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MS2 Endocrine > 10/21- Pathology of the Adrenal Glands and Thymus > Flashcards

10/21- Pathology of the Adrenal Glands and Thymus Flashcards

1
Q

What are the layers of the adrenal glands?

A
  • Cortex: more hormonally active
  • Zona glomerulosa: mineralocorticoids (aldosterone)
  • Zona fasciculata: glucocorticoids (cortisol)
  • Zona reticularis: sex steroids (estrogen, androgen)
  • Medulla: fight/flight hormones
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2
Q

What is seen here?

A

Layers of adrenal gland

  • Cortex: more hormonally active
  • Zona glomerulosa: mineralocorticoids (aldosterone)
  • Zona fasciculata: glucocorticoids (cortisol)
  • Zona reticularis: sex steroids (estrogen, androgen)
  • Medulla: fight/flight hormones
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3
Q

IMPORTANT

A
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4
Q

What is seen with adrenocortical hyperfunction (syndromes)?

A

Hyperadrenalism: syndromes with overlapping clinical features

  • Hypercortisolism (Cushing’s)
  • Hyperaldosteronism
  • Adrenogenital (virilizing) syndromes
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5
Q

What are clinical features of Hypercortisolism (excess glucocorticoids)?

A
  • Hypertension
  • Easy bruisability
  • Weight Gain
  • Menstrual irregularity
  • Central obesity
  • Hirsutism
  • Decreased muscle mass
  • Mood swings/psychosis
  • Osteoporosis
  • Immunosuppression
  • Diabetes
  • Cutaneous striae
  • Thin skin
  • Poor wound healing
  • “Cushingoid” appearance
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6
Q

What are causes of hypercortisolism?

A

Exogenous steroid use

  • Steroid treatment for autoimmune diseases or post-organ transplants
  • Weight lifters, athletes, etc. (rare)

Endogenous:

  • ACTH hypersecretion (hypothal-pit), 70-80%
  • Cortisol hypersecretion (adrenal), 10-20%
  • Ectopic ACTH secretion (paraneoplastic), 10%
  • Common in small cell tumors of the lung
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7
Q

What are causes of ACTH hypersecretion?

  • Lab values
  • Epidemiology
A

Labs:

  • High ACTH
  • High cortisol

Causes:

  • Pituitary (Cushing Disease)
  • 5x more in females; 20s-30s
  • Microadenoma, adenoma (>1cm)
  • Primary corticotrophic cell hyperplasia
  • Hypothalamic
  • CRH producing tumor (2’ cortioctrophic cell hyperplasia in pituitary)
  • Secondary adrenocortical hyperplasia
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8
Q

What are causes of cortisol hypersecretion?

  • Lab values
A

Labs:

  • ACTH-independent, so low ACTH
  • High cortisol

Seen in:

  • Adrenocortical adenoma (#1)
  • Adrenocortical carcinoma
  • Primary adrenocortical hyperplasia (uncommon)
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9
Q

What are causes of ectopic ACTH production?

  • Lab values
  • Epidemiology
A

Paraneoplastic syndrome

Labs:

  • High ACTH
  • High cortisol

Epidemiology:

  • More males
  • 40s-50s

Causes:

  • Small cell carcinoma of lung (#1)
  • Carcinoid tumor (typ small intestine/colon), Medullary thyroid CA, Islet cell tumor of pancreas
  • Ectopic CRH secretion also possible (rare)
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10
Q

What is seen here?

A

Adrenal hyperplasia

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11
Q

What is seen here?

A

Tumor of the adrenal cortex

  • Much fat (medulla will look different; different tissue composition)

Microscopically, see very different cell size/morphology

  • Hard to tell benign vs. malignant with adrenal tumors - Even benign may look scary
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12
Q

What is the pathology of Cushing’s syndrome on the pituitary?

A

Crooke hyaline change

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13
Q

What is the pathology of Cushing’s syndrome on the adrenal?

A
  • Atrophy: if low ACTH (exogenous steroids, functioning tumor)
  • Diffuse hyperplasia: if high ACTH (hypothalamic-pituitary)
  • Nodular hyperplasia: if high ACTH (hypthalamic pituitary)
  • Mass
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14
Q

How do you make the lab diagnosis of Cushing’s syndrome?

A
  • 24 hour urine free cortisol level increased due to loss of normal diurnal pattern of cortisol secretion
  • Serum ACTH level
  • Dexamethasone suppression test
  • Urinary 17-hydroxycorticosteroid level
  • After low dose and high dose dexamethasone
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15
Q

What is seen in the various causes of Cushing’s (pituitary disease, ectopic ACTH, and adrenal disease) for:

  • ACTH levels
  • Cortisol levels
  • Response to low and high dose Dex
A

Pituitary disease: high ACTH, high cortisol

  • Low dose dex -> no suppression of 17OH-CCS
  • High dose dex -> suppression of 17OH-CCS

Ectopic ACTH: high ACTH, high cortisol

  • Low dose dex -> no suppression
  • High dose dex -> no suppression

Adrenal disease: low ACTH, high cortisol

  • Low dose dex -> no suppression
  • High dose dex -> no suppression
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16
Q

What are clinical and lab features of hyperaldosteronism (excess mineralocorticoids)?

A

Sodium retention and potassium excretion

  • Hypernatremia (increased fluid volume)
  • Hypokalemia (weakness, paresthesia, visual disturbance, tetany)
  • Hypertension
  • EKG changes, cardiac decompensation

Primary (adrenal)

  • Suppression of renin-angiotensin system
  • Low renin

Secondary (extra-adrenal)

  • Activation of renin-agniotensin system. High renin
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17
Q

What are causes of secondary hyperaldosteronism?

A

RAAS system

  • Decreased renal perfusion
  • Renal artery stenosis
  • Arteriolar sclerosis
  • Arterial hypovolemia and edema
  • CHF
  • Cirrhosis
  • Nephrotic syndrome
  • Pregnancy
  • Estrogen-induced increase in plasma renin
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18
Q

What are causes of primary hyperaldosteronism?

A

Adrenocortical neoplasm

  • Most often adenoma (Conn syndrome)

Primary adrenocortical hyperplasia

  • Idiopathic
  • May be genetic
  • Cells resemble ZG

Glucocorticoid-remediable hyperaldosteronism

  • Uncommon
  • Genetic in some families
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19
Q

What is Conn syndrome?

  • Epidemiology
  • Gross appearance
  • Histo features
A

Adenoma (causing primary hyperaldosteronism)

Epidemiology

  • Adults, 2x more females
  • 30s-40s

Features:

  • Small bright yellow nodule.
  • May have spironolactone bodies.
  • May be multiple adenomas.
  • No associated cortical atrophy due to normal ACTH
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20
Q

What are adrenogenital syndromes

A

Excess androgens causing virilization

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21
Q

What is broad pathway for androgen production?

A

Dehydroepiandrosterone (DHEA) and androstenedione produced by adrenal cortex

  • Converted to testosterone in peripheral tissues
  • Production regulated by ACTH
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22
Q

What are causes of adrenogenital syndromes?

A
  • Adrenocortical neoplasms
  • More often carcinomas
  • Often association with Cushing syndrome
  • Congenital Adrenal Hyperplasia (CAH)
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23
Q

What is Congenital Adrenal Hyperplasia

  • Sporadic or genetic
  • Mechanism
A

Inherited AR metabolic disorders of steroid biosynthesis (CYP21B gene defect)

Deficient or absent activity of enzymes:

  • 21-hydroxylase (90%)
  • Other: 17-hydroxylase, 11-hydroxylase

Decreased cortisol (+/- aldosterone) production

  • Alternate pathway of steroidogenesis results in excess androgens
  • Increased ACTH – Adrenocortical hyperplasia
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24
Q

Describe the genital defect in CAH

A

Inherited AR metabolic disorders of steroid biosynthesis (CYP21B gene defect)

  • Abnormal recombination with non-functional CYP21A, a neighboring “pseudogene”
  • Highest carriers: Hispanic, Ashkenazi Jewish
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25
Q

What happens with 21-hydroxylase deficiency in CAH (congenital adrenal hyperplasia)?

A
  • Defective conversion of progesterone to 11-deoxycorticosterone (aldosterone pathway)
  • Defective conversion of 17-OH progesterone to 11-deoxycortisol (cortisol pathway)
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26
Q

What are the clinical features of Congenital Adrenal Hyperplasia?

A

Variable; Mild to Severe (life-threatening)

  • Androgen excess
  • Ambiguous genitalia at birth: masculinization in females and precocious puberty in males
  • Variable aldosterone and glucocorticoid deficiency
  • Salt-wasting (if low aldosterone production)
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27
Q

What are the different CAH syndromes?

A
  • Salt-wasting (classic) form
  • Simple virilizing form
  • Late-onset (non classic) form
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28
Q

What is seen with salt-wasting (Classic) form of CAH?

A
  • Absent activity: most severe disease; seen at birth.
  • Ambiguous genitalia
  • More easily recognized in girls
  • Salt-wasting
  • Hyponatremia, hyperkalemia, hypotension, vomiting, dehydration, acidosis, cardiovascular collapse, death
  • Also, impaired catecholamine production
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29
Q

What is seen with simple virilizing form of CAH?

A
  • Decreased activity; less severe disease
  • Ambiguous genitalia, but no salt-wasting
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30
Q

What is seen with Late-onset (non-classic) form of CAH?

A
  • Most common
  • Mildest disease
  • Recognized late in life
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31
Q

What is the pathology seen in CAH?

A

Bilateral adrenocortical hyperplasia

  • 10-15 x normal weight
  • Diffusely thick and nodular cortex
  • May be tan-brown due to decreased lipid.
  • Secondary hyperplasia of ACTH-producing corticotroph cells in pituitary
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32
Q

What may cause adrenocortical insufficiency?

A

Primary hypoadrenalism

  • Loss of cortex
  • CAH, Adrenoleukodystrophy, Autoimmune adrenal insufficiency, infection, amyloidosis, sarcoidosis, hemochromatosis, metastasis
  • Metabolic failure
  • CAH, Drug and steroid-induced inhibition

Secondary hypoadrenalism

  • Hypothalamic-Pituitary disease
  • Neoplasm, inflammation
  • Hypothalamic-Pituitary suppression
  • Long-term steroid administration, steroid-producing neoplasms
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33
Q

What is adrenal crisis? What causes it?

A

Acute adrenocortical insufficiency

Caused by:

  • Stress crisis in patients with chronic adrenal insufficiency
  • Rapid withdrawal of exogenous steroids
  • Massive adrenal hemorrhage
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34
Q

What may result in massive adrenal hemorrhage and constitute and adrenal crisis (acute adrenocortical insufficiency)?

A

- Neonate after prolonged delivery, trauma, hypoxia

- Anticoagulation therapy

- DIC

- Waterhouse-Friderichsen Syndrome (bacteremia): picture below

  • Neisseria meningitidis (Meningococcemia)
  • Other: Pseudomonas, S. pneumoniae, H. flu, Staphylococcus
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35
Q

What is Addison disease?

A

Primary chronic adrenal insufficiency

36
Q

What are clinical features of Addison disease?

A

- Insidious onset

  • Progressive weakness, fatiguability, GI disturbance (anorexia, N/V/D, weight loss), Skin hyperpigmentation, hyperkalemia, hyponatremia, volume depletion, hypotension, hypoglycemia

- Acute adrenal crisis

  • Triggered by stress (infection, trauma, surgery)
  • Life-threatening
37
Q

What are causes of primary chronic adrenal insufficiency (Addison disease)?

A
  • Autoimmune adrenalitis (60-70%)
  • Tuberculosis (historically, 90%)
  • Fungal (histoplasma, coccidioides)
  • AIDS (CMV, MAI, KS)
  • Metastatic cancer (Lung, breast)
  • Also: GI, lymphoma, melanoma
  • Genetic disorders
  • Adrenoleukodystrophy
  • Congenital adrenal hypoplasia
38
Q

What is seen here?

A

Progressive destruction of adrenal glands

  • Very small and atrophic with few residual cortical cells
  • Lymphocytes
39
Q

What is Autoimmune Adrenalitis

  • Epidemiology
  • Types
A

Epidemiology:

  • More in Caucasian women
  • Autoantibodies to 21OH-ase, 17OH-ase

Types

- Isolated autoimmune Addison disease

- Autoimmune polyendocrinopathy syndrome type I (APS1)

  • Chronic mucocutaneous candidiasis, skin/nail/enamel abnormalities, hypoadrenalism, hypoparathyroidism, hypogonadism, pernicious anemia
  • AIRE gene

- Autoimmune polyendocrinopathy syndrome type II (APS2)

  • Early adulthood; May be HLA-related
  • Adrenal insufficiency, autoimmune thyroiditis, type I diabetes
40
Q

What are causes of secondary hypoadrenalism? ACTH level?

A
  • Hypothalamic- Pituitary cause
  • Low ACTH
41
Q

What are clinical features of secondary hypoadrenalism?

A
  • Similar to Addison Disease

BUT:

  • Normal or near-normal aldosterone
  • No evidence of skin hyperpigmentation

ACTH administration causes increase in cortisol

  • No response to ACTH in primary adrenal disease due to loss or non-function of adrenal cortex
42
Q

T/F: There is no morphologic distinction between functioning and non-functioning adrenocortical neoplasms

A

True

43
Q

What are broad features of adrenocortical adenomas?

A
  • Often incidental and asymptomatic
  • Well-circumscribed, yellow to yellow-brown (2.5cm)
  • May have “endocrine atypia”
44
Q

What are broad features of adrenocortical carcinomas?

A
  • RARE, adults and children.
  • Larger mass, often necrosis and hemorrhage.
  • Malignancy defined by vascular invasion or mets!
45
Q

What is seen here?

A

Benign adrenocortical adenoma

  • Circumscribed, yellow, sitting in surface
46
Q

What is seen here?

A

Benign adrenocortical adenoma ?

47
Q

What is seen here?

A

Adrenocortical carcinoma

48
Q

What is seen here?

A

Adrenocortical carcinoma

49
Q

What is seen here?

A

Adrenocortical carcinoma

50
Q

What is a pheochromocytoma?

  • Cell type
  • Lab values
  • Clinical presentation
A
  • Chromaffin cells
  • Release catecholamines
  • Elevated urinary VMA, HVA

Clinical presentation

  • Hypertension
  • Paroxysmal BP elevation with tachycardia, HA, sweating, tremor, apprehension. May be life-threatening.
  • Chronic sustained hypertension with some lability
  • Amenable to surgical cure
51
Q

What condition does the rule of 10s apply to?

A

Pheochromocytoma

52
Q

What is the rule of 10s for a pheochromocytoma?

A

Familial, extra-adrenal, bilateral, malignant, childhood

- 10% familial

  • MEN2A/2B
  • NF1
  • VHL, SW

- 10% extraadrenal

  • Paraganglioma

- 10% bilateral

- 10% malignant

- 10% childhood

53
Q

Describe the appareance/characteristics of a pheochromocytoma

  • Size
  • EM
A
  • Variable size, avg 100g
  • Often hemorrhagic
  • Dark brown in Zenker’s fixative (potassium dichromate) – oxidation of catecholamines
  • “Zellballen”; vascular
  • Stippled chromatin and nuclear pleomorphism
  • Neural markers (chromogranin, synaptophysin)
  • EM: electron dense granules
54
Q

Are pheochromocytomas more commonly benign or malignant?

  • Histologic distinction
A

Benign

  • No reliable histological criteria to differentiate
  • Defined by mets
55
Q

What is seen here?

A

Pheochromocytoma

56
Q

What is seen here?

A

Pheochromocytoma

57
Q

What is seen here?

A

Pheochromocytoma

58
Q

What is Multiple Endocrine Neoplasia?

  • Associations?
A

Endocrine hyperplasia, adenomas, carcinomas

Associated with:

  • Younger age
  • Multiple organ sites (synchronous or metachronous)
  • Multifocality
  • Asymptomatic stage of hyperplasia
  • More aggressive course (recurrence)
59
Q

What are the features/conditions of MEN1?

A

3Ps:

  • Pituitary adenomas
  • Parathyroid (hyperplasia, adenomas)
  • Pancreas (endocrine tumors: gastrinoma, insulinoma)

Also, gastrinomas in duodenum, carcinoid tumors, thyroid and adrenal adenomas, lipomas

60
Q

What are the features/conditions of MEN2A?

  • Gene involved?
A

MEN2A = RET gene

  • Medullary thyroid CA (100%)
  • Pheochromocytoma (40-50%)
  • Parathyroid hyperplasia (10-20%)
  • Also, Familial Medullary Thyroid CA syndrome
61
Q

What are the features/conditions of MEN2B?

  • Gene involved?
A

MEN2B = RET gene

  • Medullary thyroid CA
  • Pheochromocytoma
  • Mucosal neuromas
  • Skin, oral, eyes, respiratory, GI
  • Marfanoid features
62
Q

What is seen here?

A

?

63
Q

What are the most common extracranial solid tumors of childhood?

A

Neuroblastic tumors

  • Often in infants and kids under 5
64
Q

What is the spectrum of differentiation with neuroblastic tumors?

A
  • Neuroblastoma
  • Ganglioneuroblastoma
  • Ganglioneuroma
65
Q

Where can neuroblastic tumors occur?

A
  • Adrenal gland

Also:

  • Retroperitoneal
  • Posterior mediastinal
66
Q

What is seen here?

A

Neuroblastoma of adrenal gland?

67
Q

What is seen here?

A

Neuroblastoma of adrenal gland?

68
Q

Embryology of the thymus?

A

Derived from 3rd (and 4th) pharyngeal pouches

69
Q

What cell types are found in the thymus?

A
  • Thymic epithelial cells
  • Lymphocytes
  • Few B cells, macrophages, DCs
  • Rare neutrophils and eosinophils
70
Q

What immune organ has Hassal’s corpuscles?

A

Thymus

71
Q

What is seen here?

A

Thymus..?

72
Q

Describe the structure of the thymus (micro)

A
  • Has lobes and lobules
  • Encapsulated
  • Reticulum of epithelial cells
  • Cortex with blood-thymus barrier
  • Medulla
  • Hassall’s corpuscles
73
Q

What is seen here?

A

Normal thymus structure

74
Q

What is seen here?

A

Thymus: Hassal’s corpuscles (?)

75
Q

SINCE LECTURE WASN’T FINISHED IN THE STREAM, THYMUS PATHOLOGY WILL NOT BE COVERED ON THE TEST

A

SINCE LECTURE WASN’T FINISHED IN THE STREAM, THYMUS PATHOLOGY WILL NOT BE COVERED ON THE TEST

76
Q

Describe the development of the thymus

A

Growth during childhood, followed by involution

  • 10-35 g at birth
  • 20-50 g at puberty (maximum)
  • 5-15 g in adults, replaced by fatty tissue
77
Q

What can cause rapid involution of the thymus?

  • Histologic features?
A

Stress/illness

  • Blurred corticomedullary junction
  • “Starry-sky” appearance
  • Lymphocyte depletion
78
Q

Where is the thymus located?

A

Inferior to thyroid gland; more in the thorax

79
Q

What are the different types of thymic hyperplasia?

  • Histologic characteristics?
A

True thymic hyperplasia

  • Increased size and weight
  • Histologically normal

Follicular hyperplasia

  • Lymphoid follicles (germinal centers)
80
Q

What can cause follicular hyperplasia?

A
  • Associated with myasthenia gravis (65-75%)
  • Other chronic inflammatory conditions
  • Grave’s disease
  • SLE
  • Scleroderma
  • RA
81
Q

What is seen here?

A

Thymic hyperplasia??

82
Q

What are the different thymic neoplasms?

  • Subtypes
A

Thymoma

  • Benign
  • Medullary type (spindled)
  • Mixed med-cortical
  • Malignant
  • Malignant thymoma type 1 (invasive)
  • Malignant thymoma type 2 (thymic carcinoma)
83
Q

Describe thymoma

  • Cells involved
  • Epidemiology
  • Location
  • DDx
  • Clinical features
A
  • Tumor of thymic epithelium

Also lymphocytes in the background

Epidemiology:

  • Adults > 40 yo
  • Equal genders

Location: superior mediastinum

DDx:

  • Lymphoma
  • Germ cell
  • Carcinoid

Clinical features:

  • Local mass effect (40%)
  • Myasthenia (30-45%)
  • Incidental
  • Paraneoplastic phenomena (acquired hypogram, pure red cell aplasia, Grave’s, pernicious anemia, DM-PM, Cushing’s); possibly with abnormal T cell selection in tumor environment
84
Q

What is malignant thymoma type 1?

  • Benign or malignant
  • Cells involved
  • Prognosis
A

Invasive thymoma

  • Cytologically benign
  • Mostly cortical (epithelial)
  • Locally invasive; prognosis related to invasion:
  • >90% minimal invasion
  • 50% extensive invasion
85
Q

What is malignant thymoma type 2?

  • Histology
  • Prognosis
  • Subtypes
A

Thymic carcinoma

  • Cytologially malignant
  • Biologically aggressive

May be:

  • Squamous
  • Lymphoepithelioma-like
  • Sarcomatoid
  • Basaloid
  • Clear cell
86
Q

What is seen here?

A

Medullary ?

87
Q

What is seen here?

A

Cortical ?

MS2 Endocrine (25 decks)

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