Week 3: Parathyroid and Adrenal Gland Flashcards

1
Q

ID

A

Thyroid Gland with the Parathryoid Gland in the top right

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2
Q

ID

A

Normal Parathyroid gland

oxyphil cells (blue arrow), stromal fat (black arrow), and chief cells are all the other darker pink/purple cells

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3
Q

ID

A

Oxyphil cells (left) and Chief/principle cells (right)

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4
Q

ID

A

Parathyroid Adenoma

capsulated Parathyroid Adenoma is lighter in pink (black arrow) and normal PTH tissue (blue arrow)

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5
Q

ID

A

Parathyroid Adenoma

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6
Q

Symptoms of Hypercalcemia

A

Bones (fractures and osteoporosis), Stones (renal), Groans (constipation, nausea, peptic ulcers), Thrones (polyuria), and Psychiatric Overtones (depression, lethargy, seizures)

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7
Q

Severe Hyperparathyroidism is termed

A

von Recklinghausen disease of bone (generalized osteitis fibrosa cystica)

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8
Q

characteristics of von Recklinghausen disease of bone (generalized osteitis fibrosa cystica)

A

osteoporosis (fractures)
dissecting osteitis (osteoclast boring in central trabeculae)
brown tumors (hemosiderin deposition in microfractures)

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9
Q

ID

A

Multiple lytic expansile lesions with ground glass appearance in whole pelvis. Radiograph of right knee shows lytic expansile lesions in the metadiaphyseal region of lower third right femur and upper third of right tibia

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10
Q

ID

A

Dissecting osteitis

Osteoclasts (orange arrows) boring into the center of trabeculae. normal bony matrix surrounded with osteocytes (yellow arrow)

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11
Q

Hypoparathyroidism signs (low calcium levels)

A

Tetany - neuromuscular irritability
Chvostek’s sign - tap on facial nerve to induce muscle contractions
Trousseau sign: carpal tunnel spasms produced by occlusion of circulation

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12
Q

ID the layers of the Adrenal Gland

A
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13
Q

ID

A

normal adrenal gland

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14
Q

ID

A

adrenal gland hyperplasia (increased levels of ACTH)

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15
Q

ID

A

adrenal adenoma

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16
Q

ID

A

adrenal carcinoma (note kidney below for size reference)

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17
Q

ID

A

Adrenal Adenoma

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18
Q

ID

A

Adrenal Carcinoma

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19
Q

What hormones do the layers of the adrenal gland secrete?

A
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20
Q

What is the purpose of a low dose Dexamethasone test?

A

test helps to differentiate healthy people from those who produce too much cortisol

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21
Q

What is the purpose of a high dose Dexamethasone test?

A

determine if the abnormality is in the pituitary gland (Cushing’s disease)

High dose causes suppression of cortisol level in patients with Cushing’s disease (but won’t cause suppression of cortisol in patients with ectopic ACTH producing tumors)

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22
Q

What are the defining features of Cushing Syndrome?

A

HTN and weight gain
Buffalo hump
Moon face
Abdominal striae

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23
Q

What causes Primary Hyperaldosteronism

A

Mostly Aldosterone producing adenomas

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24
Q

Adrenocortical Hypofunction: Massive adrenal hemorrhage - newborns with prolonged, difficult delivery, anticoagulant therapy, DIC with adrenal hemorrhage in setting of bacterial infection (i.e. Neisseria meningitidis)

A

Waterhouse-Friedrichsens syndrome (WFS)

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25
Q

Most common cause of Addison’s disease

A

autoimmune adrenalitis gland destruction

26
Q

What are causes of Addison’s disease that are not autoimmune?

A

Infections (TB and fungal like Histoplasma capsulatum and Coccidioides immitis)
Metastatic disease (lung and breast carcinoma)
Genetics

27
Q

Why do patient’s with Addison’s disease have skin hyperpigmentation (bronzing skin)?

A

elevated levels of pro-opiomelanocortin (POMC) from anterior pituitary. POMC is a precursor of ACTH and melanocyte-stimulating hormone (MSH).

28
Q

ID

A
29
Q

ID

A

Waterhouse-Friderichsen Syndrome
(WFS) - adrenal gland hemorrhage

Usually septicemic from Neisseria gonorrheae

30
Q

ID

A

Hemorrhagic Adrenal Cortex

hemorrhage begins in the medulla thin-walled sinusoids (yellow arrow) and spread to cortex (green arrow)

31
Q

What are two tumors of the Adrenal Medulla

A

Pheochromocytoma (children and adults) and Neuroblastoma (children under 5)

32
Q

What are protein markers on IHC are pheochromocytomas positive for and what other pathology is positive for these same markers?

A

Chromogranin and Synaptophysin

Small Cell Carcinoma and Carcinoid Tumor

33
Q

What is a pheochromocytoma called if it is located outside the adrenal gland

A

paraganglioma

34
Q

What are the characteristic signs of a Pheochromocytoma or paraganglioma?

A

The 5 P’s:
Pressure (HTN)
Pain (Headache)
Palpitations
Perspiration (Diaphoresis)
Pallor (Pale)

35
Q

What are the characteristic features of a neuroblastoma?

A

Proptosis (dropping) and periorbital ecchymosis (racoon eyes - bruising around the eyes)

36
Q

of the 1-2% familial neuroblastomas what is the genetic cause of

A

germline mutation in the ALK gene

37
Q

What lab values would be seen in a neuroblastoma

A

Elevated urine vanillylmandelic acid (VMA) and homovanillic acid (HVA)

38
Q

ID

A

Pheochromocytoma

Can’t identify on scan alone. Need symptoms and markers

39
Q

ID

A

Phemochromocytoma

Can’t identify on biopsy. Need symptoms and markers

40
Q

ID

A

Phemochromocytoma

nest or clusters of spindle shaped chromaffin/chief cells (orange) surrounded by sustentacular cells/Zellballen architecture (yellow arrow)

41
Q

ID

A

Pheochromocytoma

salt and pepper fine chromatin (yellow arrow)

42
Q

ID

A

Pheochromocytoma

You can see catecholamine vesicles on EM

43
Q

ID what cells in a Pheochromocytoma arise from each IHC

A

Left - Synaptophysin (sustentacular cells)
Right - Chromogranin (polygonal/nest/trabeculae cells)

44
Q

ID

A

Disseminated metastatic cutaneous lesions

Used to be called Blueberry Muffin Baby Syndrome. Feature of Neuroblastoma

45
Q

ID

A

Neuroblastoma

Homer Wright pseudo-rosettes (yellow circles; psuedo because there is no lumen and it is light pink neuropil at the center)

46
Q

ID

A

Neuroblastoma

Homer Wright pseudo-rosettes with neuropil centers

47
Q

ID

A

neuroblastoma

neuropil corresponding to neuritic processes of primative neuroblasts (yellow arrow)

48
Q

What determines if a neuroblastoma is high risk?

A

Molecular MCYN amplication

49
Q

What 3 conditons are Multiple endocrine Neoplasia 1 (MEN1)

A

Pituitary Adenoma (most often prolactinoma)
Parathyroid Adenoma
Pancreatic Tumors (PanNETs - Insulinoma, Gastrinoma, and VIPoma)

(3P 0M)

50
Q

What is the genetic cause of MEN1

A

MEN1 tumor suppressor gene that endcodes a protein called menin

51
Q

What 3 conditons are Multiple endocrine Neoplasia 2a (MEN2a)

A

Parathyroid hyperplasia
Pheochromocytoma
Medullary thyroid carcinoma

(2P 1M)

52
Q

What is the genetic cause of MEN2a

A

gain-of-function mutations in RET proto-oncogene

53
Q

What 3 conditons are Multiple endocrine Neoplasia 2b (MEN2b)

A

Pheochromocytoma
Medullary thyroid carcinoma (multifocal and more aggressive than 2a)
Marfanoid body habitus or mucosal neuromas

(1P 2M)

54
Q

What is the genetic cause of MEN2b

A

missense mutation in RET proto-oncogene

55
Q

What Multiple endocrine Neoplasia 4 (MEN4)

A

Features of MEN-1 but with different gene mutation

56
Q

What is the genetic cause of MEN4

A

CDKN1B (cyclin dependent kinase inhibitor 1B) mutations

57
Q

What is the embryology of the adrenal cortex?

A

splanchnic mesoderm (mesothelium)

58
Q

What is the embyrology of the adrenal medulla?

A

Neural Crest

59
Q

What is the embryology of the Parafollicular (C-cells) of the parathryoid gland?

A

Neural Crest

60
Q

What is the embryology of the Parathyroid Glands?

A

Inferior Parathyroid - 3rd pouch (endoderm)
Superior Parathryoid - 4th pouch (endoderm)

61
Q

this portion(s) of the adrenal cortex functions independently of ACTH

A

Zona Glomerulosa

62
Q

this portion(s) of the adrenal cortex functions dependently of ACTH

A

Zonae Fasciculata and Reticularis