Flashcards in Adrenal Deck (131)
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Spoardic adrenal cortical adenoma associated wth Conn's syndrome: Genes (3).
KCNJ5: Potassium channel.
ATP1A1, ATP2B3: Na/K-ATPases.
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Adrenal cortical adenoma: Most common hormonal excess.
None: Most adenomas are nonfunctioning.
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Adrenal cortical adenomas: Presentations of the functioning types (4).
Cushing's syndrome.
Conn's syndrome.
Virilization (rare).
Feminization (strongly suggests adenocarcinoma).
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Adrenal cortical adenomas: Possible colors (3).
Golden yellow: Conn's syndrome.
Mahogany: Oncocytic.
Black: Pigmented (lipofuscin).
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Adrenal cortical adenoma associated with Conn's syndrome: Histopathology (2).
Clear, lipid-rich cytoplasm.
Spironolactone bodies (if spironolactone has been given).
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Adrenal cortical adenoma: Histopathology of non-adenomatous tissue (2).
Cushing's syndrome: Atrophy of zona reticularis.
Conn's syndrome: Paradoxical hyperplasia of zona glomerulosa.
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Electron microscopy of adrenal cortical adenomas: General (3).
Much lipid.
Much smooth endoplasmic reticulum.
Many mitochondria.
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Electron microscopy of adrenal cortical adenomas: Mitochondria (2).
Aldosterone-producing cells: Lamellar cristae.
Steroid-producing cells: Tubulovesicular cristae.
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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.
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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.
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Adrenal cortical carcinoma: Genes mutated in sporadic tumors (6).
TP53.
β-Catenin.
Menin.
PRKAR1A.
IGF-II.
MC2-R.
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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.
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Adrenal cortical carcinoma: Treatment (2).
Complete resection if possible; otherwise, mitotane.
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Adrenal cortical carcinoma: Typical size and weight.
14-15 cm; 100-1000 g.
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Adrenal cortical carcinoma: Features diagnostic for malignancy (3).
Weight greater than 100 g.
Vascular invasion.
Metastasis.
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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.
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Adrenal cortical carcinoma: Prognostic immunohistochemistry (2).
Ki-67.
Cyclin E: Positive staining implies advanced stage.
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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.
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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.
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MEN 2a and MEN 2b: Gene and its location.
RET on chromosome 10q11.2.
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MEN 2b:
A. Inheritance.
B. Components.
A. Autosomal dominant.
B. Same as those of MEN 2a, plus mucosal neuromas.
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Adrenal medullary hyperplasia: Gross pathology (3).
Usually bilateral.
Nodular or diffuse.
By definition, nodules are less than 1 cm.
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Adrenal medullary hyperplasia: Histopathology (2).
Cells may be enlarged or pleomorphic; may show increased mitotic activity.
Hyperplasia is histopathologically indistinguishable from pheochromocytoma.
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Adrenal medullary hyperplasia: Special histopathologic feature.
Hyaline globules in MEN 2 syndromes.
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