Parathyroid and Adrenal Pathology- Hunt Flashcards

1
Q

What are some potential developmental abnormalities of the parathyroid glands?

A
  • Ectopic glands
    • In thymus
    • Anterior mediastinum
    • Carotid sheath
  • 10% of people have only 2-3 glands instead of 4
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2
Q

How does a parathyroid gland appear grossly?

How much does one weigh?

A

A yellow-brown ovoid nodule

30-45mg

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3
Q

The parathyroid gland parenchyma is made up of what two cell types?

Which cell types secretes PTH?

What other tissue type has a noticeable presence?

A
  • Parenchyma:
    • Mostly Chief cells (secrete PTH)
    • Some oxyphil cells
  • Large amount of stromal fat
    • ​30-70%
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4
Q

Name three causes of primary hyperparathyroidism (increased PTH secretion) and their relative occurance rates.

A
  • Hyperplasia (10-15%)
  • Adenoma (75-80%)
  • Carcinoma (<5%)
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5
Q

Name four general causes of parathyroid hypofunction (decreased PTH secretion).

A
  • Congenital (DiGeorge)
  • Iatrogenic (surgery)
  • Familial (genetic)
  • Autoimmune
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6
Q
  1. How common is primary hyperparathyroidism in general?
  2. Is there a sex predisposition?
  3. What age group it is typically seen in?
A
  1. 25:100,000 [aka 1:4000?]
  2. F:M = 3:1
  3. Middle age to older adults
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7
Q

List three types of bone disease that can occur secondary to hyperparathyroidism.

A
  1. Osteitis fibrosa cystica
  2. Brown tumor
  3. Osteoporosis
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8
Q
  1. What is the pathogenesis of osteitis fibrosa cystica?
  2. How does the bone appear histologically?
A
  1. Erosion of bone matrix by osteoclasts secondary to high PTH
  2. Bone morphology:
    • Grossly thinned cortex
    • Fibrosis of marrow
      • Hemorrhage
      • Cyst formation
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9
Q
  1. What is the pathogenesis of a Brown tumor?
  2. Is it benign or malignant?
  3. How does the bone appear histologically?
A
  1. Excessive osteoclast activity
  2. Benign lesion - cab be mistaken for a bone neoplasm
  3. Appearance:
    • Osteoclasts & reactive giant cells
    • Hemorrhage
    • Morphologically identical to giant cell tumor of bone
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10
Q
  1. How many of the parathyroid glands does parathyroid adenoma typically affect?
  2. How will the other glands appear?
  3. Typically, how large (mass) is a parathyroid adenoma?
A
  1. Just one
  2. Normal or atrophic (negative feedback)
  3. 0.5-5.0g
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11
Q

How does a parathyroid adenoma appear histologically?

A
  • Sheets of chief cells
  • Decreased stromal fat
  • Oxyphils may be present
  • May show a rim of normal paraythyroid at the periphery
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12
Q

How many glands are affected in parathyroid hyperplasia?

A
  • Classically, all four
  • May be relative sparing of one or two glands

(contrast with adenoma)

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13
Q

How does parathyroid hyperplasia appear histologically?

A
  • Chief cell hyperplasia and fat cell loss just as in adenomas
  • Difficult to distinguish from adenoma histologically
    • Key difference: Hyperplasia will lack the rim of normal tissue that adenomas typically (but not always) feature
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14
Q
  1. How common is parathyroid carcinoma?
  2. How does it appear histologically?
A
  • Very rare
  • Appearance:
    • Mitotic activity
    • (Sometimes thick) Fibrous bands
    • Capsular / vascular invasion
    • Cellular atypia alone is not a relaible marker
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15
Q
  1. At what stage is parathyroid cancer typically first diagnosed?
  2. What implications does this have for treatment?
A
  1. Usually not until already invasive / metastatic
  2. Difficult to remove from surrounding tissue during surgery
    • fibrous adhesions
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16
Q
  1. How is parathyroid carcinoma diagnosed, as opposed to an adenoma?
  2. What physical exam finding may increase your suspicion of carcinoma over an adenoma?
A
  1. By demonstrating invasion into surrounding tissues or metastasis (which an adenoma would not have)
  2. Carcinomas are typically firm lesions whereas adenomas are soft
17
Q

What are the three zones of the adrenal cortex?

What is produced in each zone?

A

Zona Glomerulosa- aldosterone

Zona Fasciculata- glucocorticoids

Zona Reticularis- Sex hormones

“It gets sweeter as you go deeper”

18
Q

What cells are found in the adrenal medulla?

What is produced in the medulla?

A

Chromaffin cells

catecholamines (epinephrine)

19
Q

What three over-producing pathologies can affect the adrenal cortex?

What over-producing pathology can affect the adrenal medulla?

A

Cortex

  • Adrenocortical hyperplasia
  • Adrenocortical adenoma
  • Adrenocortical carcinoma

Medulla

  • Pheochromocytoma
20
Q

What area of the adrenal gland typically enlarges in adrenocortical hyperplasia?

What cells are mostly affected?

A

Zona fasciculata

Clear cells

21
Q

What area of the adrenal glands does adrenocortical adenoma affect?

What does the adenoma look like grossly?

A

Zona Fasciculata

Yellow, encapsulated

THESE ARE TYPICALLY NON-FUNCTIONAL

22
Q

Adrenocortical carcinoma or adenoma?

Large?

Well-differentiated?

Necrosis?

Pleimorphic?

Common?

A

Large- carcinoma

Well-differentiated- either

Necrosis-carcinoma

Pleimorphic-carcinoma

Common-adenoma

23
Q

Patient present with hypercortisolism. What is the cause if the adrenals show:

atrophy?

bilateral enlargement?

a unilateral mass?

A

atrophy- exogenous glucocorticoids

bilateral enlargement- increased ACTH (pituitary adenoma)

unilateral mass- adrenocortical adenoma/carcinoma

24
Q

Patient presents with hyperaldosteronism. What are they mostly to have?

What is the name for this disease?

A

adrenal cortical adenoma

Conn Syndrome

25
Q

What is congenital adrenal hyperplasia?

What adrenal pathologies are seen?

A

Any recessive mutation that blocks steroidogenesis from the adrenal glands

bilateral hyperplasia

26
Q

What does a pheochromocytoma look like histologically?

What markers could identify this as a pheochromocytoma?

A

Zellballen (cells nests)

granular, basophilic cytoplasm

stain for chromogranin or synaptophysin (neuroendocrine markers)

27
Q

What is a paraganglioma?

Where are they most likely to be found?

A

Extra-adrenal pheochromocytoma

jugulotympanic, carotid body, vagal, aorticopulmonary

28
Q

What is the rule of tens for pheochromocytomas?

A
  • 10% are malignant
  • 10% are bilateral
  • 10% are extra-adrenal
  • 10% are pediatric