Adrenal Flashcards Preview

Anatomic pathology > Adrenal > Flashcards

Flashcards in Adrenal Deck (131)
Loading flashcards...
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.