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Anatomic pathology > Adrenal > Flashcards

Flashcards in Adrenal Deck (131):
1

Deficiency of 21-hydroxylase: Laboratory finding.

Elevated 17-hydroxyprogesterone.

2

Congenital lipoid adrenal hyperplasia: Definition.

Severest form of congenital adrenal hyperplasia: All gonadal and adrenal cortical steroids are markedly underproduced.

3

Congenital lipoid adrenal hyperplasia: Genes (2).

StAR (steroidogenic acute regulatory) protein.

P-450scc.

4

Classic presentation of congenital adrenal hyperplasia in females:

A. Newborn.
B. Older (3).

A. Virilization.

B. Postpubertal: Oligomenorrhea, hirsutism, acne.

5

Classic presentation of congenital adrenal hyperplasia in males:

A. Newborn.
B. Older (3).

A. Salt-losing crisis.

B. Prepubertal: Precocious puberty.

6

Non-classic presentation of congenital adrenal hyperplasia (3).

Newborns: Normal.

Around puberty: Virilization.

Pregnancy: May present with adrenal insufficiency.

7

Congenital adrenal hyperplasia: Histopathology (3).

All three cortical layers are thickened, but especially the zona reticularis.

Loss of zonation.

Lipid-poor cells.

8

Congenital lipoid adrenal hyperplasia: Histopathology.

Cholesterol-overloaded cells with rupture and cholesterol esters.

9

Congenital adrenal hyperplasia: Complication.

Adrenal cortical adenoma or carcinoma.

10

Congenital adrenal hyperplasia: Effect on adrenal medulla.

Glucocorticoid deficiency can impair development of the medulla, resulting in epinephrine deficiency and hypoglycemia.

11

Autoimmune adrenal insufficiency:

A. Frequency.
B. Antibodies (3).

A. Accounts for 75-90% of cases of primary adrenal insufficiency.

B. 21-hydroxylase, 17-hydroxylase, P-450scc.

12

Primary vs. secondary adrenal insufficiency: Biochemical differences (3).

Primary: Low ACTH, low aldosterone, high renin.

Secondary: High ACTH, normal aldosterone, normal renin.

13

Primary vs. secondary adrenal insufficiency: Gross-pathology difference.

Primary: Small, pale adrenal gland.

Secondary: Enlarged gland.

14

Adrenal insufficiency: How much adrenal tissue is essential to normal function?

About 10%.

15

Autoimmune polyglandular syndromes.

APS-1: Includes candidiasis and alopecia; mutation in AIRE-1.

APS-2: Schmidt's syndrome.

15

Primary adrenal cortical hyperplasia: Types (3).

ACTH-independent macronodular hyperplasia.

Primary pigmented nodular adrenal cortical disease.

Other.

16

ACTH-independent macronodular hyperplasia: Syndrome that can cause it, and its gene.

McCune-Albright syndrome, GNAS1.

17

Primary pigmented nodular adrenal cortical disease: Syndromes (2).

Carney's complex.

Isolated primary pigmented nodular adrenal cortical disease.

18

Primary pigmented nodular adrenal cortical disease: Genes (2).

PRKAR1A.

PDE11A.

19

Other syndromes (2) that can cause bilateral adrenal hyperplasia.

MEN-1.

FAP.

20

Secondary adrenal cortical hyperplasia: Causes (2).

Pituitary adenoma or hyperplasia.

Ectopic ACTH.

21

Adrenal cortical hyperplasia: Presentation.

Primary: Various endocrine abnormalities.

Secondary due to pituitary: Severe, typical Cushing's syndrome.

Secondary due to ectopic ACTH: Severe, atypical Cushing's syndrome.



22

Adrenal cortical hyperplasia: Pharmacological therapy.

Ketoconazole and similar drugs.

23

Adrenal cortical hyperplasia: Degree of enlargement (3).

Severe: Ectopic ACTH, AIMAH.

Mild or moderate: Pituitary disease, PPNAD.

Grossly inapparent: Conn's syndrome due to hyperplasia of zona glomerulosa.

Enlargement may be nodular or diffuse.

24

Hyperplasia of zona glomerulosa: Histopathology (3).

Involvement limited to the periphery of the gland.

Abnormal continuity of the zona.

More than 5 nests thick.

25

ACTH-independent macronodular adrenal cortical hyperplasia: Histopathology (2).

Mixture of large clear cells and small compact cells.

Nodules of cells containing dark brown pigment.

26

Primary pigmented nodular adrenal cortical disease: Histopathology.

Cortical tissue between nodules is atrophic and disorganized.

27

Adrenal cortical hyperplasia: Immunohistochemistry.

AIMAH: 3β-hydroxysteroid dehydrogenase.

PPNAD: Synaptophysin, 17α-hydroxylase.

28

Clinical findings that favor adrenal adenoma over adrenal hyperplasia (2).

Solitary, unilateral nodule.

Evidence of autonomous growth.

29

Adrenal cortical adenoma: Familial syndromes (3).

MEN-1.

Familial hyperaldosteronism.

Congenital adrenal hyperplasia.

30

Spoardic adrenal cortical adenoma associated wth Conn's syndrome: Genes (3).

KCNJ5: Potassium channel.

ATP1A1, ATP2B3: Na/K-ATPases.

31

Adrenal cortical adenoma: Most common hormonal excess.

None: Most adenomas are nonfunctioning.

32

Adrenal cortical adenomas: Presentations of the functioning types (4).

Cushing's syndrome.

Conn's syndrome.

Virilization (rare).

Feminization (strongly suggests adenocarcinoma).

33

Adrenal cortical adenomas: Possible colors (3).

Golden yellow: Conn's syndrome.

Mahogany: Oncocytic.

Black: Pigmented (lipofuscin).

34

Adrenal cortical adenoma associated with Conn's syndrome: Histopathology (2).

Clear, lipid-rich cytoplasm.

Spironolactone bodies (if spironolactone has been given).

35

Adrenal cortical adenoma: Histopathology of non-adenomatous tissue (2).

Cushing's syndrome: Atrophy of zona reticularis.

Conn's syndrome: Paradoxical hyperplasia of zona glomerulosa.

36

Electron microscopy of adrenal cortical adenomas: General (3).

Much lipid.

Much smooth endoplasmic reticulum.

Many mitochondria.

37

Electron microscopy of adrenal cortical adenomas: Mitochondria (2).

Aldosterone-producing cells: Lamellar cristae.

Steroid-producing cells: Tubulovesicular cristae.

38

Epithelioid angiomyolipoma: Biological behavior.

Can be malignant.

39

Epithelioid angiomyolipoma: Histopathology (2).

Polygonal epithelioid cells with much cytoplasm and sometimes with a large nucleolus, forming nests or sheets.

Some cells may be multinucleate or bizarre.

40

Epithelioid angiomyolipoma:

A. Immunohistochemistry.
B. Electron microscopy.

A. Cells express melanocytic and myoid markers.

B. Melanosomes and pre-melanosomes.

41

Adrenal cortical carcinoma: Hereditary syndromes (5).

Li-Fraumeni.

Beckwith-Wiedemann.

MEN-1.

Carney's complex.

Hereditary isolated glucocorticoid deficiency.

42

Adrenal cortical carcinoma: Genes mutated in sporadic tumors (6).

TP53.

β-Catenin.

Menin.

PRKAR1A.

IGF-II.

MC2-R.

43

Adrenal cortical carcinoma:

A. How many are functional?
B. What is the most common function?

A. About 79%.

B. Virilization due to secretion of 17-ketosteroids and DHEA.

44

Adrenal cortical carcinoma: Treatment (2).

Complete resection if possible; otherwise, mitotane.

45

Adrenal cortical carcinoma: Typical size and weight.

14-15 cm; 100-1000 g.

46

Adrenal cortical carcinoma: Features diagnostic for malignancy (3).

Weight greater than 100 g.

Vascular invasion.

Metastasis.

47

ACTH-independent macronodular hyperplasia: Other associated genes.

ACTH receptor.

GIP, β-adrenergic receptor, LH receptor.

48

Adrenal cortical carcinoma: Diagnostic immunohistochemistry (4,2,2).

Positive: Inhibin-α, steroidogenic factor-1, Melan-A, D11.

Negative: β-Catenin (aberrant loss), chromogranin,

Variable: Cytokeratins, synaptophysin.

49

Adrenal cortical carcinoma: Prognostic immunohistochemistry (2).

Ki-67.

Cyclin E: Positive staining implies advanced stage.

50

Vascular invasion by adrenal cortical carcinoma:

A. Definition.
B. Sites of metastasis.

A. Thrombus must accompany tumor cells.

B. Liver, lung, lymph nodes.

51

Adrenal medullary hyperplasia: Associations (4).

MEN 2a, MEN 2b.

Beckwith-Wiedemann syndrome.

Cystic fibrosis.

Not: VHL syndrome, neurofibromatosis.

52

Adrenal cortical carcinoma: Histopathologic features suggestive of malignancy (5).

Necrosis.

Cellular atypia.

Increased mitotic activity.

Invasion beyond the adrenal gland.

Broad fibrous bands.

53

MEN 2a:

A. Synonym.
B. Inheritance.
C. Components.

A. Sipple's syndrome.

B. Autosomal dominant.

C. Pituitary hyperplasia, medullary carcinoma of the thyroid, pheochromocytoma.

54

MEN 2a and MEN 2b: Gene and its location.

RET on chromosome 10q11.2.

55

MEN 2b:

A. Inheritance.
B. Components.

A. Autosomal dominant.

B. Same as those of MEN 2a, plus mucosal neuromas.

56

Adrenal medullary hyperplasia: Gross pathology (3).

Usually bilateral.

Nodular or diffuse.

By definition, nodules are less than 1 cm.

57

Adrenal medullary hyperplasia: Histopathology (2).

Cells may be enlarged or pleomorphic; may show increased mitotic activity.

Hyperplasia is histopathologically indistinguishable from pheochromocytoma.

58

Adrenal medullary hyperplasia: Special histopathologic feature.

Hyaline globules in MEN 2 syndromes.

59

Pheochromocytoma: How many are hereditary?

Almost half.

60

Pheochromocytoma: Genes (7).

VHL.

RET.

NF.

SDHA, SDHB, SDHC, SDHC.

61

Pheochromocytoma: How many are asymptomatic?

Up to 25%.

62

Pheochromocytoma: Instigators of symptoms (4).

Anesthesia.

Manipulation of the tumor.

Certain foods, drugs.

63

Pheochromocytoma: Diagnostic tests (4).

Urinary and plasma catecholamines.

Urinary metanephrines.

Urinary vanillylmandelic acid.

64

Pheochromocytoma: Histologic architecture.

Zellballen surrounded by sustentacular cells.

65

Pheochromocytoma: Cytology of tumor cells (3).

Granular cytoplasm.

Large nucleoli.

Intranuclear cytoplasmic inclusions.

66

Composite pheochromocytoma.

Also contains areas resembling neuroblastoma, ganglioneuroblastoma, or typical ganglioneuroma.

67

Pheochromocytoma: More specific immunohistochemical stains (2).

Tyrosine hydroxylase.

SDHB: Expression is lost in any tumor exhibiting a mutation of any of the genes for succinate dehydrogenase.

68

Pheochromocytoma: Significance of SDHB (2).

Usually mutated in extra-adrenal paraganglioma rather than in pheochromocytoma.

Mutation in pheochromocytoma suggests malignancy.

69

Pheochromocytoma: Electron microscopy.

Neurosecretory granules.

70

Pheochromocytoma: How to recognize a malignant one.

Only by the demonstration of distant metastases.

Histology does not help, even when there are bizarre tumor giant cells.

71

Ganglioneuroma: Origin (2).

De novo.

Maturation of neuroblastoma or ganglioneuroblastoma.

72

Ganglioneuroma: Possible laboratory findings (4).

Elevated HVA, VMA, VIP, and/or serotonin.

73

Ganglioneuroma: Cellular components (3).

Ganglion cells.

Schwann cells.

Mature fibroblasts.

74

Ganglioneuroma: Possible source of difficulty in histologic diagnosis.

Scarcity of ganglion cells can cause confusion with neurofibroma.

75

Ganglioneuroma: Immunohistochemistry (3).

Positive: S-100, synaptophysin, neurofilament.

76

Ganglioneuroma: Electron microscopy of ganglion cells (2).

Peripheral rough endoplasmic reticulum.

Neurosecretory granules.

77

Ganglioneuroma: Clinical behavior.

Benign, unless it undergoes transformation to an MPNST.

78

Ganglioneuroblastoma: Epidemiology.

Occurs mainly in toddlers.

79

Ganglioneuroblastoma: Most common site.

Abdomen.

(Ganglioneuroma: Posterior mediastinum.)

80

Ganglioneuroblastoma: Components.

Ganglioneuromatous component: Usually more than 50%.

Neuroblastomatous component.

81

Ganglioneuroblastoma: Subtypes (3).

Nodular classic.

Nodular atypical.

Intermixed.

82

Ganglioneuroblastoma: Nodular classic subtype.

Sharp demarcation between neuroblastomatous nodule and surrounding ganglioneuromatous component.

83

Ganglioneuroblastoma: Nodular atypical subtype (3).

No gross or microscopic nodules.

Ganglioneuromatous component forms a thin rim.

Metastases resemble neuroblastoma.

84

Ganglioneuroblastoma: Intermixed subtype (2).

No gross or microscopic nodules.

Microscopic foci of neuroblastomatous component.

85

Ganglioneuroblastoma: Prognostic difference among subtypes.

Intermixed: Better.

Nodular: Worse.

86

Ganglioneuroblastoma: Biochemical difference among subtypes.

Nodular subtype secretes more catecholamines.

87

Neuroblastoma: Most common sites (2).

Adrenal gland.

Posterior mediastinum.

88

Neuroblastoma: Classic signs of metastasis (3).

Periorbital ecchymoses.

Proptosis.

"Blueberry-muffin" skin.

89

Neuroblastoma: Paraneoplastic syndromes (2).

Intractable diarrhea due to secretion of VIP.

Opsoclonus-myoclonus syndrome.

90

Neuroblastoma: Histologic architecture.

Cells are arranged in vague lobules and may form Homer Wright rosettes (filled with pink fibrillary matter).

91

Pheochromocytoma: Prognosis.

Five-year survival rate

− Benign: 95%.
− Malignant: 44%.

92

Neuroblastoma: Subtypes.

Undifferentiated.

Undifferentiated and pleomorphic.

Poorly differentiated.

Differentiating.

93

Neuroblastoma: Undifferentiated (2).

Neuroblasts show no differentiation toward ganglion cells.

No neuropil.

94

Neuroblastoma: Undifferentiated and pleomorphic.

Same as undifferentiated neuroblastoma, except that neuroblasts are larger and more pleomorphic and have more cytoplasm.

95

Neuroblastoma: Poorly differentiated.

Fewer than 5% of neuroblasts show differentiation toward ganglion cells.

96

Neuroblastoma: Differentiating.

More than 5% of neuroblasts show differentiation toward ganglion cells.

97

Mitosis-karyorrhexis index: Number of cells to be counted.

5000.

98

Mitosis-karyorrhexis index: Ranges.

Low: Less than 2%.

Intermediate: 2-4%.

High: More than 4%.

99

Neuroblastoma: Mutation that confers poor outcome.

Amplification of MYCN (more than 10 copies).

100

Prognosis of neuroblastoma: Patient over 5 years of age.

Any neuroblastoma is considered to have unfavorable histology.

101

Prognosis of neuroblastoma: Patient between 18 months and 5 years of age, inclusive (3).

Undifferentiated or poorly differentiated: Unfavorable histology.

Differentiating: Favorable as long as the MKI is low (i.e. not intermediate or high).

102

Prognosis of neuroblastoma: Patient under 18 months of age (3).

Undifferentiated: Unfavorable histology.

Poorly differentiated or differentiating: Favorable as long as the MKI is low or intermediate.

Any tumor with a high MKI: Unfavorable.

103

Prognosis of neuroblastoma: DNA index.

A. Use.
B. Interpretation.

A. Used in patients under 1 year of age.

B. Hyperdiploidy or near-triploidy is better than near-diploidy or near-tetraploidy.

104

Prognosis of neuroblastoma: Expression of TRK.

TRK A, B, and C: Low or absent expression imparts poorer prognosis.

105

Neuroblastoma: Extracellular components (2).

Fibrillar matrix resembling neuropil.

Delicate fibrovascular septa separating the lobules.

106

Neuroblastoma: Definition of stage 4S.

Small, localized primary tumor with metastases to liver, skin, or bone marrow that nearly always spontaneously regress.

107

Neuroblastoma: Immunohistochemistry of tumor cells (5,2).

Positive: NSE, synaptophysin, chromogranin, neurofilament, Neu-N.

Negative: Cytokeratins and other non-neural markers.

108

Neuroblastoma: Associated hereditary syndromes (4).

Hirschsprung's disease.

Congenital central hypoventilation.

Neurofibromatosis-1.

109

Neuroblastoma: Electron microscopy (3).

Cytoplasmic filaments.

Dense-core granules.

Microtubules.

110

Primary melanoma of the adrenal gland: Criteria (4).

Unilateral.

No previous melanoma.

No endocrine disorder.

No doubt about histology.

111

Primary melanoma of the adrenal gland: Gross pathology.

Often locally advanced at presentation, with renal adhesions.

112

Primary melanoma of the adrenal gland: Prognosis.

Death within 2 years.

113

Primary melanoma of the adrenal gland vs. pigmented adrenal cortical adenoma: Immunohistochemistry (3).

Both are positive for Melan-A.

Adenoma is negative for S100, HMB-45.

114

Primary melanoma of the adrenal gland vs. pheochromocytoma: Immunohistochemistry.

Both are positive for HMB-45.

The sustentacular cells of pheochromocytoma are positive for S-100.

115

Myelolipoma: Age group.

Middle-aged.

116

Myelolipoma: Presentation (2).

Usually asymptomatic.

Rarely causes Cushing's syndrome or Conn's syndrome.

117

Myelolipoma:

A. Gross pathology.
B. Histopathology.

A. Red and yellow cut surface.

B. Mature bone marrow and mature fat.

118

Myelolipoma: Variant.

Adenolipoma combines myelolipoma and adrenal cortical adenoma.

119

Myelolipoma: Extraadrenal sites (3).

Liver.

Retroperitoneum.

Stomach.

120

Adrenal cyst: Size and structure.

Usually small and unilocular.

121

Adrenal cyst: Types (4).

Endothelial (most common).

Pseudocyst.

Epithelial.

Parasitic.

122

Endothelial adrenal cyst: Types (3).

Lymphangioma.
Angioma.
Hamartoma.

123

Adrenal pseudocyst:

A. Etiology.
B. Histopathology.

A. Previous hemorrhage.

B. Fibrous wall; no epithelial or endothelial lining.

124

Epithelial adrenal cyst: Types (3).

Adenoma.
Glandular cyst or retention cyst.
Embryonal.

125

Parasitic adrenal cyst:

A. Etiology.
B. Histopathology.

A. Parasites, esp. Echinococcus.

B. May contain fat or bone marrow.

126

Adrenal cysts: Radiography (2).

Pseudocyst: Mural calcification.

Endothelial cyst: Septal calcification.

127

Metastasis to the adrenal gland: Most frequent primary sites (4).

Lung.

Stomach.

Esophagus.

Liver.

128

Metastasis to the adrenal gland: Gross pathology (2).

Usually multifocal but can be solitary.

Usually involve the cortex.

129

Metastasis to the adrenal gland: Renal-cell carcinoma.

May mimic adrenal cortex histologically but does not express inhibin A or SF-1.

130

Metastasis to the adrenal gland: Hepatocellular carcinoma (2).

May mimic adrenal cortex histologically but can show bile staining.

Positive for HepPar-1, canalicular polyclonal CEA, canalicular CD10.