Adrenal Pathology (5/16) Flashcards
Where are the adrenal glands?
In the retroperitoneal tissues at the upper poles of the kidney (4-6g)
What shape is the right adrenal?
Pyramidal, behind the right lobe of the liver
What shape is the left adrenal
Crescent shape, lies behind the omentum and stomach
Define Addison’s dis
Adrenal insufficiency: dec levels of glucocorticoid production which eventually affects production of mineralocorticoids and sex steroids
What is the pathology/cause of Addison’s dis
- Destruction of cortical cells-cortical atrophy
- Chronic inflamm cells (autoimm)
- Infectious agents (granulomatous inflammation in TB)
- Metastatic tumor from other sites
- Amyloid deposition
What are some clinical features of addison’s
- Fatigue, malaise, weakness, anorexia, vomiting, wt loss, hyperpigmentation, postural hypoT
- Infectious: fever, septic shock
What are some diseases of adrenal cortex hyperfxn?
Cushings (glucocorticoid xs) Conn's (aldosterone xs) Adrogenital syndrome (androgen xs)
What are some causes of cushings?
-Bilateral hyperplasia (80%) (pituitary ACTH sec adenoma, ectopic ACTH, primary, idiopathic)
- Adrenal cortical adenoma (10%)
- Adrenal cortical carcinoma (10%)
Most common cause of cushings in adults?
Hyperplasia
Most common cause of cushings in children?
Carcinoma
What are the 3 types of bilateral hyperplasia in cushings?
Simple diffuse (65%, females) Bilateral nodular (20%, females) Ectopic (15%, males)
What is adrenal cortical adenoma?
Benign neoplastic proliferation of adrenal cortical tissue
What is the gross pathology of adrenal cortical adenoma?
- Unilateral and solitary
- Well circumscribed
- Yellow tan
- <100g
What is the microscopic pathology of adrenal cortical adenoma?
- Tumor cells grow in mixture of growth patterns (nests, cords, solid)
- Usually cells have clear cytoplasm-resembling cells of zona fasciculata
- No mitosis or atypical cells
What is adrenal cortical carcinoma?
Malignant neoplasm arising from adrenal cortical cells
What is the gross path of adrenal cortical carcinoma?
- Large bulky tumor
- Fleshy cut surface with hemorrhage and necrosis
- > 100g
- Invasion
What is the micro path of adrenal cortical carcinoma?
- Invasion
- Necrosis
- Cellular pleomorphism
- Tumor capsule invasion
- Vascular invasion
- Distant metastases (lung and liver most common)
What causes hyperaldosteronism?
- Adenoma (65%)
- Bilateral hyperplasia (35%)
- Carcinoma (rare)
What is Conn’s syndrome
Aldosterone XS
What is the microscopic pathology of adrenal cortical adenoma causing Conn’s syndrome??
- Variety of cell types
- Most have cells resembling Zona Fasciculata with minority Zona Glomerulosa
What are the clinical features of Conn’s dis caused by adrenal cortical adenoma?
- Hyper T
- Muscle weakness, fatigue, polyuria
- Periodic paralysis
- Inc cardiac arrhythmias
What are some causes of adrogenital syndrome?
- Congenital adrenal hyperplasia
- Adrenal cortical adenoma (rare)
What are some clinical features of adrogenital syndrome?
- F: male hair pattern with facial hair
- M: gynecomastia, dec libido, feminized hair pattern and testicular atrophy
What is congenital adrenal hyperplasia?
Inherited disorder caused by def of enzymes req for biosynth of glucocort and mineralocort leading to androgen excess
What is the pathology of congenital adrenal hyperplasia?
Enlarged/hyperplastic adrenal glands leading to hyperandrogenism
What are some causes of adrenal medulla hyperfxn?
- Neoplasm (pheochromocytoma)
- Hyperplasia
- Neuroblastoma
What is pheochromocytoma?
AKA: Adrenal medullary paraganglioma
- Catecholamine secreting tumor arising from chromaffin cells of adrenal medulla
- Ie hyperfxn of adrenal medulla
What are the clinical features of pheochromocytoma?
- Mostly sporadic, 20-35% familial, autosomal dominant
- Excess catecholamine: hyperT, palpitations, headaches, diaphoresis, flushing, anxiety, nausea, pain and epistaxis
- Elevated urine catecholamines
What are some familial syndromes associated with pheochromocytoma?
- Mult endocrine neoplasia type 2A or 2B
- Von hippel lindau syndrome
- neurofibromatosis
- germline mutation in SDHB, SDHD, VHL, RET or NF1
What are the gross findings of pheochromocytoma?
- Sporadic: unilateral
- Familial: bilat or mult tumors
- Well circumscribed
- 6cm
- Firm cut surface, vascular (endocrine tumors are vascular)
What are the microscopic findings of pheochromocytoma?
- Nested, trabecular or solid growth pattern
- Zellballen/Organoid pattern: round groups of tumor cells supported by rich vascular network and delicate spindle cell sustentacular network
- Cell nuclei show salt and pepper aka neuroendocrine chromatin
- Malignancy is associated with necrosis, mitosis, vasc envasion
What are multiple endocrine neoplasia syndromes (MEN)>
Genetic syndromes in which mult endocrine organs are involved. Signs and symp vary because of this. Can also have lesions in non endocrine systems
What are the 3 MEN syndromes
- MEN 1 (werner)
- MEN2A (sipple)
- MEN 2B
What is MEN 1?
Neoplasia of pituitary, parathy, pancreatic islets (determines clinical prognosis) . MENIN gene is involved with mutl sites of mutations
What are MEN 2A and 2B mutations in and what is the most impt prognostic lesion?
Mutations in ret protooncogene. Medullary thyroid carcinoma is the most prognostically impt lesion, can be fatal
What is men 2A?
Neoplasia and hyperplasia of Thyroid C cells, adrenal medulla, parathyroid
What is MEN 2B?
Neoplasia and hyperplasia of thyroid C cells, adrenal medulla AND neural tissue of oral and GI systems, skeletal and eye lens abnormalitles-marfanoid habitus, 96% of pts show ret codon 918 mut
