Endocrine Pathology Test Preparation

Endocrine Pathology
Study Questions
2012
Control – L full screen

Notes
• 2 Study Files for Examination

– Anatomy / Histology Review = RANDOM
– Pathology Study Questions : Current File
• Contain Anatomy and Histology questions at the
A
beginning of the appropriate section
01 • Followed by pathology test questions
Pituitary
A
Macroscopic Challenge:
The organ shown in the image (cross-sectioned) is a
A. Parathyroid
B. Thyroid
C. Pituitary
D. Adrenal
A ANSWER
A. Parathyroid - Posterior Lobe

- Neurohypophysis
B. Thyroid - Pars Nervosa
C. Pituitary
D. Adrenal - Intermediate Lobe
- Pars Interomedialis
- Anterior Lobe
- Adenohypophysis
- Pars Distalis
B
Microscopic Challenge:
A. Parathyroid
B. Thyroid
C. Pituitary
D. Adrenal
B ANSWER
A. Parathyroid
B. Thyroid
C. Pituitary
D. Adrenal
Anterior: Nests of neuroendocrine cells;

surrounded by fibrovascular cores (reticulin stain)
- Acidophilic (red) cytoplasm: Prolactin, GH
- Basophilic (blue) cytoplasm: FSH / LH / TSH /
ACTH
- Chromophobic cytoplasm: ACTH
C
A. Parathyroid
B. Thyroid
C. Pituitary
D. Adrenal
C ANSWER
Pituicytes / Astrocytes = support cells = Spindle-
shaped nucleus
A. Parathyroid
B. Thyroid
C. Pituitary
D. Adrenal
Posterior:
- Axons = descending from hypothalamic nuclei
- Axons highlighted by neurofilament immunohistochemistry
- Pituicytes / Astrocytes = support cells
- Spindled nuclei are “pituicytes”
(DeMasters Lecture)
C ANSWER
Normal Pituitary
Posterior Pituitary
(Neurohypophysis)
(Dr. Kerr)
Anterior: Nests of neuroendocrine cells;

surrounded by fibrovascular cores (reticulin stain)
- Acidophilic (red) cytoplasm: Prolactin, GH
- Basophilic (blue) cytoplasm: FSH / LH / TSH / ACTH
- Chromophobic cytoplasm: ACTH
Intermediate Anterior Pituitary

Region = Rathke (Hypophysis)
pouch remnants
Posterior:
- Axons = descending from hypothalamic nuclei
- Pituicytes / Astrocytes = support cells
01
Question
A 32-year-old female patient is noted to have
mild galactorrhea and a mild elevation of her
serum prolactin level. MRI of the pituitary
identifies a large sellar and suprasellar mass.
Each of the following is a reasonable
explanation for these clinical findings
EXCEPT
A. Mass is a secretory / functional prolactinoma
B. Neoplasm is a null cell adenoma and
galactorrhea and elevated serum prolactin
are due to stalk effect (Dr. Kerr)
C. Prolactin is being secreted by a small cell
carcinoma of the lung
01
Answer
1. A 32-year-old female patient is noted to have
mild galactorrhea and a mild elevation of her
serum prolactin level. MRI of the pituitary
identifies a large sellar and suprasellar mass.
Each of the following is a reasonable
explanation for these clinical findings
EXCEPT
A. Mass is a secretory / functional prolactinoma
B. Neoplasm is a null cell adenoma and
galactorrhea and elevated serum prolactin
are due to stalk effect (Dr. Kerr)
C. Prolactin is being secreted by a small cell
carcinoma of the lung
C. is incorrect: to my knowledge small cell carcinomas have not been noted to produce prolactin – although
they CAN produce ACTH Cushing syndrome (paraneoplastic / ectopic production (ectopic to pituitary)).
A. is clearly an option: usually (if “secretory”) level is quite high and not “mild.” B. is also correct:
compression of infundibulum (“Stalk Effect”) can reduce dopamine getting to the anterior pituitary. The
control of prolactin secretion is via inhibition by dopamine rather than stimulation (other pituitary
endocrine cells need to be stimulated (e.g. thyrotrophs need TRH to stimulate their secretion of TSH))
Lack of inhibition mildly elevated prolactin level (but usually small elevations, not super-
high as seen in some prolactinomas)
02
Question
1. Functional pituitary adenomas secrete a
hormone. Each of the following is a
“syndrome” associated with a functional
pituitary adenomas EXCEPT
A. Cushing syndrome
B. Acromegaly
C. Hyperthyroidism
D. Galactorrhea and amenorrhea
E. Hyperparathyroidism
02
Answer
1. Functional pituitary adenomas secrete a
hormone. Each of the following is a
“syndrome” associated with a functional
pituitary adenomas EXCEPT
A. Cushing syndrome
B. Acromegaly
C. Hyperthyroidism
D. Galactorrhea and amenorrhea
E. Hyperparathyroidism
Note:
Clarification re. Nomenclature based on Size
►Microadenoma:
- Tumor less than or equal to 1 cm (10 mm) in greatest dimension
- Remains confined to the sellar region
►Macroadenoma:
- Greater than 1 cm in greatest dimension
- More severely distorts adjacent tissues (compared to microadenoma)
- Traction on the dura of the sellar floor or dura overlying the sella (diaphragma sella)
- causing headaches.
(Extracted from Dr. DeMasters’ PowerPoint File)

03
Question
NON-functional pituitary adenoma
neoplastic cells can be “null cell” (not making
a hormone) or be non-secretory (making
hormone but don’t secrete it or secrete it in
physiologically non-detectable levels). Each
of the following is an example of mass effect
that can be seen in non-functional (and large
functional) adenomas EXCEPT
A. Vision: Visual deficits due to Compression of
optic chiasm
B. Pituitary: Panhypopituitarism due to
destruction of normal pituitary parenchyma
C. Bone: Osteoporosis due to uncontrolled
secretion of parathormone
D. Barest: Lactation due to “stalk effect” and
loss of dopamine inhibition on normal
prolactin-secreting cells
03
Answer
NON-functional pituitary adenoma
neoplastic cells can be “null cell” (not making
a hormone) or be non-secretory (making
hormone but don’t secrete it or secrete it in
physiologically non-detectable levels). Each
of the following is an example of mass effect
that can be seen in non-functional (and large
functional) adenomas EXCEPT
A. Vision: Visual deficits due to Compression of
optic chiasm
B. Pituitary: Panhypopituitarism due to
destruction of normal pituitary parenchyma
C. Bone: Osteoporosis due to uncontrolled
secretion of parathormone
D. Barest: Lactation due to “stalk effect” and
loss of dopamine inhibition on normal
prolactin-secreting cells
04
Question
A 2 cm lesion in a patient’s pituitary is
resected and shows the histologic
findings seen at right. Each of the
following is correct EXCEPT:
A. Monomorphic population of cells (lack of
variably eosinophilic, basophilic, and
chromophobic cytoplasm) with similar
nuclear and cytoplasmic features
B. Sheets of cells are seen rather than the
typical nests of neuroendocrine cells
surrounded by fibrovascular cores
C. Findings are consistent with the diagnosis
pituitary adenoma
D. Consistent with metastatic ductal
adenocarcinoma from breast
04
Answer
A 2 cm lesion in a patient’s pituitary is
resected and shows the histologic
findings seen at right. Each of the
following is correct EXCEPT:
A. Monomorphic population of cells (lack of
variably eosinophilic, basophilic, and
chromophobic cytoplasm) with similar
nuclear and cytoplasmic features
B. Sheets of cells are seen rather than the
typical nests of neuroendocrine cells
surrounded by fibrovascular cores
C. Findings are consistent with the diagnosis
pituitary adenoma
D. Consistent with metastatic ductal
adenocarcinoma from breast
Expect gland formation

(adenocarcinoma) in setting of
ductal carcinoma of breast.
05
Question
A 55-year-old woman presents with a
severe headaches of sudden onset
and loss of vision; she later dies of
pneumonia. Each is correct EXCEPT:
A. Hemorrhage
B. Findings consistent with pituitary Normal Control
“apoplexy”
C. Craniopharyngioma
D. Adenoma commonly underlies such
lesions
Patient
05
Answer
A 55-year-old woman presents with a
severe headaches of sudden onset
and loss of vision; she later dies of
pneumonia. Each is correct EXCEPT:
A. Hemorrhage
B. Findings consistent with pituitary Normal Control
“apoplexy”
C. Craniopharyngioma
D. Adenoma commonly underlies such
lesions
Patient
05
Pituitary Apoplexy- Defined

• “Apoplexy”
– Acute pituitary-related event: usually hemorrhage into pituitary,
• normal gland (consider vascular malformation)
• hyperplasia (e.g. physiologic, in pregnancy)
• adenoma
AND
– Clinical features:
• Headache - acute onset
• +/- visual impairment
• +/- neurologic impairment (mental status changes)
• +/- endocrine or hemodynamic changes
• Histologic Features
– Hemorrhage
• Acute (Dr. DeMasters’ Lecture)
• Chronic
– Blood breakdown products (including cholesterol clefts, hemosiderin laden
macrophages) - - discern from contents of Rathke cleft cyst,
craniopharyngioma
– **Rule out NEOPLASM - - adenoma, metastasis, Rathke cleft cyst,
craniopharyngioma
6
Question
The lesion in this patient’s

pituitary is best classified as
A. Hemorrhage
B. Inflammation
C. Neoplasm
D. Abbess
6
Answer
The lesion in this patient’s

pituitary is best classified as
A. Hemorrhage
B. Inflammation
C. Neoplasm
D. Abbess
7
Question
The descriptive diagnosis for this
lesion is
A. Abscess
B. Adenoma
C. Lymphocytic hypophysitis
D. Granuloma
7
Answer
The descriptive diagnosis for this
lesion is
A. Abscess
B. Adenoma
C. Lymphocytic hypophysitis
D. Granuloma
Etiologic Approach to Differential Diagnosis: Endocrine
Symptoms / Signs / Imaging / Biopsy / Laboratory Analysis
Normal Abnormal
Reactive /
Neoplastic Hyperplastic Non-Neoplastic
Primary “Metastatic” Developmental Other
Hematopoietic
Spindle Cell Traumatic
Lymphoma Toxic -
Small Round
• External Metabolic
Blue Cell • Compression by
Leukemia adjacent structure • Exogenous
Epithelial / - Toxin
Epithelioid Degenerative - Mineral
(e.g. carcinoma) - Vitamin
- Protein deficiency
- etc.
Infectious Inflammatory Vascular • Endogenous
• Bacterial • Autoimmune • Infarct (hypoxia- - Endocrine
ischemia) - Enzyme Defect
• Fungal (connective tissue
• Viral disease) - Focal vs. Global - Lysosomal storage
- Pale disease
• Parasitic • Paraneoplastic
- Hemorrhagic - Peroxisomal
• Sarcoid
conversion disease
• Hemorrhage - Other protein defect
(neoplastic divide)
Lymphocytic hypophysitis (primary hypophysitis) is an autoimmune
disorder classically affecting young women during late pregnancy or in
the early postpartum period, mimics adenoma on neuroimaging, and
shows sheets
of cytologically bland pure lymphocytic infiltrates.
(Extracted from Dr. DeMasters’ PowerPoint File)

Thyroid
A
A. Parathyroid
B. Thyroid
C. Pituitary
D. Adrenal
A ANSWER
A. Parathyroid
B. Thyroid
C. Pituitary
D. Adrenal
Double- Lobed organ

- Isthmus dividing the lobes
B
A. Parathyroid
B. Thyroid
C. Pituitary
D. Adrenal
B ANSWER
Perifascicular / C Cell
A. Parathyroid
B. Thyroid
C. Pituitary
D. Adrenal
Follicle:
- Follicular EPITHELIAL Cells
- Colloid
B ANSWER
Perifascicular / C Cell
Thyroid Gland Histology
H+E Calcitonin
Immunohistochemistry
01 A
Question
A 31-year-old woman noticed a progressive
increase in the size of her neck along with a
ravenous appetite in the absence of weight gain.
On examination of her eyes, there was evidence of
proptosis / exophthalmos. Laboratory testing: free
T4 2.2 ng/dL (normal range 0.76-1.8), TSH <0.1
(normal range 0.4 and 5.0 mIU/mL). A radioactive
iodine scan identified diffuse, markedly increased
uptake, well above the upper limits of normal.
Complete thyroidectomy was performed and
histologic evaluation showed the above features
diffusely in the right and left lobes. Each of the
following is consistent with this scenario EXCEPT
A. The low TSH level is the result of oversecretion of
thyroid hormone
B. In the photograph, the cleared out areas of colloid
are called "scalloping"
C. Findings are consistent with Hashimoto thyroiditis
D. Anti-TSH receptor antibodies were found in her
serum
E. The patient likely had tachycardia
01 A
Answer
A 31-year-old woman noticed a progressive
increase in the size of her neck along with a
ravenous appetite in the absence of weight gain.
On examination of her eyes, there was evidence of
proptosis / exophthalmos. Laboratory testing: free
T4 2.2 ng/dL (normal range 0.76-1.8), TSH <0.1
(normal range 0.4 and 5.0 uIU/mL). A radioactive
iodine scan identified diffuse, markedly increased
uptake, well above the upper limits of normal.
Complete thyroidectomy was performed and
histologic evaluation showed the above features
diffusely in the right and left lobes. Each of the
following is consistent with this scenario EXCEPT
A. The low TSH level is the result of oversecretion of
thyroid hormone
B. In the photograph, the cleared out areas of colloid
are called "scalloping"
C. Findings are consistent with Hashimoto
thyroiditis
D. Anti-TSH receptor antibodies were found in her
serum
E. The patient likely had tachycardia
We are not seeing the inflammatory infiltrates of
Hashimoto thyroiditis. Graves disease can have some
inflammation, but the hyperplasia and hypertrophy
along with “scalloping” of the colloid is characteristic.
01 A
GravE Disease HashimOtO Disease
Thyroid Hormone
HypERthyroidism Hyp Othyrodism
Release
Antibody(ies) - TSH receptor - TSH receptor
Against = STIMULATE = Damage / Destroy
- Thyroglobulin
- Peroxidase
- Microsomal
Inflammation - Lymphocytic infiltrates: not as - Lymphocytic infiltrates AND Germinal
prominent as Hashimoto centers
Epithelium - Hyperplasia within follicles - Pink = “Hürthle cell change”
- Colloid Scalloping
Colloid - Scalloped (untreated - Reduced volume
Long-Term - Progression - Fibrosis / end stage
(Robbins 2010)
Exophthalmos PrE-Tibial
Myxedema
01 B
Question
The most likely ongoing etiologic

process in the images taken from a
resected thyroid is:
A. Neoplastic
B. Inflammatory
01 B
Answer
The most likely ongoing etiologic

process in the images taken from a
resected thyroid is:
A. Neoplastic
B. Inflammatory
01 C
Question
The best diagnosis in this case is:
A. Thyroiditis
B. Vasculitis
01 C
Answer
The best diagnosis in this case is:
A. Thyroiditis
B. Vasculitis
(Robbins 2010)
01 C
GravE Disease HashimOtO Disease
Thyroid Hormone
HypERthyroidism Hyp Othyrodism
Release
Antibody(ies) - TSH receptor - TSH receptor
Against = STIMULATE = Damage / Destroy
- Thyroglobulin
- Peroxidase
- Microsomal
Inflammation - Lymphocytic infiltrates: not as - Lymphocytic infiltrates AND Germinal
prominent as Hashimoto centers
Epithelium - Hyperplasia within follicles - Pink = “Hürthle cell change”
- Colloid Scalloping
Colloid - Scalloped (untreated - Reduced volume
Long-Term - Progression - Fibrosis / end stage
(Robbins 2010)
Exophthalmos PrE-Tibial
Myxedema
02
Question
Each of the following is true about
medullary thyroid carcinoma EXCEPT
A. Neoplasm of C / parafollicular cells

B. Neoplastic cell produce calcitonin (may
not be functional / secreted)
C. Neoplastic cells secrete parathormone
02
Answer
Each of the following is true about
medullary thyroid carcinoma EXCEPT
A. Neoplasm of C / parafollicular cells

B. Neoplastic cell produce calcitonin (may
not be functional / secreted)
C. Neoplastic cells secrete parathormone
Calcitonin
03
Question
Histologic evaluation of a resected thyroid
neoplasm identifies small follicles which
merge with the adjacent normal thyroid with
no separation by a capsule / pseudocapsule.
Examination of the cells forming the follicles
(cytology) reveals grooves in the nuclei and
intracytoplasmic cytoplasmic extensions /
cleared out areas. No vascular invasion is
identified. This neoplasm is best classified as
A. Follicular carcinoma
B. Papillary carcinoma
C. Follicular variant of papillary carcinoma
D. Medullary carcinoma
03
Answer
Histologic evaluation of a resected thyroid
neoplasm identifies small follicles which
merge with the adjacent normal thyroid with
no separation by a capsule / pseudocapsule.
Examination of the cells forming the follicles
(cytology) reveals grooves in the nuclei and
intracytoplasmic cytoplasmic extensions /
cleared out areas. No vascular invasion is
identified. This neoplasm is best classified as
A. Follicular carcinoma
B. Papillary carcinoma
C. Follicular variant of papillary carcinoma
D. Medullary carcinoma
- Architecture: Follicular
- Cytology: Papillary carcinoma features
While confusing by nomenclature, the diagnosis

of “papillary carcinoma” is based on cytologic
features of the neoplastic cells.
04
Question
You see a patient who has a large,
symmetrical neck mass. Each of the
following would support the diagnosis of
multinodular goiter EXCEPT
A. Abundant nodules on palpation and
ultrasound
B. Normal to low normal thyroid hormone levels
C. Living in a location where iodine deficiency is
present
D. Anti-thyroglobulin antibodies on serologic
evaluation
E. Hyperplastic nodules and cystic
degeneration on histologic evaluation
04
Answer
You see a patient who has a large,
symmetrical neck mass. Each of the
following would support the diagnosis of
multinodular goiter EXCEPT
A. Abundant nodules on palpation and
ultrasound
B. Normal to low normal thyroid hormone levels
C. Living in a location where iodine deficiency is
present
D. Anti-thyroglobulin antibodies on
serologic evaluation
E. Hyperplastic nodules and cystic
degeneration on histologic evaluation
Antithyroglobulin antibodies (destroy!!) are characteristic of

Hashimoto thyroiditis.
05
Question
1. Each of the following is correct about
Hashimoto thyroiditis EXCEPT:
A. Associated with destruction of thyroid
epithelium
B. Associated with anti-thyroglobulin antibodies
C. Results ultimately in hypothyroidism
D. TSH receptor antibodies are present and
stimulate release of thyroid hormone
E. Hashimoto thyroiditis is precursor of most
primary thyroid lymphomas
05
Answer
1. Each of the following is correct about
Hashimoto thyroiditis EXCEPT:
A. Associated with destruction of thyroid
epithelium
B. Associated with anti-thyroglobulin antibodies
C. Results ultimately in hypothyroidism
D. TSH receptor antibodies are present and
stimulate release of thyroid hormone
E. Hashimoto thyroiditis is precursor of most
primary thyroid lymphomas
Anti-TSH receptor antibodies (stimulate!!) are

characteristic of Graves disease
Antithyroglobulin antibodies (destroy!!) are

characteristic of Hashimoto thyroiditis.
06
Question
The images are from a resected
thyroid lesion. The inset shows
high-magnification of nuclei from
the lesion on a cytologic
preparation. The BEST diagnosis
is:
A. Follicular adenoma
B. Folicular carcinoma
C. Multinodular goiter
D. Papillary carcinoma
E. Graves disease
06
Answer
thyroid lesion. The inset shows
high-magnification of nuclei from
the lesion on a cytologic
preparation. The BEST diagnosis
is:
B. Folicular carcinoma
E. Graves disease
- Architecture: Papillary
- Cytology: Papillary carcinoma features
The diagnosis of “papillary carcinoma” is

based on cytologic features of the
neoplastic cells.
07
Question
thyroid lesion. The small photo
shows a blood vessel seen in the
capsule surrounding the lesion.
The BEST diagnosis is:
B. Follicular carcinoma
E. Graves disease
07
Answer
thyroid lesion. The small photo
shows a blood vessel seen in the
capsule surrounding the lesion.
The BEST diagnosis is:
B. Follicular carcinoma
E. Graves disease
- Architecture: Follicular
- Cytology: Nothing special: would need high
power image to evaluate
= rule out follicular cytologic features
- Grooved nuclei
- Cleared nuclei
- Invasion: capsule (arrows) AND vessel lumen
08
Question
Regarding thyroid neoplasms, each of the
following associations is correct EXCEPT:
A. Follicular adenoma: no capsular or vascular
invasion
B. Papillary carcinoma: nuclear clearing and
grooves
C. Follicular carcinoma: capsular and vascular
invasion present
D. Anaplastic carcinoma: excellent prognosis
E. Medullary carcinoma: tumor of C cells
08
Answer
Regarding thyroid neoplasms, each of the
following associations is correct EXCEPT:
A. Follicular adenoma: no capsular or vascular
invasion
B. Papillary carcinoma: nuclear clearing and
grooves
C. Follicular carcinoma: capsular and vascular
invasion present
D. Anaplastic carcinoma: excellent
prognosis
E. Medullary carcinoma: tumor of C cells
Anaplastic Thyroid Carcinoma:

“Spindle Cell” variant
- No papillary features (Drs. Marshall &
- No follicular features Haugen Lecture)
Poor prognosis
09
Question
The most likely diagnosis for the
lesion in this patient’s neck is:
A. Parathyroid adenoma
B. Parathyroid hyperplasia
B. Thyroid goiter
C. Thyroid medullary carcinoma
D. Thyroid papillary carcinoma
(Essentials of Rubin’s
Pathology, 5th ed., 2009)
09
Answer
The most likely diagnosis for the
lesion in this patient’s neck is:
B. Parathyroid hyperplasia
B. Thyroid goiter
D. Thyroid papillary carcinoma
Iodine deficiency-related Goiters can

achieve massive proportions, as in this
patient’s neck, and can manifest with
(ultimately) hypOthoridism, airway
obstruction, and swallowing deficits.
The other lesions do not achieve such
massive size.
10
Question
Compared to a control
thyroid at top, the BEST
diagnosis for the
Control Thyroid
macroscopic (gross)
findings in the resected
thyroid below is:
A. Adenoma Right Lobe

Left Lobe
B. Carcinoma
C. Goiter
Cross-Section
10
Answer
Compared to a control
thyroid at top, the BEST
diagnosis for the
Control Thyroid
macroscopic (gross)
findings in the resected
thyroid below is:
A. Adenoma Right Lobe

Left Lobe
B. Carcinoma
C. Goiter
Cross-Section
-Both
lobes
enlarged,
diffusely
- Adenoma
and
carcinoma
Usually
consist of
a single
lesion
10
Goiter = Enlargement of
Thyroid Gland
• “Goiters” most commonly form when

some derangement that hampers
thyroid hormone output (e.g. iodine
deficiency) causes compensatory
elevation of the TSH level.
– The TSH causes hyperplasia and
hypertrophy of thyroid epithelial
cells
• Variations
– Diffuse goiter
– Multinodular goiter
• Functional Outcome
– Euthyroidism – hyperplasia
overcomes deficits
– Hyperthyroidism -- when there is
an autonomous hyperfunctioning
nodule
• “Toxic Goiter” (~10%)
– Hypothyroidism – ultimate
outcome
(Robbins 2010)
10
Goiter Histology
Normal-Sized Follicles for Comparison
“Colloid Nodule”: “Colloid Nodule”: Degenerative / Traumatic

Low Magnification Medium Magnification changes
Only PART of one of MANY “colloid
nodules” is shown.
H+E Stain
11
Question
Images show histologic findings in a
resected thyroid. Given the abnormality
depicted, which is INCORRECT?
A. C-Cell hyperplasia
B. Can be seen in MEN I
C. Can be seen in MEN II
Calcitonin
Immuno.
11
Answer
resected thyroid. Given the abnormality
depicted, which is INCORRECT?
A. C-Cell hyperplasia
B. Can be seen in MEN I
C. Can be seen in MEN II
MEN I
-P
-P Calcitonin
-P Immuno.
MEN II A MEN II B
-P -
-T -T
-A -A
- -N
11
Multiple Endocrine Neoplasia
MEN-1 - Wermer: PPP-D MEN-2: PTA-N-MA

- - 11q13 -- tumor suppresser gene - - Ret – tyrosine kinase receptor / 10q
- Pituitary: - Adenoma (usually MEN-2A – PTA = Sipple Syndrome

prolactinoma; occasionally
somatotroph adenoma - Parathyroid - Hyperplasia
(acromegaly))
- Thyroid - C-cell hyperplasia / medullary
carcinoma(s)
- Parathyroid: - Hyperplasia and - Adrenal - Pheochromocytoma(s)
- Adenoma(s)
- Pancreas (islet): - Pancreatic Endocrine

MEN-2B - PTAN - ma
Neoplasm (PEN / islet cell - Parathyroid
tumor(s)) (silent, - Thyroid - C-cell hyperplasia / medullary
gastrinoma, carcinoma(s)
insulinoma) - Adrenal - Pheochromocytoma
- Neuroma - Neuromas,
ganglioneuromas
- Duodenum - Gastrinoma - Marfanoid
habitus
12
22
Question
resected thyroid. Note the
immunohistochemical findings on this
neuroendocrine neoplasm. The correct
diagnosis is
A. C-cell hyperplasia
B. C-cell adenoma
C. Medullary thyroid carcinoma
Normal
Thyroid
Abnormality
Calcitonin
(Robbins 2008) Immunohistochemistry
12
22
Answer
Images show histologic findings in a “Amyloid” – local production
resected thyroid. Note the
immunohistochemical findings on this
neuroendocrine neoplasm. The correct
diagnosis is The term C-
A. C-cell hyperplasia Cell adenoma

is not used to
my
B. C-cell adenoma knowledge
C. Medullary thyroid carcinoma
Normal
Thyroid
Abnormality
Calcitonin
(Robbins 2008) Immunohistochemistry
Parathyroid
A
Macroscopic / Microscopic Challenge:

A. Parathyroid
B. Thyroid
C. Pituitary
D. Adrenal
A ANSWER
Macroscopic / Microscopic Challenge:

A. Parathyroid
B. Thyroid
C. Pituitary
D. Adrenal
Low Power Histology: H&E stain
- Lobular shape
- Capsule
- Adipose: increases with age
- IntERcelluar / - IntRAcellular
- Cells: reduced with age
- Vascularity: rich – note
congested blood vessels
B
A. Parathyroid
B. Thyroid
C. Pituitary
D. Adrenal
B ANSWER
A. Parathyroid
B. Thyroid
C. Pituitary
D. Adrenal
Note:
-“Neuroendocrine cells”:
- monomorphic nuclei
- granular cytoplasm (except pink guys)
(granularity: neurosecretory vesicles)
- Admixed adipocytes
- Rich vascularity
C
The cells toward the top secrete
parathormone. This cell type is called:
A. Oxyphil cell
B. Chief cell
C ANSWER
The cells toward the top secrete
parathormone. This cell type is called:
A. Oxyphil cell
B. Chief cell
See next slide

C ANSWER
Organ Endocrine / Neuroendocrine Cells

Cell Hormone Hyperplasia / Neoplasia
Parathyroid Chief Cell 1. Parathormone 1. Hyperplasia (X4 usually)
(chiefly - Sporadic - MEN-1 - MEN-2A
responsible
for hormone 2. Adenoma (X1 most commonly)
production; can - Sporadic - MEN-1
have a
"water clear” 3. Carcinoma – uncommon
appearance)
Oxyphil Cell 1. “None:” -

(acidophilic / "Resting" / inactive
mitochondria
increased / fat,
happy)
C ANSWER
“Neuroendocrine Cells”
H&E Chromogranin
Immunohistochemistry
-“Neuroendocrine cells”:
(neurosecretory protein)
- “salt-and-pepper” chromatin
- monomorphic nuclei
Highlights
- granular cytoplasm (except pink guys)
Neurosecretory Granules
(granularity: neurosecretory vesicles)
C ANSWER
“Neuroendocrine Cells”
Architecture:
- Nested
- Ribbon-like
Cytology:
- “Salt-and-pepper” chromatin
- Monomorphic nuclei
- Granular cytoplasm
(granularity:
neurosecretory vesicles)
Parathyroid Pancreatic Islets
Note: Adrenal
cortical cells have
abundant SER
(smooth
endoplasmic
reticulum) to
synthesize their
neurosecretory
product – look a
bit foamy.
Adrenal Cortex Pituitary Thyroid C-Cells

01
Question
The parathyroids shown at right are from a
patient with chronic renal failure. (The normal
parathyroid would be less than ½ the size of
the smallest gland shown.) Each of the
following is true about lesions of the
parathyroid EXCEPT
A. Adenoma is the most common cause of
"primary" hyperparathyroidism
B. Hyperplasia is the most common finding in
chronic renal failure
C. Hyperplasia can be seen in MEN-1 and
MEN-2A
D. Parathyroid carcinoma is a common disease
process
E. Chief cells are responsible for parathormone
secretion
Patient’s Control
Kidney Kidney
01
Answer
The parathyroids shown at right are from a
patient with chronic renal failure. (The normal
parathyroid would be less than ½ the size of
the smallest gland shown.) Each of the
following is true about lesions of the
parathyroid EXCEPT
A. Adenoma is the most common cause of
"primary" hyperparathyroidism
B. Hyperplasia is the most common finding in
chronic renal failure
C. Hyperplasia can be seen in MEN-1 and
MEN-2A
D. Parathyroid carcinoma is a common
disease process
E. Chief cells are responsible for parathormone
secretion
Parathyroid carcinoma is rare.
It is defined by biological aggressiveness: Patient’s Control

= invasion of surrounding soft tissue and metastasis. Kidney Kidney
02
Question
A patient with chronic renal failure is noted to
have hypocalcemia and an elevated
parathormone level. All of the following are
likely findings in the evaluation of his
parathyroids EXCEPT
A. Surgical Exploration: All four glands
increased in size
B. Radiology: Increased uptake in all four glands
noted by radionucleotide scan
C. Histology: increased cellularity on histologic
evaluation
D. Histology: reduced intracellular and
extracellular fat on histologic evaluation
E. Surgical Exploration: One gland enlarged;
others small
02
Answer
A patient with chronic renal failure is noted to
have hypocalcemia and an elevated
parathormone level. All of the following are
likely findings in the evaluation of his
parathyroids EXCEPT
A. Surgical Exploration: All four glands
increased in size
B. Radiology: Increased uptake in all four glands
noted by radionucleotide scan
C. Histology: increased cellularity on histologic
evaluation
D. Histology: reduced intracellular and
extracellular fat on histologic evaluation
E. Surgical Exploration: One gland enlarged;
others small
Renal failure secondary hyperparathyroidism.
- Low calcium / increased parathormone characteristic
Enlargement of all four glands is expected, likely variably enlarged but

enlarged: increased cellularity / reduced adipose
A single enlarged gland would be highly unlikely in this setting.

03
Question
A 65-year-old man has end-

stage hypertensive and
diabetic kidney disease
requiring dialysis. Laboratory
studies identify a low calcium
level but an elevated
parathyroid hormone level.
The process illustrated in this
case is most consistent with
A. Primary
hyperparathyroidism
C. Secondary
hyperparathyroidism
C. Tertiary
hyperparathyroidism
Patient’s Control
Kidney Kidney
03
Answer
A 65-year-old man has end-

stage hypertensive and
diabetic kidney disease
requiring dialysis. Laboratory
studies identify a low calcium
level but an elevated
parathyroid hormone level.
The process illustrated in this
case is most consistent with
A. Primary
hyperparathyroidism
C. Secondary
hyperparathyroidism
C. Tertiary
hyperparathyroidism
Patient’s Control
Kidney Kidney
04
Question
A patient is discovered to have a high
calcium level and a high parathormone
(parathyroid hormone) level. Technetium
radionuclide scan reveals a single enlarged
gland with high uptake. Each of the following
is compatible with this clinical scenario
EXCEPT:
A. Laboratory evaluation reveals an elevated
parathyroid hormone level
B. The patient likely has Chronic renal failure
C. This lesion could be sporadic or familial
D. This finding is seen in 85-95% of cases of
“primary” hyperparathyroidism
E. The lesion is probably an adenoma
04
Answer
A patient is discovered to have a high
calcium level and a high parathormone
(parathyroid hormone) level. Technetium
radionuclide scan reveals a single enlarged
gland with high uptake. Each of the following
is compatible with this clinical scenario
EXCEPT:
A. Laboratory evaluation reveals an elevated
parathyroid hormone level
B. The patient likely has Chronic renal failure
C. This lesion could be sporadic or familial
D. This finding is seen in 85-95% of cases of
“primary” hyperparathyroidism
E. The lesion is probably an adenoma
Parathyroid hyperplasia is noted in the photograph of a parathyroid

from a patient with hyperparathyroidism … with absent fat.
NOTE: A pathologist CANNOT distinguish

- Adenoma
- Hyperplasia
on a single gland … they are identical. Information about ALL glands (or
most glands) is needed to discern between these possibilities.
04
1° vs. 2° Parathyroid Disease / Calcium Effects

05
Question
The schematic at right depicts

A. Acromegaly
B. Hyperparathyroidism
C. Panhypopituitarism
D. Hyperthyroidism
E. Hypothyroidism
F. Cushing syndrome
G. Diabetes insipidus
(Essentials of Rubin’s Pathology, 5th ed., 2009)

05
Answer
Moans
Psychic
The schematic at right depicts

A. Acromegaly
C. Panhypopituitarism Bones
D. Hyperthyroidism Painful
E. Hypothyroidism
F. Cushing syndrome
Groans
G. Diabetes insipidus Abdominal
Stones
Renal

Pancreas:
Exocrine
A
A. Pancreas
B. Thyroid
C. Pituitary
D. Adrenal
A ANSWER
A. Pancreas
B. Thyroid
C. Pituitary
D. Adrenal
See next slide

A ANSWER
Exocrine and Endocrine Histology and Chemistry
Islet Cells (Neuroendocrine)
A. Secrete: Insulin, Glucagon, Somatostatin, VIP
Acinar Cells B. Neoplasm: Pancreatic Endocrine Neoplasm (PEN)
(Epithelial)
A. Secrete Duct Cells
(1) Proteases (Epithelial)
- Trypsinogen A. Secrete:
- Chymotrypsi (1) Bicarbonate
nogen
- Proelastase B. Express
- Etc. (1) 19-9 mucin-
(2) Lipases associated
(3) Amylase protein
(4) Nucleases
(5) Other C. Neoplasm
Proteins (1) Pancreatic
- Trypsin intraepithelial
inhibitors neoplasia
- Etc. (PanIn)
= precursor
B. Neoplasms lesion
(rare) (2) Adenocarcinoma
(1) Acinar cell
carcinoma
(2) Pancreato- Normal Pancreas: Note both (1) exocrine components
blastoma (acini and ducts) and (2) endocrine (islet of Langerhans)
(pediatric)
components
01
Question
A 50-year-old man develops Cushing-type
features. Laboratory evaluation identifies a
high ACTH level but the source of his ACTH
is not identified by extensive workup
including whole-body imaging and petrosal
sinus sampling. He dies and an autopsy is
performed. A mass is identified in his
pancreas. Histology reveals nests of
neuroendocrine cells with ACTH labeling by
immunohistochemistry. The most likely
diagnosis of this mass given the is most
likely
A. Adenocarcinoma of pancreas
B. Pancreatic neuroendocrine neoplasm
C. Lymphoma
H&E ACTH
01
Answer
is not identified by extensive workup
including whole-body imaging and petrosal
sinus sampling. He dies and an autopsy is
performed. A mass is identified in his
pancreas. Histology reveals nests of
immunohistochemistry. The most likely
diagnosis of this mass given the is most
likely
A. Adenocarcinoma of pancreas
C. Lymphoma
The neoplasm has neuroendocrine features histologically and
immunohistochemistry for ACTH clinched the diagnosis. There was no
gland formation to suggest “adenocarcinoma.” The small cells were not
lymphocytes.
Ectopic secretion can be from

- Small cell CA of lung
- “Carcinoid” (neuroendocrine neoplasm) of lung
- “Carcinoid” of other sites (e.g. gut)
- Pancreas neuroendocrine neoplasm
While unusual – this neoplasm looked to be primary in the pancreas H&E ACTH
and secreted ACTH.
02
Question
is not identified by whole-body imaging and
petrosal sinus sampling. He dies and an
autopsy is performed. A mass is identified in
his pancreas. Histology reveals nests of
immunohistochemistry. Evaluation of the
patient’s liver identifies multifocal
(macroscopically subtle) metastases. The
best diagnosis of this mass given this
additional data is:
A. Adenocarcinoma
C. Malignant pancreatic neuroendocrine
neoplasm
D. Lymphoma
02
Answer
immunohistochemistry. Evaluation of the
patient’s liver identifies multifocal
(macroscopically subtle) metastases. The
best diagnosis of this mass given this
additional data is:
A. Adenocarcinoma
C. Malignant pancreatic neuroendocrine
neoplasm
D. Lymphoma
Malignancy in “Pancreatic Neuroendocrine Neoplasm”

-Local invasion
- Vascular invasion
- Metastasis
03 A
Question
Findings in the pancreas of an 11-
year-old boy, shown in the image at
right, are most consistent with:
A. Inflammatory disease
B. Toxic-metabolic disease
C. Neoplastic disease - primary
03 A
Answer
Findings in the pancreas of an 11-
year-old boy, shown in the image at
right, are most consistent with:
A. Inflammatory disease
B. Toxic-metabolic disease
C. Neoplastic disease - primary
03 B
Question
Findings in this pancreatic islet

are most consistent with:
A. Diabetes Type I
B. Diabetes Type II
C. Pancreatic endocrine
neoplasm
(Robbins 2010)
03 B
Answer
Findings in this pancreatic islet

are most consistent with:
A. Diabetes Type I
B. Diabetes Type II
C. Pancreatic endocrine
neoplasm
(Robbins 2010)
03 B
Islets of Langerhans
• Normal • Type I: • Type II Diabetes:
Inflammatory Toxic-Metabolic:
Insulin Resistance
(Robbins 2010)
Beta-Cells: Beta-Cells:
- Immune attack - ~Normal number /
- Reduced number some
reduction
Chronic:
- Fibrosis (scar Amyloid:
- Occasionally seen
- Chronicity
- Poor control
04
Question
In CHRONIC Diabetes type I, the
number of Beta cells in pancreatic
islets:
A. Remains the same

B. Is markedly reduced
C. Is increased
04
Answer
In CHRONIC Diabetes type I, the
number of Beta cells in pancreatic
islets:
Glucagon
A. Remains the same
B. Is markedly reduced
C. Is increased
Insulin
05
Question
An 18-year-old patient collapsed during
gym class. He had previously
experienced episodes of confusion
during physical activity. He had an
elevated pulse and was breathing
spontaneously. He was transported to
an emergency room. A metabolic panel
identified a serum glucose level of 37
mg/dL (normal 60-100 mg/dL). MRI
identified a small mass in his pancreas
and resection was carried out. In the
context of the clinical scenario, the most
likely diagnosis of the lesion indicated by
the red arrow is:
A. Adenocarcinoma of the pancreas
B. Pheochromocytoma
C. Pancreatic endocrine neoplasm
D. Acute pancreatitis
E. Metastatic testicular seminoma
05
Answer
An 18-year-old patient collapsed during
gym class. He had previously
experienced episodes of confusion
during physical activity. He had an
elevated pulse and was breathing
spontaneously. He was transported to
an emergency room. A metabolic panel
identified a serum glucose level of 37
mg/dL (normal 60-100 mg/dL). MRI
identified a small mass in his pancreas
and resection was carried out. In the
context of the clinical scenario, the most
likely diagnosis of the lesion indicated by
the red arrow is:
A. Adenocarcinoma of the pancreas
B. Pheochromocytoma
C. Pancreatic endocrine neoplasm
D. Acute pancreatitis
E. Metastatic testicular seminoma
05
Pancreatic Endocrine Neoplasm – Islet Cell Tumor
H&E Stain
06
Question
Findings at autopsy from
a patient with (1) poorly-
controlled diabetes and
(2) poorly-controlled
hypertension are shown.
Structures within the
glomerular mesangium
are called
A. Mallory bodies
B. Kimmelstiel-Wilson bodies
C. Normal
PAS Stain
06
Answer
Findings at autopsy from
a patient with (1) poorly-
controlled diabetes and
(2) poorly-controlled
hypertension are shown.
Structures within the
glomerular mesangium
are called
A. Mallory bodies
B. Kimmelstiel-Wilson bodies
C. Normal
K-W (Kimmelstiel-Wilson) bodies result from glycosylation of
mesangial proteins. They are seen in chronic, poorly controlled
diabetes and contribute to the problems in a diabetic patient’s kidneys.
Mallory bodies are seen in hepatocytes. They are seen in

- Ethanolism – but are NOT pathognomonic
- Ethanolism = Toxic-Metabolic
- Seen in Wilson disease (and other diseases)
- Ethanolism = Toxic-Metabolic
PAS Stain
07
45
Question
Same patient: What change is

noted in the renal artery indicated
by the blue arrow:
A. Atherosclerosis
B. Arteriolosclerosis
C. Vasculitis
07
45
Answer

noted in the renal artery indicated
by the blue arrow:
A. Atherosclerosis
C. Vasculitis
There is circumferential
arteriolosclerosis in the artery. This
change is seen in:
- Hypertension
- Diabetes
- Combinations of the two
Thee is no evidence of atherosclerotic

debris in the vessel wall to suggest
atherosclerosis.
There is no evidence of inflammation

in and damage to the vessel wall to
suggest vasculitis.
46
08
Question

noted in his aorta:
A. Atherosclerosis
C. Vasculitis
46
08
Answer

noted in his aorta:
A. Atherosclerosis
C. Vasculitis
09
49
Question
Histologic findings in pancreas of 56-
year-old man with a mass in the
pancreatic head identified the finding
shown at right. This disease is best
classified as
A. Neuroendocrine neoplasm
(pancreatic endocrine
neoplasm / islet cell tumor /
carcinoma)
B. Epithelial neoplasm
(pancreatic adenocarcinoma)
09
49
Answer
Histologic findings in pancreas of 56-
year-old man with a mass in the
pancreatic head identified the finding
shown at right. This disease is best
classified as
A. Neuroendocrine neoplasm
(pancreatic endocrine
neoplasm / islet cell tumor /
carcinoma)
B. Epithelial neoplasm
(pancreatic adenocarcinoma)
Adrenal
A
A. Parathyroid
B. Thyroid
C. Pituitary
D. Adrenal
A ANSWER
A. Parathyroid
B. Thyroid Cortex:
C. Pituitary Golden Yellow
D. Adrenal
Medulla:
Brown / Mahogany
B
A. Pancreas
B. Thyroid
C. Pituitary
D. Adrenal
B ANSWER
A. Pancreas
B. Thyroid
C. Pituitary
D. Adrenal
See next slide

B ANSWER
Adrenal
Capsule
Glomerulosa
- Aldosterone
(Mineralocorticoid)
Cortex GFR
Fasciculata
- Cortisol
- Cortical Adenoma (Glucocorticoid)
- Cortical Carcinoma
Reticularis
- Sex hormones
Pheochromocytoma
Medulla
01
Question
A 16-year-old girl being treated with
immunosuppressive medication for lupus develops
a fever and headache. Emergency Department
evaluation including laboratory studies identifies a
septic physiology, DIC, and petechial hemorrhages
in the skin. N. meningitis is cultured from blood. She
does not survive. At autopsy, her adrenals are
diffusely hemorrhage in appearance. The best
diagnosis in this case is
1. Addison disease due to steroid use

2. Waterhouse-Frederickson syndrome
3. Adrenal hyperplasia
4. Kaposi sarcoma
01
Answer
A 16-year-old girl being treated with
immunosuppressive medication for lupus develops
a fever and headache. Emergency Department
evaluation including laboratory studies identifies a
septic physiology, DIC, and petechial hemorrhages
in the skin. N. meningitis is cultured from blood. She
does not survive. At autopsy, her adrenals are
diffusely hemorrhage in appearance. The best
diagnosis in this case is
1. Addison disease due to steroid use

2. Waterhouse-Frederickson syndrome
3. Adrenal hyperplasia
4. Kaposi sarcoma
The clinical and autopsy findings fit perfectly with
Waterhouse-Frederickson syndrome.
The adrenal pathology results in acute adrenal

insufficiency, perhaps complicated by steroid therapy-
related atrophy of the fasciculata cells. Note: “Addison
disease” is generally used to denote a (1) chronic and (2)
primary (non-pituitary / hypothal.-related) disease process.
01
Developmental
Toxic-Metabolic
Inflammatory
Infectious – direct and complications of infection
Toxic-Metabolic
Neoplastic - Metastatic
Mixed: Neoplastic / Inflammatory / Infectious
Toxic-Metabolic
(Robbins
2008)
02
Question
The arrow is pointing at which portion of the
adrenal
A. Cortex
B. Medulla
02
Answer
The arrow is pointing at which portion of the
adrenal
A. Cortex
B. Medulla
Adrenal cortex has a yellow color due to the production

of lipid-based hormonal products.
Adrenal medulla has a darker, brownish color, especially

after exposure to the air, due to the creation of its
neurotransmitter-type product.
03
Question
1. At autopsy, the right and left adrenals of a
70-year-old morbidly obese (over 400 pound)
woman had an appearance as seen at the
bottom, compared to a control (30-year-old
woman) patient. Based on the appearance of Normal Control
the adrenal, the most accurate diagnosis
for this finding is:
A. Normal
B. Adrenal cortical adenoma
C. Adrenal cortical carcinoma
D. Pheochromocytoma
D. Adrenal cortical hyperplasia
Patient
03
Answer
1. At autopsy, the right and left adrenals of a
70-year-old morbidly obese (over 400 pound)
woman had an appearance as seen at the
bottom, compared to a control (30-year-old
woman) patient. Based on the appearance of Normal Control
the adrenal, the most accurate diagnosis
for this finding is:
A. Normal
B. Adrenal cortical adenoma
C. Adrenal cortical carcinoma
D. Pheochromocytoma
D. Adrenal cortical hyperplasia
The adrenal cortex is diffusely expanded in a nodular
manner, characteristic of hyperplasia (or “nodular
hyperplasia”). The fact that both adrenals were the same
in appearance seals the diagnosis of hyperplasia. Note the
yellow-orange color of the normal and hyperplastic
adrenal.
Adenomas and carcinomas tend to be single, well-

circumscribed masses, yellow-orange in color.
Patient
Pheochromocytomas are well-circumscribed, brown-tan
masses.
04
Question
Based on its location and color, the
diagnosis of the lesion shown at right is
most likely
1. Adrenal cortical adenoma

2. Pheochromocytoma
3. Metastasis
4. Hyperplasia
04
Answer
most likely

2. Pheochromocytoma
3. Metastasis
4. Hyperplasia
The lesion appears to arise from and fuse with the adrenal cortex.
See arrows. This finding suggests an adrenal cortical origin.
Adrenal cortical neoplasms tend to retain the yellowish color of

the adrenal cortex, as seen in this image.
Pheochromocytomas tend to have a darker, brownish color.
Hyperplasia should be diffuse in the cortex.
Metastasis tend to be ill-defined. They tend to appear to be

located in the medulla but can involve cortex as well. Most would
not be expected to be distinctly yellow.
05
Question
diagnosis of the lesion removed
surgically (along with surrounding
adipose tissue) is most likely
2. Pheochromocytoma
3. Metastasis
05
Question
diagnosis of the lesion removed
surgically (along with surrounding
adipose tissue) is most likely
1. Adrenal cortical
adenoma
2. Pheochromocytoma
3. Metastasis
Adrenal cortical neoplasms tend to retain the
yellowish color of the adrenal cortex, as seen
in this image.
Pheochromocytomas tend to have a darker,

brownish color.
Metastasis tend to be ill-defined. They tend to

appear to be located in the medulla but can
involve cortex as well. Most would not be
expected to be distinctly yellow.
06
Question
most likely

2. Pheochromocytoma
3. Metastasis
06
Answer
most likely

2. Pheochromocytoma
3. Metastasis
Abnormal / Neoplasm / FAVOR Primary … like it’s at home

Appears to arise from the adrenal medulla.
- Medulla: The lesion has a similar color as normal adrenal

medulla (red arrow).
- Pheochromocytomas tend to have a dark
brownish or mahogany color.
- Cortex: Some adrenal cortex is discernible (see blue arrow,

yellow strips of tissue) … expanded / attenuated by the neoplasm.
- Adrenal cortical neoplasms tend to retain the
yellowish color of the adrenal cortex.
- Metastasis tend to be ill-defined. They tend to appear to be

located in the medulla but can involve cortex as well.
07
Question
From top to bottom, based on
macroscopic (gross) findings alone, the
order of the lesions at right is most likely
1. Normal, Adenoma, Pheochromocytoma

2. Normal Pheochromocytoma, adenoma
3. Adenoma, pheochromocytoma, normal
07
Answer
From top to bottom for these formalin
fixed specimens, based on macroscopic
(gross) findings alone, the order of the
lesions at right is most likely
1. Normal, Adenoma, Pheochromocytoma
2. Normal Pheochromocytoma, adenoma
3. Adenoma, pheochromocytoma, normal
Adrenal cortical neoplasms tend to retain the

yellowish color of the adrenal cortex.
Pheochromocytomas tend to have a darker,

brownish color.
08
Question
autopsy is performed. The best
macroscopic diagnosis of his adrenals
(both had the same appearance – with the
cortex markedly expanded; the color was
brown rather than yellow-orange with yellow
streaks admixed) is:
A. Adrenal adenoma
B. Adrenal carcinoma
C. Adrenal hyperplasia
08
Answer
autopsy is performed. The best
macroscopic diagnosis of his adrenals
(both had the same appearance – with the
cortex markedly expanded; the color was
brown rather than yellow-orange with yellow
streaks admixed) is:
A. Adrenal adenoma
B. Adrenal carcinoma
C. Adrenal hyperplasia
09
Question
immunohistochemistry. Assuming it is
primary, the most likely diagnosis of this
mass
A. Adenocarcinoma
C. Lymphoma
H&E ACTH
09
Answer
immunohistochemistry. Assuming it is
primary, the most likely diagnosis of this
mass
A. Adenocarcinoma
C. Lymphoma
H&E ACTH
10
Question
immunohistochemistry. The source of his
ACTH and then the etiology of his Cushing
syndrome is classified as:
A. Pituitary in origin = Cushing DISEASE

B. Ectopic = “paraneoplastic”
C. Adrenal in origin
H&E ACTH
10
Answer
immunohistochemistry. The source of his
ACTH and then the etiology of his Cushing
syndrome is classified as:
A. Pituitary in origin = Cushing DISEASE

B. Ectopic = “paraneoplastic”
C. Adrenal in origin
H&E ACTH
10
Cushing Disease / Cushing Syndrome
ACTH adenoma Syndrome
"Disease is in Da Pit"
(Robbins 2010)
11
Question
A 51-year-old woman develops a “moon
facies,” gains weight with an abdominal
predominance to the accumulation of fat, and
develops abdominal stria. She also
complains of being tired. Abdominal imaging
identifies a mass on the superior pole of the
right kidney measuring 22 X 13.5 cm in
greatest dimensions (image). The left
adrenal is atrophic. Serum ACTH is reduced;
urine cortisol level is increased. Based on
the patient’s symptoms, the most accurate
diagnosis clinically is:
A. Cushing disease
B. Cushing syndrome
11
Answer
A 51-year-old woman develops a “moon
facies,” gains weight with an abdominal
predominance to the accumulation of fat, and
develops abdominal stria. She also
complains of being tired. Abdominal imaging
identifies a mass on the superior pole of the
right kidney measuring 22 X 13.5 cm in
greatest dimensions (image). The left
adrenal is atrophic. Serum ACTH is reduced;
urine cortisol level is increased. Based on
the patient’s symptoms, the most accurate
diagnosis clinically is:
A. Cushing disease
B. Cushing syndrome
Findings are characteristic of Cushing

Syndrome.
Disease is a subset of “Syndrome.” It

arises in the context of
11
Diagnosis: Cushing Syndrome
(Robbins
2010)
(Robbins (Essentials of Rubin’s

2010) Pathology, 5th ed., 2009)
12
Question
1. The patient ultimately dies and an autopsy is
performed. Note the yellow color of the
mass. Given the color, the most likely
diagnosis is
A. Adrenal cortical lesion (adenoma vs.

carcinoma)
B. Pheochromocytoma
12
Answer
1. The patient ultimately dies and an autopsy is
performed. Note the yellow color of the
mass. Given the color, the most likely
diagnosis is
A. Adrenal cortical lesion (adenoma vs.

carcinoma)
B. Pheochromocytoma
Adrenal cortical neoplasms tend to retain

the yellowish color of the adrenal cortex.
Pheochromocytomas tend to have a

darker, brownish color.
13
Question
1. The patient’s mass measures 22 X 13.5 cm
in greatest dimensions (image). Based on
size alone, the most likely diagnosis is:
A. Adrenal cortical adenoma

B. Adrenal cortical carcinoma
13
Answer
1. The patient’s mass measures 22 X 13.5 cm
in greatest dimensions (image). Based on
size alone, the most likely diagnosis is:
A. Adrenal cortical adenoma

B. Adrenal cortical carcinoma
Adrenal cortical adenomas generally

measure
- less than 2.5 cm
but can occasionally be larger.
Adrenal cortical carcinomas, at the time of

detection, tend to measure
- greater than 2.5 cm in dimensions but
can occasionally be smaller.
They behave aggressively: e.g. metastasize

… as occurred in this patient.
The massive size of this lesion is strongly

suggestive of carcinoma.
14
Question
1. At autopsy a patient is found to have a 3 cm
yellow adrenal nodule associated with the
adrenal cortex. Autopsy also reveals lesions
in the vertebral bodies (top) and liver). Based
on this combined data, which of the following
is the MOST LIKELY diagnosis of the lesion
in the adrenal
A. Adrenocortical adenoma
B. Adrenocortical carcinoma
Vertebral Bodies
Liver
14
Answer
1. At autopsy a patient is found to have a 3 cm
yellow adrenal nodule associated with the
adrenal cortex. Autopsy also reveals lesions
in the vertebral bodies (top) and liver). Based
on this combined data, which of the following
is the MOST LIKELY diagnosis of the lesion
in the adrenal
A. Adrenocortical adenoma
B. Adrenocortical carcinoma
Adenomas do not metastasize.
Adrenal cortical carcinomas behave badly

(aggressively) and (1)locally invade and (2)
metastasize.
15
Question
A 32-year-old woman with hypertension was
evaluated. Workup revealed an adrenal
medulla mass. It was resected. The
pathologist’s diagnosis was
pheochromocytoma. Additional workup one
year later was undertaken which two
separate thyroid masses and the diagnosis
of medullary thyroid carcinoma was made.
Which do you favor:
A. Sporadic occurrence of neoplasms
B. Familial process
Neuron-like (ganglion cell) appearance

of pheochromocytoma.
15
Answer
A 32-year-old woman with hypertension was
evaluated. Workup revealed an adrenal
medulla mass. It was resected. The
pathologist’s diagnosis was
pheochromocytoma. Additional workup one
year later was undertaken which two
separate thyroid masses and the diagnosis
of medullary thyroid carcinoma was made.
Which do you favor:
A. Sporadic occurrence of neoplasms
B. Familial process
Sounds familial on first glance, like there is a germ line
mutation predisposing her to “multiple endocrine
neoplasms” – suggestive of MEN 2. Cannot distinguish
2A and 2B from what we know at the moment.
Neuron-like (ganglion cell) appearance
Thyroid mass should be evaluated and treated.
of pheochromocytoma.
It’s worth pursuing the diagnosis in this patient … and if
genetically confirmed … suggesting genetic counseling to
family members.
16
Question
This 32-year-old woman’s thyroid was
resected and a diagnosis was made. If this
patient indeed has MEN 2, which of the
following do you favor as the diagnosis of her
thyroid lesions:
A. Medullary carcinoma
B. Follicular carcinoma Adrenal Lesion
C. Follicular adenoma
Thyroid Lesion
16
Answer
This 32-year-old woman’s thyroid was
resected and a diagnosis was made. If this
patient indeed has MEN 2, which of the
following do you favor as the diagnosis of her
thyroid lesions:
A. Medullary carcinoma
B. Follicular carcinoma Adrenal Lesion
C. Follicular adenoma
Thyroid Lesion
17
Question
A 38-year-old woman with a history of lupus
sought medical attention after noting a
gradual 30 pound gain in weight with
development of prominent stria of the skin of
the abdomen and legs. She also had
developed hair growth on her face and chest
and irregularity in her periods. Her blood
pressure is elevated. Each of the following
processes could manifest with this
constellation of symptoms EXCEPT
A. Pituitary adenoma secreting ACTH
B. Neuroendocrine tumor (lung, gut, pancreas)
secreting “ectopic” ACTH
C. Adrenal cortical adenoma secreting cortisol
D. Pheochromocytoma of adrenal medulla
E. High-dose exogenous corticosteroids for
treatment of lupus
17
Answer
A 38-year-old woman with a history of lupus
sought medical attention after noting a
gradual 30 pound gain in weight with
development of prominent stria of the skin of
the abdomen and legs. She also had
developed hair growth on her face and chest
and irregularity in her periods. Her blood
pressure is elevated. Each of the following
processes could manifest with this
constellation of symptoms EXCEPT
A. Pituitary adenoma secreting ACTH
B. Neuroendocrine tumor (lung, gut, pancreas)
secreting “ectopic” ACTH
C. Adrenal cortical adenoma secreting cortisol
D. Pheochromocytoma of adrenal medulla
E. High-dose exogenous corticosteroids for
treatment of lupus
17
Diagnosis: Cushing Syndrome
(Robbins
2010)
(Robbins (Essentials of Rubin’s

2010) Pathology, 5th ed., 2009)
18
Question
Etiologic processes that can manifest with
adrenal Insufficiency include all of the
following EXCEPT
A. Infectious: M. tuberculosis, Mycobacterium
avium intracellulare (AIDS), CMV (AIDS)
B. Inflammatory: autoimmune destruction
C. Vascular: Bilateral hemorrhages in context of
N. meningitides infection, with survival
D. Neoplastic: ectopic ACTH production by
small cell carcinoma
E. Toxic-Metabolic: exogenous corticosteroids,
prolonged
F. Vascular: hemorrhage within pituitary
(apoplexy); necrosis of pituitary after
parturition (Sheehan’s syndrome)
18
Answer
Etiologic processes that can manifest with
adrenal Insufficiency include all of the
following EXCEPT
A. Infectious: M. tuberculosis, Mycobacterium
avium intracellulare (AIDS), CMV (AIDS)
B. Inflammatory: autoimmune destruction
C. Vascular: Bilateral hemorrhages in context of
N. meningitides infection, with survival
D. Neoplastic: ectopic ACTH production by
small cell carcinoma
E. Toxic-Metabolic: exogenous corticosteroids,
prolonged
F. Vascular: hemorrhage within pituitary
(apoplexy); necrosis of pituitary after
parturition (Sheehan’s syndrome)
18
Developmental
Toxic-Metabolic
Inflammatory
Infectious – direct and complications of infection
Toxic-Metabolic
Neoplastic - Metastatic
Mixed: Neoplastic / Inflammatory / Infectious
Toxic-Metabolic
(Robbins
2008)
19
Question
A 14-year-old girl is discovered to have a
blood pressure of 155/90 on a routine school
physical examination. The lesion to the right
identified and resected. Based on the history,
the MOST LIKELY secretory product of this
lesion is
A. Metanephrine
B. Sex hormones
C. Cortisol
D. Aldosterone
E. Adrenocorticotrophic hormone
19
Answer
A 14-year-old girl is discovered to have a
blood pressure of 155/90 on a routine school
physical examination. The lesion to the right
identified and resected. Based on the history,
the MOST LIKELY secretory product of this
lesion is
A. Metanephrine
B. Sex hormones
C. Cortisol
D. Aldosterone
20
Question
A 38-year-old woman with a history of
infiltrating ductal breast carcinoma succumbs
to her disease. The bilateral adrenal glands
are abnormal, with a rim if yellow cortex
discernible but a firm white material present
in the medullary area and outside of the
gland. Which diagnosis do you favor?
A. Mycobacterium infection due to
immunosuppressive treatment of breast
carcinoma
B. Metastatic breast carcinoma
C. Pheochromocytoma
D. Cortical hyperplasia
20
Answer
A 38-year-old woman with a history of
infiltrating ductal breast carcinoma succumbs
to her disease. The bilateral adrenal glands
are abnormal, with a rim if yellow cortex
discernible but a firm white material present
in the medullary area and outside of the
gland. Which diagnosis do you favor?
A. Mycobacterium infection due to
immunosuppressive treatment of breast
carcinoma
B. Metastatic breast carcinoma
C. Pheochromocytoma
D. Cortical hyperplasia
Abnormal. Neoplasm (inside and outside the gland).

Probably breast carcinoma metastasis.
Metastasis to the adrenal glands is fairly common … in

the differential diagnosis.
PolyEndocrine
01
Question
1. Each of the following is a component of
MEN-2A EXCEPT
A. Parathyroid hyperplasia
B. Pancreas islet cell tumor
D. Thyroid C-cell hyperplasia
E. Adrenal pheochromocytoma
01
Answer
MEN-2A EXCEPT
A. Parathyroid hyperplasia
MEN-2A
-P
-T
-A
01


(acromegaly)) - Thyroid - C-cell hyperplasia / medullary
carcinoma(s)
- Parathyroid: - Hyperplasia and - Adrenal - Pheochromocytomas)
- Adenoma(s)
MEN-2B - PTAN - ma
- Pancreas (islet): - Pancreatic Endocrine - Parathyroid
Neoplasm (PEN / islet cell
ganglioneuromas
habitus
02
Question
Thyroid Lesion
MEN-2B EXCEPT
B. Neuromas of mucosa and skin
F. Marfanoid habitus
Calcitonin
02
Answer
Thyroid Lesion
MEN-2B EXCEPT
B. Neuromas of mucosa and skin
F. Marfanoid habitus
MEN-2B
-P
-T
-A
-N
- ma Calcitonin
02


(acromegaly)) - Thyroid - C-cell hyperplasia / medullary
carcinoma(s)
- Parathyroid: - Hyperplasia and - Adrenal - Pheochromocytomas)
- Adenoma(s)
MEN-2B - PTAN - ma
- Pancreas (islet): - Pancreatic Endocrine - Parathyroid
Neoplasm (PEN / islet cell
ganglioneuromas
habitus
03
Question
MEN-1 EXCEPT
Pancreas Lesion
C. Pituitary adenoma
D. Parathyroid hyperplasia
E. Adrenal cortical adenoma
03
Answer
MEN-1 EXCEPT
Pancreas Lesion
C. Pituitary adenoma
D. Parathyroid hyperplasia
E. Adrenal cortical adenoma
MEN-1
- Pituitary: adenoma
- Parathyroid: hyperplasia / adenoma
- Pancreas: islet cell tumor / pancreatic
endocrine neoplasm
04
Question
1. Each of the following is true about multiple
endocrine neoplasia syndromes EXCEPT
A. Tumors occur at younger age than sporadic
tumors
B. In an involved organ, tumors are often
multifocal
C. Asymptomatic stage (e.g. C-cell hyperplasia)
precedes clinically symptomatic stage (e.g.
medullary carcinoma)
D. Have an underlying genetic germ line mutation
E. Tend to be more aggressive than sporadic
tumors
F. Tend to recur in higher proportion of cases
than sporadic tumors
G. Arise in only a single organ in any one patient
04
Answer
1. Each of the following is true about multiple
endocrine neoplasia (MEN) syndromes
EXCEPT
A. Tumors occur at younger age than sporadic
tumors A tumor in one organ
B. In an involved organ, tumors are often (e.g. pheochromocytoma
in right adrenal) may
multifocal occur first
C. Asymptomatic stage (e.g. C-cell hyperplasia) (“metachronously” – at
precedes clinically symptomatic stage (e.g. different times) than other
tumors (e.g.
medullary carcinoma) pheochromocytoma in
D. Have an underlying genetic germ line mutation left adrenal, medullary
thyroid carcinoma) … or
E. Tend to be more aggressive than sporadic tumors in multiple organs
tumors can be detected at the
F. Tend to recur in higher proportion of cases same time
(“synchronously”).
than sporadic tumors
G. Arise in only a single organ in any one
patient
05
Question
1. A patient develops a mass in the left lobe of
her thyroid. Fine needle aspiration and then
resection identifies medullary thyroid
carcinoma. Additional workup reveals a
mass in her left adrenal that was resected,
pictured to the right. Each of the following
statements would be consistent with this
scenario EXCEPT
A. The mass in her adrenal is likely a
pheochromocytoma
B. The neoplastic cells in the thyroid express
calcitonin
C. The patient has features consistent with MEN
I
D. Undisclosed parathyroid hyperplasia could be
present
E. C cell hyperplasia is likely present in thyroid
not affected by neoplasm
05
Answer
1. A patient develops a mass in the left lobe of
her thyroid. Fine needle aspiration and then
resection identifies medullary thyroid
carcinoma. Additional workup reveals a
mass in her left adrenal that was resected,
pictured to the right. Each of the following
statements would be consistent with this
scenario EXCEPT
A. The mass in her adrenal is likely a
pheochromocytoma
B. The neoplastic cells in the thyroid express
calcitonin
C. The patient has features consistent with
MEN I
D. Undisclosed parathyroid hyperplasia could be
present
E. C cell hyperplasia is likely present in thyroid
not affected by neoplasm
Syndromes
01
Question
1. The schematic at right depicts
A. Acromegaly
D. Hyperthyroidism
E. Hypothyroidism
F. Cushing syndrome

01
Answer
A. Acromegaly
D. Hyperthyroidism
E. Hypothyroidism
F. Cushing syndrome

02
Question
A. Acromegaly
D. Hyperthyroidism
E. Hypothyroidism
F. Cushing syndrome

02
Answer
A. Acromegaly
D. Hyperthyroidism
E. Hypothyroidism
F. Cushing syndrome

03
Question
A. Acromegaly
D. Hyperthyroidism
E. Hypothyroidism
F. Cushing syndrome

03
Answer Moans
Psychic

A. Acromegaly
Bones
D. Hyperthyroidism Painful
E. Hypothyroidism
F. Cushing syndrome Groans
G. Diabetes insipidus Abdominal
Stones
Renal

04
Question
A. Acromegaly
D. Hyperthyroidism
E. Hypothyroidism
F. Cushing syndrome

04
Answer
A. Acromegaly
D. Hyperthyroidism
E. Hypothyroidism
F. Cushing syndrome
e.g. HashimOtO
- Mentally slOw
- MyxOedema
- COld intolerance
- Overweight usually / Obese
NOT GravE Disease

- Exophthalmos
- PrE-tibial myxEdema
05
Question
A. Acromegaly
D. Hyperthyroidism
E. Hypothyroidism
F. Cushing syndrome

05
Answer
A. Acromegaly Enlarged
Thyroid =
B. Hyperparathyroidism “Goiter”
D. Hyperthyroidism
E. Hypothyroidism
F. Cushing syndrome
Findings are consistent with Graves disease

variant of hyperthyroidism.
Note: Goiter = enlargement of thyroid. In

Graves disease enlargement represents both
hypertrophy of cells and follicles and
hyperplasia of epithelial cells.

06
Question
A. Acromegaly
D. Hyperthyroidism
E. Hypothyroidism
F. Cushing syndrome

06
Answer
A. Acromegaly
D. Hyperthyroidism
E. Hypothyroidism
F. Cushing syndrome

07
Question
A. Acromegaly
D. Hyperthyroidism
E. Hypothyroidism
F. Cushing syndrome

07
Answer
A. Acromegaly
D. Hyperthyroidism
E. Hypothyroidism
F. Cushing syndrome
Diagram depicts various routes toward
excess systemic cortisol … and then, if
present over a prolonged period of time,
Cushing syndrome, including exogenous
corticosteroids.
Cushing DISEASE is the result of excess

ACTH secretion the (“Da”) Pituitary.
Disease is a subset of the all-encompassing

SYNDROME. (Essentials of Rubin’s Pathology, 5th ed., 2009)
08
Question
A. Acromegaly
D. Hyperthyroidism
E. Hypothyroidism
F. Cushing syndrome

08
Answer
A. Acromegaly
D. Hyperthyroidism
E. Hypothyroidism
F. Cushing syndrome
Diagram depicts various etiologies of
diabetes insipidus (DI) including trauma
(disruption of infundibulum in traumatic
incident or due to surgical trauma) and
neoplasm (pituitary or hypothalamic) in
addition to familial / developmental causes.

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Endocrine Pathology

Control – L full screen

• 2 Study Files for Examination

A. Parathyroid - Posterior Lobe

Anterior: Nests of neuroendocrine cells;

Anterior: Nests of neuroendocrine cells;

Intermediate Anterior Pituitary

Clarification re. Nomenclature based on Size

(Extracted from Dr. DeMasters’ PowerPoint File)

Expect gland formation

Pituitary Apoplexy- Defined

The lesion in this patient’s

The lesion in this patient’s

Symptoms / Signs / Imaging / Biopsy / Laboratory Analysis

(Extracted from Dr. DeMasters’ PowerPoint File)

Double- Lobed organ

Thyroid Gland Histology

Colloid - Scalloped (untreated - Reduced volume

Long-Term - Progression - Fibrosis / end stage

The most likely ongoing etiologic

The most likely ongoing etiologic

The best diagnosis in this case is:

The best diagnosis in this case is:

Colloid - Scalloped (untreated - Reduced volume

Long-Term - Progression - Fibrosis / end stage

A. Neoplasm of C / parafollicular cells

A. Neoplasm of C / parafollicular cells

While confusing by nomenclature, the diagnosis

Antithyroglobulin antibodies (destroy!!) are characteristic of

Anti-TSH receptor antibodies (stimulate!!) are

Antithyroglobulin antibodies (destroy!!) are

The diagnosis of “papillary carcinoma” is

Anaplastic Thyroid Carcinoma:

Iodine deficiency-related Goiters can

A. Adenoma Right Lobe

A. Adenoma Right Lobe

• “Goiters” most commonly form when

“Colloid Nodule”: “Colloid Nodule”: Degenerative / Traumatic

Multiple Endocrine Neoplasia

MEN-1 - Wermer: PPP-D MEN-2: PTA-N-MA

- Pituitary: - Adenoma (usually MEN-2A – PTA = Sipple Syndrome

- Pancreas (islet): - Pancreatic Endocrine

A. C-cell hyperplasia Cell adenoma

C. Medullary thyroid carcinoma

Macroscopic / Microscopic Challenge:

Macroscopic / Microscopic Challenge:

See next slide

Organ Endocrine / Neuroendocrine Cells

Oxyphil Cell 1. “None:” -

Adrenal Cortex Pituitary Thyroid C-Cells

Parathyroid carcinoma is rare.

It is defined by biological aggressiveness: Patient’s Control

Enlargement of all four glands is expected, likely variably enlarged but

A single enlarged gland would be highly unlikely in this setting.

A 65-year-old man has end-

A 65-year-old man has end-

Parathyroid hyperplasia is noted in the photograph of a parathyroid

NOTE: A pathologist CANNOT distinguish