SM_179b: Adrenal Pathology: Cortex/Medulla Flashcards
Outside of adrenal gland is ____ and inside is ____
Outside of adrenal gland is cortex and inside is medulla
Describe histology of adrenal gland
Adrenal gland histology
- Capsule (outside)
- Zona glomerulosa
- Zona fasciculata
- Zona reticularis
- Medulla
Zone glomerulosa of adrenal gland produces ____
Zone glomerulosa of adrenal gland produces mineralocorticoids
Zone fasciculata of adrenal gland produces ____
Zone fasciculata of adrenal gland produces glucocorticoids
(largest layer by volume)
Zone reticularis of adrenal gland produces ____
Zone reticularis of adrenal gland produces androgens
Describe adrenal pathology
Adrenal pathology
Atrophic adrenal cortex is ___
Atrophic adrenal cortex is hypofunctional
(hyperplastic is hyperfunctional)
Describe causes of hypofunction of the adrenal cortex
Adrenal cortex hypofunction
- Pituitary insufficiency
- Addison’s disease (chronic adrenal insufficiency): usually autoimmune
- Abrupt withdrawal of steroid therapy (iatrogenic)
- Waterhouse Friderichsen Syndrome
Adrenal cortical hypofunction is caused by ____ and presents as ____, ____, ____, and ____
Adrenal cortical hypofunction is caused by atrophic cortex and presents as fatigue, hypotension, hyperpigmentation, and hyperkalemia
Waterhouse Friderichsen syndrome is ____ with pathological findings of ____ and ____
Waterhouse Friderichsen syndrome is acute adrenal failure (cortex) with pathological findings of hemorrhage and necrosis
- Often caused by meningococcal sepsis, DIC, or severe hypotension
Describe causes of adrenal cortex hyperfunction
Adrenal cortex hyperfunction
- ACTH secreting pituitary adenoma (Cushing’s disease)
- Other ACT secreting tumors (lung carcinoma, etc)
- Primary adrenal cortical hyperplasia
- Cortisol producing adrenal cortical tumors
Increased cortisol levels, central obesity, moon facies, purple striae, and hirsutism is ____
Increased cortisol levels, central obesity, moon facies, purple striae, and hirsutism is Cushing’s syndrome
Types of cortical hyperplasia are ____, ____, and ____
Types of cortical hyperplasia are diffuse hyperplasia, micronodular hyperplasia, and macronodular hyperplasioa
- Diffuse: diffuse symmetric enlargement of the cortex
- Micronodular: multiple nodules present, all < 1.0 cm
- Macronodular: multiple nodules present, some > 1.0 cm
Most adult adrenal masses are ____
Most adult adrenal masses are non-functioning cortical adenoma
- Pheochromocytoma and metastatic carcinoma are next most common
Cushing’s disease is when ____
Cushing’s disease is when pituitary (adenoma or hyperplasia) produces excess ACTH leading to Cushing’s syndrome
- Cushing’s syndrome: increased cortisol levels, central obesity, moon facies, purple striae, and hirsutism
____ is the most common adrenal tumor and is most often non-functional
Adrenal cortical adenoma is the most common adrenal tumor and is most often non-functional
- Often discovered incidentally
Describe functional cortical adenoma
Functional cortical adenoma
- Aldosterone producing adenoma (Conn syndrome)
- Glucocorticoid producing adenoma (Cushing syndrome)
- Androgen producing adenomas associated with feminization or virilization
- Adenomas with mixed hormone production
Describe inherited syndromes associated with adrenal cortical syndrome
Inherited syndromes associated with adrenal cortical syndrome
- Beckwith-Weidemann syndrome
- Multiple endocrine neoplasia type 1 (MEN 1: Wermer syndrome)
- Hyperparathyroidism jaw tumor syndrome
- Li Fraumeni syndrome
- Gardner syndrome
Adrenal ___ is often > 6.5 cm and weights often > 100 g
Adrenal cortical carcinoma is often > 6.5 cm and weights often > 100 g
Describe the criteria for cortical carcinoma
Criteria for cortical carcinoma: need > 3 criteria
- Pink cytoplasm
- Difference pattern
- Necrosis
- High nuclear grade
- > 5 mitosis / 50 HPF
- Atypical mitosis
- Venous invasion
- Capsular invasion
- Extra-adrenal invasion
This is ___
This is adrenal cortical carcinoma
This is ___
This is adrenal cortical carcinoma
Adrenal cortical adenoma is composed of tumor cells similar to ___
Adrenal cortical adenoma is composed of tumor cells similar to adrenal cortex
Common primary tumors of adrenal medulla are ____, ____, and ____
Common primary tumors of adrenal medulla are pheochromocytoma, ganglioneuroma, and neuroblastoma
Adrenal medulla is derived from ____ and is composed of ____, ____, and ____
Adrenal medulla is derived from neural crest and is composed of pheochromocytes, sustenacular cells, and other stromal cells
Adrenal medulla produces ____
Adrenal medulla produces catecholamines
Describe syndromes associated with pheochromocytoma
Syndromes associated with pheochromocytoma
- Multiple endocrine neoplasia 2A (MEN 2A)
- Multiple endocrine neoplasia 2B (MEN 2B)
- Von Hippel Lindau disease
- Von Recklinghausen syndrome (NF-1)
- Sturge-Weber
Pheochromocytoma is a tumor of the ____ that produces ____
Pheochromocytoma is a tumor of the adrenal medulla that produces catecholamines
Pheochromocytoma histology involves ____ and ____
Pheochromocytoma histology involves grossly pink / red / grey color and large tumor cells forming nests (Zellballen)
- Microscopic criteria do not accurately predict the tumor behavior
- Only definitive criteria for malignancy is metastasis
This is ____
This is pheochromocytoma
Describe gross appearance of pheochromocytoma
Pheochromocytoma gross appearance
- Grey, brown, or red in color
- Can be heterogeneous cut surface
- Surrounded by a rim of cortex
Pheochromocytoma histological appearance is ____, ____, and ____
Pheochromocytoma histological appearance is functional, purple cytoplasm, and sustenacular cells present
- Difficult to predict malignancy
Describe gross and histological appearance of adrenal cortical adenoma
Adrenal cortical adenoma gross and histological appearance
- Gross: golden yellow, homogeneous cut surface, contiguous with cortex
- Histological: most non-functional, foamy cytoplasm, sustenacular cells absent, malginancy may occur
These are ____ and ____ in ____
These are zellballen and sustenacular cells in pheochromocytoma
Ganglioneuroma is composed of ___ and ___
Ganglioneuroma is composed of ganglion cells and schwannoma stroma
- Derived from neural crest
- Not hormonally active
Neuroblastoma is ____ that most commonly occurs at the ____
Neuroblastoma is a malignant tumor of neural crest (neuroblastic) derivation that occurs at the adrenal medulla
- Mostly children
Palpable abdominal mass ± abdominal pain is ____
Palpable abdominal mass ± abdominal pain is neuroblastoma
- Catecholamine metabolites (VMA and HMA) elevated in urine
Ganglioneuroma and neuroblastoma exhibit ___
Ganglioneuroma and neuroblastoma exhibit different degrees of neuroblastic differentiation
Other adrenal lesions typically involve ____
Other adrenal lesions typically involve both cortex and medulla
- Benign conditions: cysts, granuloma, myelolipoma
- Malignant conditions: metastatic carcinomas, other malignant tumors
Most common type of adrenal cyst is ___
Most common type of adrenal cyst is vascular / endothelial cyst
Adrenal myelolipoma components are ____ and ____
Adrenal myelolipoma components are myelo (marrow elements) and lipoma (mature adipose)
- Grossly: red (marrow) and yellow (fat)
- Benign tumor of adults
Metastatic tumors in the adrenal glands are commonly ___, often ___, and often arise from ___ or ___
Metastatic tumors in the adrenal glands are commonly carcinoma, often bilateral, and often arise from renal or lung
- Can cause adrenal insufficiency
*
Heterotopic / ectopic adrenal cortical tissue is _____ due to _____
Heterotopic / ectopic adrenal cortical tissue is normal cortical tissue in an abnormal location due to abnormalities in adrenal cortical cell migration during development
- Sites: celiac axis (most common), testis, ovary, intra-renal, hernia sacs
