Endocrine 2 Flashcards

1
Q
A

Normal Endocrine Pancreas

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2
Q
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Normal Endocrine Pancreas

Antibody staining for glucagon in alpha-cells

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3
Q
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Normal Endocrine Pancreas

Antibody staining for insulin in beta-cells

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4
Q
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Diabetes Mellitus Type 1

Autoimmune destruction of islets.

Inflammation and infiltration by T lymphocytes (dark cells) = insulinitis

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5
Q
A

Diabetes Mellitus Type 1

Deposits of extracellular amyloid composed of amylin, which is co-secreted with insulin

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6
Q
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Diabetes Mellitus Type 1

Congo red stain of amyloid

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7
Q
A

Diabetes Mellitus Type 1

Amyloid deposits

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8
Q

Diabetes Complications (Picture 1)

A
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9
Q

Diabetes Complications (Picture 2)

A
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10
Q
A

Pancreatic Endocrine Neoplasm

(Islet Cell Tumor)

Circumscribed/encapuslated.

In pancreas or region around pancreas.

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11
Q
A

Pancreatic Endocrine Neoplasm

(Islet Cell Tumor - on the left)

Circumscribed/encapuslated.

In pancreas or region around pancreas.

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12
Q
A

Pancreatic Endocrine Neoplasm

(Islet Cell Tumor - arranged in groups)

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13
Q
A

Pancreatic Endocrine Neoplasm

(Islet Cell Tumor - cells are well-differentiated)

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14
Q
A

Insulinoma

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15
Q

-omas

A
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16
Q
A

Necrolytic Migratory Erythema

(Glucagonoma)

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17
Q

Gastrinoma Triangle

A
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18
Q
A

Normal Parathyroid Gland

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19
Q
A

Acini of cheif cells; granular cells = oxyphil cells

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20
Q
A

Parathyroid Hyperplasia

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21
Q
A

PT Hyperplasia

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22
Q

Manifestations of Parathyroid Adenoma

A
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23
Q
A

Parathyroid Hyperplasia

Sestamibi Scan

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24
Q
A

Parathyroid Adenoma

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25
Q
A

Parathyroid Adenoma

Solid sheets of cells.

Adenomas have compressed non-neoplastic tissue at edges.

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26
Q
A

Parathyroid Adenoma

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27
Q
A

Parathyroid Adenoma

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28
Q
A

Parathyroid Adenoma

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29
Q
A

Parathyroid Carcinoma

Very uncommon.

Tend to have higher Ca++ levels because they’re larger.

Invasive to soft tissues of the neck.

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30
Q
A

Parathyroid Carcinoma.

Bands of fibrous tissue.

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31
Q
A

Normal Adrenal Gland

Cortex is yellowish.

Medulla is reddish-brown.

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32
Q
A

Adrenal Gland

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33
Q
A

Cortex

Zona Glomerulosa

Zona Fasciculata

Zona Reticularis

Medulla

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34
Q
A

Cortex

Zona Glomerulosa

Zona Fasciculata

Zona Reticularis

Medulla

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35
Q
A

Left: Zona Fasciculata

Right: Zona Reticularis

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36
Q
A

Waterhouse-Friderichsen Syndrome purpura

Acute adrenal insufficiency due to DIC/hemorrhage caused by Neiserria meningititides infection.

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37
Q
A

Waterhouse-Friderichsen Syndrome

Hemorrhagic necrosis of adrenal glands.

Acute adrenal insufficiency due to DIC/hemorrhage caused by Neiserria meningititides infection.

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38
Q
A

Waterhouse-Friderichsen Syndrome

Hemorrhage in adrenal glands.

Acute adrenal insufficiency due to DIC/hemorrhage caused by Neiserria meningititides infection.

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39
Q
A

Waterhouse-Friderichsen Syndrome

Acute adrenal insufficiency due to DIC/hemorrhage caused by Neiserria meningititides infection.

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40
Q
A

Waterhouse-Friderichsen Syndrome

Hemorrhagic necrosis of adrenal glands.

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41
Q
A

Adrenal Tuberculosis

Caseous Necrosis

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42
Q
A

Adrenal Tuberculosis

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43
Q
A

Adrenal Tuberculosis

Histiocytes or macrophages

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44
Q
A

Adrenal Tuberculosis

Activated T cells

Langhans nucleated cells

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45
Q
A

Adrenal Tuberculosis

Acid Fast Bacilli

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46
Q
A

Adrenal Cortical Adenoma

47
Q
A

Adrenal Cortical Adenoma

48
Q
A

Adrenal Cortical Adenoma

49
Q
A

Adrenal Cortical Adenoma

50
Q
A

Adrenal Cortical Adeonoma

Left: tumor

Right: Residual normal adrenal tissue

51
Q
A

Adrenal Cortical Adenoma

Right: tumor, often with much lipid

52
Q
A

Adrenal Cortical Adenoma

Uniform, arranged in nests.

53
Q
A

Adrenal Cortical Carcinoma

54
Q
A

Adrenal Cortical Carcinoma

55
Q
A

Adrenal Cortical Carcinoma

56
Q
A

Adrenal Cortical Carcinoma

57
Q
A

Adrenal Cortical Carcinoma

58
Q
A

Adrenal Cortical Carcinoma

More mitotically active.

59
Q
A

Adrenal Cortical Carcinoma

60
Q
A

Adrenal Cortical Carcinoma

Vascular invasion, metastasis = malignancy

61
Q
A

Pheochromocytoma

62
Q
A

Pheochromocytoma

Adrenal medulla turns dark brown in a solution of chromium salts (oxidizes cathecholamines)

63
Q
A

Pheochromocytoma

64
Q
A

Pheochromocytoma

65
Q
A

Pheochromocytoma

66
Q
A

Pheochromocytoma

Secretory granules with catecholamines

Present with HTN intermittently

Can measure urinary metanephrines/VMA

67
Q
A

Neuroblastoma

68
Q
A

Neuroblastoma

69
Q
A

Neuroblastoma

70
Q
A

Neuroblastoma

Tumor cells form rosettes around a central anucleated zone.

71
Q
A

Neuroblastoma

Left: area of necrosis

72
Q
A

Multiple Endocrine Neoplasia

73
Q
A

Multiple Endocrine Neoplasia 2B

Mucosal neuromas

Have C cell hyperplasia nodules

74
Q
A

Multiple Endocrine Neoplasia 2B

75
Q

Islets of Langerhans

A

Clusters of uniform cells scattred within exorine pancreas

76
Q

4 major and 2 minor cells types of endocrine pancreas and their hormones

A
  1. Beta cell: insulin
  2. Alpha cell: glucagon
  3. delta cell: somatostain
  4. PP cell: pancreatic polypeptide
  5. D1 cell: vasoactive intestinal polypeptide
  6. Enterochromafiin cell: serotonin
77
Q

Gross findings of DM pancreas

A

Pancreas may be small, atropic

78
Q

Microscopic findings in DM pancreas (Types 1, 2)

A

Type 1: reduction in number and size of islets;

leukocytic infiltration of islets (“insulinitis”)

Type 2: hyalinization/fibrosis of islets;

amyloid deposition (compose of amylin - cosecreted with insulin)

79
Q

Comparison of Type 1 and Type 2 Diabetes Mellitus

A
80
Q

Pancreatic Endocrine Neoplasm:

Epidemiology

Area of pancreas that is affected

Functional?

Histology

A

Epi: much less common than tumor of exocrine pancreas; adults primarily affected; single or multiple; benign or malignant

Area: Usually body or tail of pancreas

Functional: 60% secrete one or several peptide hormones

Histo: uniform cells with round/oval nuclei, fine chromatin, and pale granular cytoplasm

81
Q

Presentation of Insulinoma

A

Most common functioning islet cell tumor.

Presents with episodic hypoglycemic attacks, low blood glucose, symptoms precipitated by fasting or exercise, symptoms relieved by glucose.

High plasma insulin levels, increased insulin:glucose.

90% are solitary tumors, may be small and require special imaging.

Only 10% are malignant.

82
Q

Presentation of Glucagonoma

A

Mainly adult females (perimenopausal or postmenopausal).

Associated with glucagonoma synrome: mild glucose intolerance, anemia, skin rash “necrolytic migratory erythema”, weight loss, glossitis/stomatitis

High plasma glucagon levels

Overall 60-70% malignant

Usually malignant if associated with glucagonoma syndrome, otherwise benign

83
Q

Zollinger-Ellison syndrome

A

Gastrinoma, acid hypersecretion, peptic ulcers

Associated with gastrinoma

84
Q

Presentation of Gastrinoma

A

75% located in pancreas, peripancreatic region, 23% in duodenum, and rare in stomach

70-90% are malignant

Usually solitary and malignant if not associated with other endocrine abnormalities

Usually multiple and benign in a component of MEN

85
Q

Normal Parathyroid Gland

A

Four glands (variable)

Mixture of chief cells (pale cytoplasm) and oxyphil cells (eosinophilic cytoplasm)

Variable amounts of adipose tissue (increases with age)

86
Q

Presentation of Parathyroid Adenoma

A

Women > men, 3:1

Usually too small to be palpated

Presentation related to hypercalcemia due to PTH secretion (80% asymptomatic)

Encapsulated, cellular neoplasm

Usually chief cells predominate, other cell types may be present

87
Q

Etiology of Hyperparathyroidism

A
88
Q

Layers of Adrenal Gland

A

Outer yellow cortex and red brown medulla (10:1)

Zona glomerulosa (10-15%): mineralocorticoids

Zona Fasciculata (60-70%): glucocorticoids

Zona Reticularis (20-30%): androgens

89
Q

Etiology of Hypoadrenalism

A

Adrenocortical Insufficiency

Primary: chronic (Addison’s) or acute (Waterhouse-Friderichsen)

Secondary: Pituitary insufficiency or exogenous corticosteroids

90
Q

Etiology of Hypercortisolism

A

Cushing’s Syndrome

Pituitary (Cushing’s Disease): 60-70%

Adrenal: 20-25%

Ectopic: 10-15%

91
Q

Presentation of Cushing’s Syndrome

A
92
Q

Presentation of Conn’s Syndrome

A

Primary hyperaldosteronism due to adrenal cortical adenoma.

HTN, neuromuscular symptoms (weakness, paresthesias), potassium wasting, increased aldosterone with typically decreased renin levels.

Almost invariabley caused by an adrenocortical adenoma.

93
Q

What is Waterhouse-Friderichsen Syndrome?

A

Hemorrhagic destruction of adrenals related to severe bacterial infection: Meningococcus, pneumococci, Staphylococci, Pseudomonas, Haemophilus

94
Q

Presentation of Waterhouse: Friderichsen Syndrome

A

Rapidly progressive hypotension leading to shock

DIC with purpura

Pathology: Bilateral adrenal hemorrhage and necrosis

95
Q

Presentation of Adrenal Tuberculosis

A

Infection of adrenal glands by Mycobacterium tuberculosis

Formely the most comon cause of chronic adrenal insufficiency

Granulomatous inflammation: epitheliod histiocytes, giant cells, and acid-fast bacilli

96
Q

Adrenal Cortical Adenoma gross and microscopic findings

A

Gross: Bright yellow or yellow-brown tumors, usually <5cm and <40-60gm

Microscopic: Usually composed of clear-pale cells resembling adrenal cortical cells, may have pleomorphism of cells, mitotic activity is usually low

97
Q

Diagnosis of Adrenal Cortical Carcinoma

A

May have hemorrhage and necrosis

May appear encapsulated or circumscribed

98
Q

Adrenal Cortical Adenoma

Microscopic findings

Features of malignancy

Median survival

A

Microscopy: May be frankly anaplatic or resemble tumor cells of adrenal cortical adenoma

Malignancy: vascular invasion or metastasis

Survival: ~2 years

99
Q

Pheochromoctyoma Rule of 10

A

10% extra-adrenal (paraganglioma)

10% multiple (sporadic cases)

10% malignant

10% occur in children

10% familial (MEN2A/2B, NF-1, VHL)

100
Q

Pheochromocytoma Clinical Presentation

A

Severe or episodic HTN, palpitations, tachycardia, anxiety, and excessive sweating

101
Q

Pheochromocytoma Diagnosis

A

Elevated urine metanephrines and vanillylmandelic acid (VMA), which are metabolites of cathecholamines

102
Q

Pheochromocytoma Gross and Microscopic findings

Indicator of malignancy

A

Gross: well demarcated, very vascular, pale grey to light brown tumors; tumor turns dark brown in chromium salt solution (Zenker’s solution); “pheochrome” refers to affinity for chromium salts

Microscopy: Large cells with basophilic granular cytoplasm, arranged in small nests separated by capillaries; may have nuclear pleomorphism and mitotic activty

Malignancy: metastatis

103
Q

Neuroblastoma Epi

A

7-10% of all childhood malignancies

2nd most common childhood solid malignancy

80% occur in children < 5yo

104
Q

Neuroblastoma Gross and Microscopic Findings

A

Gross: Large tumors (6-8cm avg.), sof, white, with areas of hemorrhage, necrosis, or calcification

Microscopic: Small blue cell tumor arranged in diffuse sheets (may see Homer Wright rosettes)

90% produce catecholamines (urinary metanephrines/VMA elevated)

105
Q

MEN-1: Wermer’s Syndrome

A

Mutation in MEN-1 gene (11q13)

Parathyroid hyperplasia/adenoma

Pituitary adenoma

Pancreatic (or duodenal) islet cell tumor (symptomatic tumors are commonly gastrinoma or insulinoma)

106
Q

MEN-2A: Sipple’s Syndrome

A

Mutation in RET oncogene (10q11)

Medullary carcinoma of thyroid

Pheochromocytoma of adrenal medulla

Parathyroid hyperplasia

107
Q

MEN-2B: Gorlin’s Syndrome

A

Mutation in RET oncogene

Medullary carcinoma of thyroid

Pheochromocytoma of adrenal gland

Mucosal neuromas (oral cavity, GI tract, etc.)

Marfanoid appearance

108
Q

Multiple Endocrine Neoplasia (MEN) Syndromes

A
109
Q

MEN-1 vs. MEN-2

A
110
Q

RET Protooncogene mutations in MEN type 2

A
111
Q

How can you differentiate between hypoglycemia caused by beta-cell tumors and self-induction by insulin/sulfonylureas?

A

Beta-cell tumors are accompanied by high serum levels of C peptide.

112
Q

What is the Whipple triad?

A
  1. Sx of hypoglycemia are present (esp. confusion, stupor, loss of consciousness)
  2. Hypoglycemia is present, typically glucose less than 50 mg/dL
  3. Attacks are precipitated by fasting or exercise and are promptly relieved by administration of glucose
113
Q

What are the common causes of hypoadrenalism?

A

Autoimmune adrenalitis

Granulomatous infections of the adrenal gland

Anterior Pituitary failure