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Flashcards in Adrenal Deck (131)
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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.