Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

IMPORTANT

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is seen with adrenocortical hyperfunction (syndromes)?

A

Hyperadrenalism: syndromes with overlapping clinical features

  • Hypercortisolism (Cushing’s)
  • Hyperaldosteronism
  • Adrenogenital (virilizing) syndromes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is seen here?

A

Adrenal hyperplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

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

A

Crooke hyaline change

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What are adrenogenital syndromes

A

Excess androgens causing virilization

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What are causes of adrenogenital syndromes?

A
  • Adrenocortical neoplasms
  • More often carcinomas
  • Often association with Cushing syndrome
  • Congenital Adrenal Hyperplasia (CAH)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
What happens with 21-hydroxylase deficiency in CAH (congenital adrenal hyperplasia)?
- Defective conversion of progesterone to 11-deoxycorticosterone (aldosterone pathway) - Defective conversion of 17-OH progesterone to 11-deoxycortisol (cortisol pathway)
26
What are the clinical features of Congenital Adrenal Hyperplasia?
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)
27
What are the different CAH syndromes?
- Salt-wasting (classic) form - Simple virilizing form - Late-onset (non classic) form
28
What is seen with salt-wasting (Classic) form of CAH?
- 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
29
What is seen with simple virilizing form of CAH?
- Decreased activity; less severe disease - Ambiguous genitalia, but no salt-wasting
30
What is seen with Late-onset (non-classic) form of CAH?
- Most common - Mildest disease - Recognized late in life
31
What is the pathology seen in CAH?
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
32
What may cause adrenocortical insufficiency?
**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
33
What is adrenal crisis? What causes it?
**Acute adrenocortical insufficiency** _Caused by_: - Stress crisis in patients with chronic adrenal insufficiency - Rapid withdrawal of exogenous steroids - Massive adrenal hemorrhage
34
What may result in massive adrenal hemorrhage and constitute and adrenal crisis (acute adrenocortical insufficiency)?
**- Neonate after prolonged delivery, trauma, hypoxia** **- Anticoagulation therapy** **- DIC** **- Waterhouse-Friderichsen Syndrom**e (bacteremia): picture below * Neisseria meningitidis (Meningococcemia) * Other: Pseudomonas, S. pneumoniae, H. flu, Staphylococcus
35
What is Addison disease?
Primary chronic adrenal insufficiency
36
What are clinical features of Addison disease?
**- 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
What are causes of primary chronic adrenal insufficiency (Addison disease)?
- 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
What is seen here?
Progressive destruction of adrenal glands - Very small and atrophic with few residual cortical cells - Lymphocytes
39
What is Autoimmune Adrenalitis - Epidemiology - Types
_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
What are causes of secondary hypoadrenalism? ACTH level?
- Hypothalamic- Pituitary cause * Low ACTH
41
What are clinical features of secondary hypoadrenalism?
- 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
**T/F**: There is no morphologic distinction between functioning and non-functioning adrenocortical neoplasms
True
43
What are broad features of adrenocortical adenomas?
- Often incidental and asymptomatic - Well-circumscribed, yellow to yellow-brown (2.5cm) - May have “endocrine atypia”
44
What are broad features of adrenocortical carcinomas?
- RARE, adults and children. - Larger mass, often necrosis and hemorrhage. - Malignancy defined by vascular invasion or mets!
45
What is seen here?
Benign adrenocortical adenoma - Circumscribed, yellow, sitting in surface
46
What is seen here?
Benign adrenocortical adenoma ?
47
What is seen here?
Adrenocortical carcinoma
48
What is seen here?
Adrenocortical carcinoma
49
What is seen here?
Adrenocortical carcinoma
50
What is a pheochromocytoma? - Cell type - Lab values - Clinical presentation
- 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
What condition does the rule of 10s apply to?
Pheochromocytoma
52
What is the rule of 10s for a pheochromocytoma?
Familial, extra-adrenal, bilateral, malignant, childhood **- 10% familial** * MEN2A/2B * NF1 * VHL, SW **- 10% extraadrenal** * Paraganglioma **- 10% bilateral** **- 10% malignant** **- 10% childhood**
53
Describe the appareance/characteristics of a pheochromocytoma - Size - EM
- 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
Are pheochromocytomas more commonly benign or malignant? - Histologic distinction
Benign - No reliable histological criteria to differentiate - Defined by mets
55
What is seen here?
Pheochromocytoma
56
What is seen here?
Pheochromocytoma
57
What is seen here?
Pheochromocytoma
58
What is Multiple Endocrine Neoplasia? - Associations?
Endocrine hyperplasia, adenomas, carcinomas _Associated with:_ - Younger age - Multiple organ sites (synchronous or metachronous) - Multifocality - Asymptomatic stage of hyperplasia - More aggressive course (recurrence)
59
What are the features/conditions of MEN1?
3Ps: - **Pituitary adenomas** - **Parathyroid** (hyperplasia, adenomas) - **Pancreas** (endocrine tumors: gastrinoma, insulinoma) Also, gastrinomas in duodenum, carcinoid tumors, thyroid and adrenal adenomas, lipomas
60
What are the features/conditions of MEN2A? - Gene involved?
MEN2A = RET gene - Medullary thyroid CA (100%) - Pheochromocytoma (40-50%) - Parathyroid hyperplasia (10-20%) - Also, Familial Medullary Thyroid CA syndrome
61
What are the features/conditions of MEN2B? - Gene involved?
MEN2B = RET gene - Medullary thyroid CA - Pheochromocytoma - Mucosal neuromas * Skin, oral, eyes, respiratory, GI - Marfanoid features
62
What is seen here?
?
63
What are the most common extracranial solid tumors of childhood?
Neuroblastic tumors - Often in infants and kids under 5
64
What is the spectrum of differentiation with neuroblastic tumors?
- Neuroblastoma - Ganglioneuroblastoma - Ganglioneuroma
65
Where can neuroblastic tumors occur?
- Adrenal gland Also: - Retroperitoneal - Posterior mediastinal
66
What is seen here?
Neuroblastoma of adrenal gland?
67
What is seen here?
Neuroblastoma of adrenal gland?
68
Embryology of the thymus?
Derived from 3rd (and 4th) pharyngeal pouches
69
What cell types are found in the thymus?
- Thymic epithelial cells - Lymphocytes - Few B cells, macrophages, DCs - Rare neutrophils and eosinophils
70
What immune organ has Hassal's corpuscles?
Thymus
71
What is seen here?
Thymus..?
72
Describe the structure of the thymus (micro)
- Has lobes and lobules - Encapsulated - Reticulum of epithelial cells - Cortex with blood-thymus barrier - Medulla - Hassall's corpuscles
73
What is seen here?
Normal thymus structure
74
What is seen here?
Thymus: Hassal's corpuscles (?)
75
SINCE LECTURE WASN'T FINISHED IN THE STREAM, THYMUS PATHOLOGY WILL NOT BE COVERED ON THE TEST
SINCE LECTURE WASN'T FINISHED IN THE STREAM, THYMUS PATHOLOGY WILL NOT BE COVERED ON THE TEST
76
Describe the development of the thymus
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
What can cause rapid involution of the thymus? - Histologic features?
Stress/illness - Blurred corticomedullary junction - “Starry-sky” appearance - Lymphocyte depletion
78
Where is the thymus located?
Inferior to thyroid gland; more in the thorax
79
What are the different types of thymic hyperplasia? - Histologic characteristics?
**True thymic hyperplasia** - Increased size and weight - Histologically normal **Follicular hyperplasia** - Lymphoid follicles (germinal centers)
80
What can cause follicular hyperplasia?
- Associated with myasthenia gravis (65-75%) - Other chronic inflammatory conditions * Grave's disease * SLE * Scleroderma * RA
81
What is seen here?
Thymic hyperplasia??
82
What are the different thymic neoplasms? - Subtypes
Thymoma - Benign * Medullary type (spindled) * Mixed med-cortical - Malignant * Malignant thymoma type 1 (invasive) * Malignant thymoma type 2 (thymic carcinoma)
83
Describe thymoma - Cells involved - Epidemiology - Location - DDx - Clinical features
- 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
What is malignant thymoma type 1? - Benign or malignant - Cells involved - Prognosis
Invasive thymoma - Cytologically benign - Mostly cortical (epithelial) - Locally invasive; prognosis related to invasion: * \>90% minimal invasion * 50% extensive invasion
85
What is malignant thymoma type 2? - Histology - Prognosis - Subtypes
Thymic carcinoma - Cytologially malignant - Biologically aggressive _May be:_ - Squamous - Lymphoepithelioma-like - Sarcomatoid - Basaloid - Clear cell
86
What is seen here?
Medullary ?
87
What is seen here?
Cortical ?

MS2 Endocrine (25 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions