the adrenal glands - columbia university · the adrenal glands diane hamele-bena, m.d. i. normal...
TRANSCRIPT
![Page 1: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/1.jpg)
The Adrenal Glands
Diane Hamele-Bena, M.D.
![Page 2: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/2.jpg)
I. Normal adrenal gland: Gross and microscopic features
II. Hypoadrenalism
III. Hyperadrenalism
IV. Adrenal cortical neoplasms
V. Adrenal medulla
![Page 3: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/3.jpg)
• Normal adult adrenal gland: 3.5 - 4.5 grams
Normal Adrenal Gland
![Page 4: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/4.jpg)
• Cortex: 3 zones:
– Glomerulosa: mineralocorticoids (aldosterone)
– Fasciculata
– Reticularis
Adrenal Cortex Morphology
:glucocorticoids; sex steroids (<)
:androgens
![Page 5: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/5.jpg)
Capsule
lomerulosa
asciculata
eticularis
C
O
R
T
E
X
G
F
R
![Page 6: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/6.jpg)
Hypoadrenalism
![Page 7: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/7.jpg)
Hypoadrenalism
• Primary Adrenocortical Insufficiency
• Secondary Adrenocortical Insufficiency
–Due to primary failure of adrenal glands
–ACTH is elevated
–Due to disorder of hypothalamus or pituitary
–ACTH is decreased
![Page 8: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/8.jpg)
HypoadrenalismClinical Manifestations
•Fatigue, weakness, depression
•Anorexia
•Dizziness
•N&V, diarrhea
•Hyponatremia, hyperkalemia
•Hypoglycemia
•Hyperpigmentation
![Page 9: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/9.jpg)
HypoadrenalismClinical Manifestations
Primary adrenal insufficiency:
Deficiency of
glucocorticoids, mineralocorticoids, and androgens
Hypoglycemia
Fatigue
Anorexia
Weight loss
Hyponatremia
Hyperkalemia
Hypotension
Dizziness
aldosterone
Reduced pubic
and axillary
hair in women
![Page 10: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/10.jpg)
HypoadrenalismClinical Manifestations
Primary adrenal insufficiency:
Concomitant hypersecretion of ACTH
Hyperpigmentation
MSH-like effect
![Page 11: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/11.jpg)
HypoadrenalismClinical Manifestations
Secondary adrenal insufficiency:
Deficiency of ACTH
NO hyperpigmentation
![Page 12: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/12.jpg)
Pathology of Hypoadrenalism
• Primary Adrenocortical Insufficiency
– Acute
– Chronic = Addison Disease
• Secondary Adrenocortical Insufficiency
•Waterhouse-Friderichsen Syndrome
Acute hemorrhagic necrosis, most often due to Meningococci
![Page 13: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/13.jpg)
Meningococci
Waterhouse-Friderichsen Syndrome
•Hypotension
•Purpura
•Cyanosis
Adapted from Netter
Massive adrenal
hemorrhage
![Page 14: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/14.jpg)
Waterhouse-Friderichsen Syndrome
![Page 15: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/15.jpg)
Waterhouse-Friderichsen Syndrome
![Page 16: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/16.jpg)
Pathology of Hypoadrenalism
• Primary Adrenocortical Insufficiency
– Acute
– Chronic = Addison Disease
•Waterhouse-Friderichsen Syndrome
Acute hemorrhagic necrosis, most often due to Meningococci
•Autoimmune adrenalitis
•Infections (e.g., tuberculosis, fungi)
•Metastatic tumors•Other: Amyloidosis, hemochromatosis
![Page 17: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/17.jpg)
Addison DiseaseClinical findings
Mineralocorticoid deficiency Glucocorticoid deficiency
Androgenic deficiency
•Hypotension
•Hyponatremia
•Hyperkalemia
•Loss of pubic and axillary
hair in women
•Weakness and fatigue
•Weight loss
•Hyponatremia
•Hypoglycemia
•Pigmentation
•Abnormal H2O metabolism
•Irritability and mental sluggishness
![Page 18: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/18.jpg)
Autoimmune Adrenalitis
Three settings:
•Autoimmune Polyendocrine Syndrome type 1 (APS1) =
Autoimmune Polyendocrinopathy, Candidiasis, and
Ectodermal Dysplasia (APECED)
•Autoimmune Polyendocrine Syndrome type 2 (APS2)
•Isolated Autoimmune Addison Disease
![Page 19: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/19.jpg)
Addison Disease
Before treatment After treatment
![Page 20: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/20.jpg)
Pathologic Changes in Autoimmune Adrenalitis
•Gross:
–Very small glands (1 - 1.5 grams)
–Cortices markedly thinned
•Micro:
–Diffuse atrophy of all cortical zones
–Lymphoplasmacytic infiltrate
–Medulla is unaffected
![Page 21: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/21.jpg)
Metastatic carcinoma in adrenal
Tumor
![Page 22: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/22.jpg)
Pathology of Hypoadrenalism
• Primary Adrenocortical Insufficiency
– Acute
• Waterhouse-Friderichsen Syndrome
– Chronic = Addison Disease
• Secondary Adrenocortical Insufficiency
– Any disorder of the hypothalamus or pituitary leading
to diminished ACTH; e.g., infection; pituitary tumors,
including metastatic carcinoma; irradiation
![Page 23: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/23.jpg)
Hyperadrenalism
![Page 24: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/24.jpg)
Hyperadrenalism
Three distinctive clinical syndromes:
•Excess cortisol: Cushing Syndrome
•Excess aldosterone: Conn Syndrome
•(Excess androgens: Adrenogenital or Virilizing Syndrome)
![Page 25: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/25.jpg)
Hyperadrenalism
In clinical practice, most cases of
Cushing Syndrome are the result of
administration of exogenous glucocorticoids
(“exogenous” or iatrogenic Cushing Syndrome).
![Page 26: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/26.jpg)
Endogenous
Exogenous
(Iatrogenic)
Cushing Syndrome
Most common
Pituitary adenoma
Cushing Disease
Adrenal neoplasm
ACTH-producing tumor
70-80%
10%
10-20%
Paraneoplastic
Syndrome
![Page 27: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/27.jpg)
“Endogenous” Cushing Syndrome
Etiology Pathology
•Cushing Disease
•Ectopic ACTH production
•Hypersecretion of cortisol by
adrenal neoplasm
I. ACTH-dependent:
II. ACTH-independent:
Pituitary adenoma
Extra-adrenal ACTH-producing tumor
Adrenal neoplasm
Adrenal cortical hyperplasia
Adrenal cortical hyperplasia
![Page 28: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/28.jpg)
Adapted from Netter
Adrenal
carcinoma
Adrenal
adenoma
Pituitary adenoma
Adrenal cortical
hyperplasia
Dorsal fat pad
Ecchymoses
Thin skin
Thin arms & legs
Poor wound
healing
Moon face
Striae
Pendulous
abdomen
C
O
R
T
I
S
O
L
Cushing Syndrome
ACTH-producing tumor
![Page 29: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/29.jpg)
Hydrocortisone Excess•Abnormal fat distribution
–Moon face
–Central obesity
•Increased protein catabolism
–Thin skin
–Easy bruisability
–Striae
–Osteoporosis with
vertebral fractures
–Impaired healing
–Muscle wasting
–Suppressed response to
infection
•Diabetes
•Psychiatric symptoms
•Hirsutism
•Deepened voice in women
•Acne
•Abnormal menses
Cushing Syndrome
•Hypokalemia with alkalosis
•Usually occurs in cases
of ectopic ACTH production
Adrenal Androgen Excess
Mineralocorticoid Excess
![Page 30: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/30.jpg)
Cushing Syndrome
![Page 31: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/31.jpg)
Pituitary adenoma
Cushing Disease
Usually not so large!
![Page 32: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/32.jpg)
Normal
Cortical hyperplasia
Cortex
Adrenal cortical hyperplasia
![Page 33: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/33.jpg)
Adrenal cortical adenoma
Tumor
Adrenal gland
![Page 34: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/34.jpg)
Pathology of Primary Hyperaldosteronism
• Aldosterone-secreting adenoma
• Adrenal cortical carcinoma
– Conn Syndrome
– Uncommon cause of hyperaldosteronism
![Page 35: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/35.jpg)
Conn Syndrome
Adapted from Netter
Adrenal
adenoma
•Hypertension
•Polydipsia
•Polyuria
•Hypernatremia
•Hypokalemia
Aldosterone
![Page 36: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/36.jpg)
Adenomas and Carcinomas
* May produce:
• Cortisol
• Sex steroids
• Aldosterone
Cortical Neoplasms
Functioning *
Non-functioning
(Cushing Syndrome))
(Conn Syndrome))
![Page 37: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/37.jpg)
Cortical Neoplasms
• Discrete, but often unencapsulated
• Small (up to 2.5 cm)
• Most <30 grams
• Yellow-orange, usually without
necrosis or hemorrhage
• Usually unencapsulated
• Large (many >20 cm)
• Frequently > 200-300 grams
• Yellow, with hemorrhagic,
cystic, & necrotic areas
• Adenomas– Gross:
– Micro:
• Carcinomas– Gross:
– Micro:
• Lipid-rich & lipid-poor cells with
little size variation
• Ranges from mild atypia to
wildly anaplastic
![Page 38: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/38.jpg)
Residual adrenal gland
Adrenal cortical adenoma
![Page 39: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/39.jpg)
Adrenal cortical adenoma
![Page 40: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/40.jpg)
Tumor
Kidney
Adrenal cortical carcinoma
![Page 41: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/41.jpg)
Adrenal cortical carcinomaMitosis
![Page 42: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/42.jpg)
Adrenal Medulla
![Page 43: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/43.jpg)
• Specialized neural crest (neuroendocrine) cells
• Part of the chromaffin system, which includes the
adrenal medullae & paraganglia
• Major source of catecholamines (epi, norepi, &
dopamine)
Adrenal Medulla
![Page 44: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/44.jpg)
Adrenal Medulla
![Page 45: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/45.jpg)
• Neuroblastoma
• Ganglioneuroblastoma
• Ganglioneuroma
• Pheochromocytoma
Tumors of the Adrenal Medulla
![Page 46: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/46.jpg)
Ganglioneuroma Ganglioneuroblastoma Neuroblastoma
B
E
N
I
G
N
M
A
L
I
G
N
A
N
T
![Page 47: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/47.jpg)
• Poorly differentiated malignant neoplasm derived
from neural crest cells
• Usually occurs in infants & small children
• “Small round blue cell tumor” of childhood
Neuroblastoma
Rhabdomyosarcoma Lymphoma
Retinoblastoma Wilms tumor
Ewing sarcoma/PNET Medulloblastoma
![Page 48: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/48.jpg)
• Gross:
– Large tumor with hemorrhage, necrosis, &
calcification
Neuroblastoma: Pathology
• Micro:
– Undifferentiated small cells resembling lymphocytes
(“Small, round, blue cell tumor”)
– May show areas of differentiation (larger cells with
more cytoplasm and Schwannian stroma)
![Page 49: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/49.jpg)
Neuroblastoma
![Page 50: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/50.jpg)
Neuroblastoma
![Page 51: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/51.jpg)
• Patient age
• Stage
• Site of 10 involvement
• Histologic grade
• DNA ploidy
• N-myc oncogene amplification
• Others: Chromosome 17q gain, Chromosome 1p loss, Trk-A
expression, Telomerase expression, MRP expression, CD44
expression
Neuroblastoma: Prognostic Factors
![Page 52: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/52.jpg)
• Differentiated neoplasm of neural crest origin
• Benign
• Occurs in older age group
• Pathology:
– Gross: Encapsulated, white, firm
– Micro: Ganglion cells & Schwann cells
Ganglioneuroma
![Page 53: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/53.jpg)
Ganglioneuroma
![Page 54: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/54.jpg)
• Composed of malignant neuroblastic elements &
ganglioneuromatous elements
• Prognosis depends on % of neuroblasts
Ganglioneuroblastoma
![Page 55: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/55.jpg)
Ganglioneuroblastoma
![Page 56: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/56.jpg)
Pheochromocytoma
• Rare, but important: surgically curable form of
hypertension
• May arise in association with familial syndromes, e.g.,
MEN2, von Hippel-Lindau, von Recklinghausen (NF1)
• May be “sporadic”: ~24% have germline mutations,
including mutations of RET, VHL, SDH-B, and SDH-D genes
• Extra-adrenal tumors (e.g., carotid body) are called
“paragangliomas”
• Catecholamine-secreting neoplasm: HYPERTENSION
![Page 57: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/57.jpg)
• Gross:
– 1 - 4000 grams (average = 100 grams)
– Areas of hemorrhage, necrosis, & cystic degeneration
Pheochromocytoma: Pathology
• Micro:
– Balls of cells resembling cells of medulla, with bizarre,
hyperchromatic nuclei; richly vascular stroma
• Benign & malignant tumors are histologically identical;
the only absolute criterion for malignancy is metastasis.
![Page 58: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/58.jpg)
Residual adrenal
Tumor
Pheochromocytoma
![Page 59: The Adrenal Glands - Columbia University · The Adrenal Glands Diane Hamele-Bena, M.D. I. Normal adrenal gland: Gross and microscopic features II. Hypoadrenalism III. Hyperadrenalism](https://reader030.vdocuments.us/reader030/viewer/2022021622/5b9ad10409d3f21b2f8c2861/html5/thumbnails/59.jpg)
Pheochromocytoma
Benign or malignant???
Benign & malignant tumors are histologically identical;
the only absolute criterion for malignancy is metastasis.