SM_179b: Adrenal Pathology: Cortex/Medulla

Question 1

Outside of adrenal gland is ____ and inside is ____

Answer 1

Outside of adrenal gland is cortex and inside is medulla

Question 2

Describe histology of adrenal gland

Answer 2

Adrenal gland histology

1. Capsule (outside)
2. Zona glomerulosa
3. Zona fasciculata
4. Zona reticularis
5. Medulla

Question 3

Zone glomerulosa of adrenal gland produces ____

Answer 3

Zone glomerulosa of adrenal gland produces mineralocorticoids

Question 4

Zone fasciculata of adrenal gland produces ____

Answer 4

Zone fasciculata of adrenal gland produces glucocorticoids

(largest layer by volume)

Question 5

Zone reticularis of adrenal gland produces ____

Answer 5

Zone reticularis of adrenal gland produces androgens

Question 6

Describe adrenal pathology

Answer 6

Adrenal pathology

Question 7

Atrophic adrenal cortex is ___

Answer 7

Atrophic adrenal cortex is hypofunctional

(hyperplastic is hyperfunctional)

Question 8

Describe causes of hypofunction of the adrenal cortex

Answer 8

Adrenal cortex hypofunction

• Pituitary insufficiency
• Addison’s disease (chronic adrenal insufficiency): usually autoimmune
• Abrupt withdrawal of steroid therapy (iatrogenic)
• Waterhouse Friderichsen Syndrome

Question 9

Adrenal cortical hypofunction is caused by ____ and presents as ____, ____, ____, and ____

Answer 9

Adrenal cortical hypofunction is caused by atrophic cortex and presents as fatigue, hypotension, hyperpigmentation, and hyperkalemia

Question 10

Waterhouse Friderichsen syndrome is ____ with pathological findings of ____ and ____

Answer 10

Waterhouse Friderichsen syndrome is acute adrenal failure (cortex) with pathological findings of hemorrhage and necrosis

• Often caused by meningococcal sepsis, DIC, or severe hypotension

Question 11

Describe causes of adrenal cortex hyperfunction

Answer 11

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

Question 12

Increased cortisol levels, central obesity, moon facies, purple striae, and hirsutism is ____

Answer 12

Increased cortisol levels, central obesity, moon facies, purple striae, and hirsutism is Cushing’s syndrome

Question 13

Types of cortical hyperplasia are ____, ____, and ____

Answer 13

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

Question 14

Most adult adrenal masses are ____

Answer 14

Most adult adrenal masses are non-functioning cortical adenoma

• Pheochromocytoma and metastatic carcinoma are next most common

Question 15

Cushing’s disease is when ____

Answer 15

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

Question 16

____ is the most common adrenal tumor and is most often non-functional

Answer 16

Adrenal cortical adenoma is the most common adrenal tumor and is most often non-functional

• Often discovered incidentally

Question 17

Describe functional cortical adenoma

Answer 17

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

Question 18

Describe inherited syndromes associated with adrenal cortical syndrome

Answer 18

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

Question 19

Adrenal ___ is often > 6.5 cm and weights often > 100 g

Answer 19

Adrenal cortical carcinoma is often > 6.5 cm and weights often > 100 g

Question 20

Describe the criteria for cortical carcinoma

Answer 20

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

Question 21

This is ___

Answer 21

This is adrenal cortical carcinoma

Question 22

This is ___

Answer 22

This is adrenal cortical carcinoma

Question 23

Adrenal cortical adenoma is composed of tumor cells similar to ___

Answer 23

Adrenal cortical adenoma is composed of tumor cells similar to adrenal cortex

Question 24

Common primary tumors of adrenal medulla are ____, ____, and ____

Answer 24

Common primary tumors of adrenal medulla are pheochromocytoma, ganglioneuroma, and neuroblastoma

Question 25

Adrenal medulla is derived from ____ and is composed of \_\_\_\_, \_\_\_\_, and \_\_\_\_

Answer 25

Adrenal medulla is derived from neural crest and is composed of pheochromocytes, sustenacular cells, and other stromal cells

Question 27

Adrenal medulla produces \_\_\_\_

Answer 27

Adrenal medulla produces catecholamines

Question 28

Describe syndromes associated with pheochromocytoma

Answer 28

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

Question 29

Pheochromocytoma is a tumor of the ____ that produces \_\_\_\_

Answer 29

Pheochromocytoma is a tumor of the adrenal medulla that produces catecholamines

Question 30

Pheochromocytoma histology involves ____ and \_\_\_\_

Answer 30

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

Question 31

This is \_\_\_\_

Answer 31

This is pheochromocytoma

Question 32

Describe gross appearance of pheochromocytoma

Answer 32

Pheochromocytoma gross appearance * Grey, brown, or red in color * Can be heterogeneous cut surface * Surrounded by a rim of cortex

Question 33

Pheochromocytoma histological appearance is \_\_\_\_, \_\_\_\_, and \_\_\_\_

Answer 33

Pheochromocytoma histological appearance is functional, purple cytoplasm, and sustenacular cells present * Difficult to predict malignancy

Question 34

Describe gross and histological appearance of adrenal cortical adenoma

Answer 34

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

Question 35

These are ____ and ____ in \_\_\_\_

Answer 35

These are zellballen and sustenacular cells in pheochromocytoma

Question 36

Ganglioneuroma is composed of ___ and \_\_\_

Answer 36

Ganglioneuroma is composed of ganglion cells and schwannoma stroma * Derived from neural crest * Not hormonally active

Question 37

Neuroblastoma is ____ that most commonly occurs at the \_\_\_\_

Answer 37

Neuroblastoma is a malignant tumor of neural crest (neuroblastic) derivation that occurs at the adrenal medulla * Mostly children

Question 38

Palpable abdominal mass ± abdominal pain is \_\_\_\_

Answer 38

Palpable abdominal mass ± abdominal pain is neuroblastoma * Catecholamine metabolites (VMA and HMA) elevated in urine

Question 39

Ganglioneuroma and neuroblastoma exhibit \_\_\_

Answer 39

Ganglioneuroma and neuroblastoma exhibit different degrees of neuroblastic differentiation

Question 40

Other adrenal lesions typically involve \_\_\_\_

Answer 40

Other adrenal lesions typically involve both cortex and medulla * Benign conditions: cysts, granuloma, myelolipoma * Malignant conditions: metastatic carcinomas, other malignant tumors

Question 41

Most common type of adrenal cyst is \_\_\_

Answer 41

Most common type of adrenal cyst is vascular / endothelial cyst

Question 42

Adrenal myelolipoma components are ____ and \_\_\_\_

Answer 42

Adrenal myelolipoma components are myelo (marrow elements) and lipoma (mature adipose) * Grossly: red (marrow) and yellow (fat) * Benign tumor of adults

Question 43

Metastatic tumors in the adrenal glands are commonly \_\_\_, often \_\_\_, and often arise from ___ or \_\_\_

Answer 43

Metastatic tumors in the adrenal glands are commonly carcinoma, often bilateral, and often arise from renal or lung * Can cause adrenal insufficiency *

Question 44

Heterotopic / ectopic adrenal cortical tissue is _____ due to \_\_\_\_\_

Answer 44

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