Flashcards in Adrenal Deck (131)
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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
