots of ACTH from pituitary–>increased androgens
What are causes of adrenal medulla hyper function? (3)
Pheochromycytoma
Hyperplasia
Neuroblastoma
Describe epidemiology for pheochromocytoma (2): Incidence and demographics
2-8M per year
No sex/age predilection–rare in children
What are clinical features of pheochromocytoma (big list)?
Excess catecholamines: hypertension, palpitations, headaches, palpitations, headaches, diaphoresis, flushing, anxiety, nausea, constipation, pain, epistaxis
How much of pheochromocytomas are familial? What is inheritance?
Between 20-35% pheochromocytomas are familial.
Mutations are passed in an autosomal dominant fashion
What are familial syndromes associated with pheochromocytoma? (3) Name some germline mutations: (5)
Multiple endocrine neoplasia, Von Hippel Lindau syndrome, neurofibromatosis
Mutations: SDHB, SDHD, VHL, RET, NF1
Describe difference between sporadic and familial pheochromocytomas on pathology
Sporadic: unilateral
Familial: bilateral or multiple tumors
Describe gross findings of pheochromocytoma (3)
Big
Well circumscribed
Vascular
Describe microscopic findings in pheochromocytoma (4)
Growth pattern: nested, solid
Organoid pattern: round group of cells supported by vascular network
“Salt and pepper” nuclei: neuroendocrine chromatin
Malignancy: necrosis, mitoses, vascular invasion
What are the multiple endocrine neoplasia syndromes?
MEN1 (Wermer syndrome)
MEN2A (Sipple syndrome)
MEN2B
Name location of neoplasia in MEN1 (6)
Main ones: Pituitary, parathyroid, pancreatic islets
Others: adrenal cortex, lung, thymus
How is clinical prognosis for MEN1 determined?
Related to pancreatic lesions, which can be malignant
What mutations are involved in MEN2A/2B? What is most important prognostic lesion?
Mutation in ret proto-oncogene
Medullary thyroid carcinoma– can be fatal
In MEN2A, observe neoplasia/hyperplasia of ______ (3)
Thyroid C cells
Adrenal medulla
Parathyroids (15-25%)
In MEN2B, observe neoplasia/hyperplasia of ______ (4)
Thyroid C cells
Adrenal medulla
Neural tissue of oral/GI systems
Skeletal and eye lens abnormalities
