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Endorine > Adrenal Pathology (5/16) > Flashcards

Adrenal Pathology (5/16) Flashcards

1
Q

Where are the adrenal glands?

A

In the retroperitoneal tissues at the upper poles of the kidney (4-6g)

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2
Q

What shape is the right adrenal?

A

Pyramidal, behind the right lobe of the liver

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3
Q

What shape is the left adrenal

A

Crescent shape, lies behind the omentum and stomach

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4
Q

Define Addison’s dis

A

Adrenal insufficiency: dec levels of glucocorticoid production which eventually affects production of mineralocorticoids and sex steroids

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5
Q

What is the pathology/cause of Addison’s dis

A
  • Destruction of cortical cells-cortical atrophy
  • Chronic inflamm cells (autoimm)
  • Infectious agents (granulomatous inflammation in TB)
  • Metastatic tumor from other sites
  • Amyloid deposition
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6
Q

What are some clinical features of addison’s

A
  • Fatigue, malaise, weakness, anorexia, vomiting, wt loss, hyperpigmentation, postural hypoT
  • Infectious: fever, septic shock
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7
Q

What are some diseases of adrenal cortex hyperfxn?

A 
Cushings (glucocorticoid xs)
Conn's (aldosterone xs)
Adrogenital syndrome (androgen xs)
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8
Q

What are some causes of cushings?

A

-Bilateral hyperplasia (80%) (pituitary ACTH sec adenoma, ectopic ACTH, primary, idiopathic)

  • Adrenal cortical adenoma (10%)
  • Adrenal cortical carcinoma (10%)
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9
Q

Most common cause of cushings in adults?

A

Hyperplasia

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10
Q

Most common cause of cushings in children?

A

Carcinoma

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11
Q

What are the 3 types of bilateral hyperplasia in cushings?

A 
Simple diffuse (65%, females)
Bilateral nodular (20%, females)
Ectopic (15%, males)
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12
Q

What is adrenal cortical adenoma?

A

Benign neoplastic proliferation of adrenal cortical tissue

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13
Q

What is the gross pathology of adrenal cortical adenoma?

A
  • Unilateral and solitary
  • Well circumscribed
  • Yellow tan
  • <100g
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14
Q

What is the microscopic pathology of adrenal cortical adenoma?

A
  • 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
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15
Q

What is adrenal cortical carcinoma?

A

Malignant neoplasm arising from adrenal cortical cells

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16
Q

What is the gross path of adrenal cortical carcinoma?

A
  • Large bulky tumor
  • Fleshy cut surface with hemorrhage and necrosis
  • > 100g
  • Invasion
17
Q

What is the micro path of adrenal cortical carcinoma?

A
  • Invasion
  • Necrosis
  • Cellular pleomorphism
  • Tumor capsule invasion
  • Vascular invasion
  • Distant metastases (lung and liver most common)
18
Q

What causes hyperaldosteronism?

A
  • Adenoma (65%)
  • Bilateral hyperplasia (35%)
  • Carcinoma (rare)
19
Q

What is Conn’s syndrome

A

Aldosterone XS

20
Q

What is the microscopic pathology of adrenal cortical adenoma causing Conn’s syndrome??

A
  • Variety of cell types

- Most have cells resembling Zona Fasciculata with minority Zona Glomerulosa

21
Q

What are the clinical features of Conn’s dis caused by adrenal cortical adenoma?

A
  • Hyper T
  • Muscle weakness, fatigue, polyuria
  • Periodic paralysis
  • Inc cardiac arrhythmias
22
Q

What are some causes of adrogenital syndrome?

A
  • Congenital adrenal hyperplasia

- Adrenal cortical adenoma (rare)

23
Q

What are some clinical features of adrogenital syndrome?

A
  • F: male hair pattern with facial hair

- M: gynecomastia, dec libido, feminized hair pattern and testicular atrophy

24
Q

What is congenital adrenal hyperplasia?

A

Inherited disorder caused by def of enzymes req for biosynth of glucocort and mineralocort leading to androgen excess

25
Q

What is the pathology of congenital adrenal hyperplasia?

A

Enlarged/hyperplastic adrenal glands leading to hyperandrogenism

26
Q

What are some causes of adrenal medulla hyperfxn?

A
  • Neoplasm (pheochromocytoma)
  • Hyperplasia
  • Neuroblastoma
27
Q

What is pheochromocytoma?

A

AKA: Adrenal medullary paraganglioma

  • Catecholamine secreting tumor arising from chromaffin cells of adrenal medulla
  • Ie hyperfxn of adrenal medulla
28
Q

What are the clinical features of pheochromocytoma?

A
  • Mostly sporadic, 20-35% familial, autosomal dominant
  • Excess catecholamine: hyperT, palpitations, headaches, diaphoresis, flushing, anxiety, nausea, pain and epistaxis
  • Elevated urine catecholamines
29
Q

What are some familial syndromes associated with pheochromocytoma?

A
  • Mult endocrine neoplasia type 2A or 2B
  • Von hippel lindau syndrome
  • neurofibromatosis
  • germline mutation in SDHB, SDHD, VHL, RET or NF1
30
Q

What are the gross findings of pheochromocytoma?

A
  • Sporadic: unilateral
  • Familial: bilat or mult tumors
  • Well circumscribed
  • 6cm
  • Firm cut surface, vascular (endocrine tumors are vascular)
31
Q

What are the microscopic findings of pheochromocytoma?

A
  • 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
32
Q

What are multiple endocrine neoplasia syndromes (MEN)>

A

Genetic syndromes in which mult endocrine organs are involved. Signs and symp vary because of this. Can also have lesions in non endocrine systems

33
Q

What are the 3 MEN syndromes

A
  1. MEN 1 (werner)
  2. MEN2A (sipple)
  3. MEN 2B
34
Q

What is MEN 1?

A

Neoplasia of pituitary, parathy, pancreatic islets (determines clinical prognosis) . MENIN gene is involved with mutl sites of mutations

35
Q

What are MEN 2A and 2B mutations in and what is the most impt prognostic lesion?

A

Mutations in ret protooncogene. Medullary thyroid carcinoma is the most prognostically impt lesion, can be fatal

36
Q

What is men 2A?

A

Neoplasia and hyperplasia of Thyroid C cells, adrenal medulla, parathyroid

37
Q

What is MEN 2B?

A

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

Endorine (24 decks)

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