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Endocrine and Reproductive Systems > P- Pituitary and Sellar Region > Flashcards

P- Pituitary and Sellar Region Flashcards

1
Q

What percent of intracranial neoplasms are pituitary adenomas?
What age people are affected?
What is the most common hormone secreted?

A

10-15% of intracranial neoplasms are pituitary adenomas.

98% occur in adults.

The most common hormone producing adenoma is one that secretes prolactin.

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2
Q

Patients with pituitary adenomas can present in what 2 ways?

A
  1. symptoms of mass effect (headache, visual disturbance, loss of normal pituitary function due to compression of the native gland)
  2. symptoms of hormone excess
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3
Q

What are the 3 most common “mass effects” caused by pituitary tumors?
Which is most common?

A
  1. headache
  2. visual deficiency due to compression of optic chiasm [more common than LOF]
  3. LOF due to compression of the pituitary gland [insidious onset]
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4
Q

In addition to compression of the normal gland, what other structures can be invaded by pituitary adenomas?

A
  1. upward –> brain
  2. lateral –> cavernous sinuses
  3. downward–> paranasal sinuses/nasal cavity
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5
Q

A young woman presents with amenorrhea and galactorrhea. What adenoma are you concerned about?

A

Lactotroph cell adenoma (secretes prolactin)

*This is the most common hormone producing adenoma

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6
Q

What adenoma are you concerned about if a younger patient presents with gigantism or an older patient presents with acromegaly?

A

Somatotroph cell adenoma (GH)

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7
Q

What adenoma are you concerned with if the patient has signs/symptoms of Cushing’s disease?

A

Corticotroph cell adenoma (ACTH)

*small and difficult to find if hormonally active

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8
Q

What is the most common presenting manifestation of thyrotroph cell adenoma?

A

hyperthyroidism (however this is a rare cause)

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9
Q

What is the most common presenting manifestation of gonadotroph cell adenoma?

A

Mass effect

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10
Q

What is the most common presenting manifestation of null cell adenoma?

A

Mass effect [may in fact be FSH/LH tumors]

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11
Q

What are the 2 most common combinations of mixed adenoma (plurihumoral)?

A
  1. GH/prolactin

2. GH/prolactin/TSH

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12
Q

What is the difference between microadenoma, macroadenoma and giant?

A 
Micro = less than 10mm diameter
Macro = 10mm to 4cm
Giant = over 4cm
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13
Q

Describe the histology of a pituitary adenoma.

A
  1. small columnar/cuboidal/polygonal cells
  2. monomorphic [all cells look the same]
  3. round/oval nuclei [2x size of lymphocyte]
  4. “salt and pepper” chromatin [neuroendocrine]

**mitotic figures are UNCOMMON

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14
Q

What is the age at which prolactin-producing adenomas are likely to occur?
What determines the degree of hyperprolactinemia?

A

20-40 years old

Hyperprolactinemia varies with tumor size.

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15
Q

How do prolactin-producing adenomas differ between men and women?

A

Women:

  • get tumors earlier
  • microadenomas
  • lower serum prolactin [400 ng/ml]
  • amenorrhea, galactorrhea

Men

  • get tumors later
  • macroadenomas
  • higher serum prolactin [2000 ng/ml]
  • decreased libido, sexual impotence
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16
Q

What is the effect of prolactin-producing adenoma on prepubertal girls and boys? Pubertal?

A

Prepubertal children have symptoms of mass effect:

  1. headache
  2. visual disturbance
  3. growth failure

Pubertal Girls:

  1. mass effect
  2. menstrual abnormalities/pubertal delay
  3. galactorrhea

Pubertal Boys:

  1. mass effect
  2. growth arrest
  3. pubertal delay
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17
Q

Why does compression of the pituitary stalk lead to elevated serum prolactin? How much does serum prolactin usually increase by?

What is this phenomenon called?

A

Dopamine is secreted from PVN and arcuate nucleus of the hypothalamus into the pituitary gland to inhibit secretion of prolactin.

If the pituitary stalk is compressed by a neoplastic or non-neoplastic mass, dopamine will not get to the pituitary gland.

Prolactin will be increased but below 100 ng/ml.

This is called “stalk effect”.

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18
Q

In general, a prolactin level over ______ indicates an adenoma.

A

250 ng/ml

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19
Q

Describe prolactin-producing adenomas:

  1. on imaging
  2. grossly
  3. histologically
A

Imaging: microadenoma
Grossly: soft, tan-red mass

Histologically:

  1. monomorphic
  2. round-to-oval nuclei [sometimes elongated, pleomorphic]
  3. architecture is “sheet-like”
  4. densly OR sparsely granulated
  5. psammoma bodies [lamillated calcific structure]
  6. interstitial deposits of amyloid
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20
Q

What genetic component is associated with prolactin-producing adenomas?

A

Most are sporadic, however, there has been a link to MEN-1 [where they are the most common pituitary adenoma type]

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21
Q

What is treatment for prolactin-producing adenomas?

A

Dopamine agonists because they:

  1. reduce tumor size
  2. lower serum prolactin concentration
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22
Q

GH producing adenomas are composed of cells differentiating as ________________________. This excess GH can lead to _________ in children and _________ in adults.

A

adenohypophyseal somatotrophs

Children –> gigantism
Adults –> acromegaly

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23
Q

What is acromegaly?

A
  • Enlargement of hands, feet due to soft tissue hypertrophy.
  • Coarse facial features
  • large tongue/uvula [leads to sleep apnea]
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24
Q

What signs/symptoms are associated with:

  1. mass effect of GH-producing adenoma [3]
  2. excess GH/IGF-1 effect of adenoma [4]
A

Mass Effect:

  1. visual symptoms due to impinging on optic chiasm
  2. hydrocephalus [3rd ventricle]
  3. ophthalmoplegia [invaded cavernous sinus]

Excess GH/IGF1:

  1. gigantism/acromegaly
  2. obstructive sleep apnea (due to enlarged tongue/uvula)
  3. peripheral arthropathy (articular cartilage overgrowth)
  4. LV hypertrophy –> arrhythmia
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25
Q

How is the laboratory diagnosis of GH- producing adenoma made?
What is treatment?

A

Diagnosis:
demonstrating elevated IGF-1 in the plasma

Treatment:

  1. surgery
  2. SST analogs—-I GH secretion
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26
Q

What are the 3 ways GH secreting adenomas are classified?

A
  1. Entirely somatotrophs - classify by the density of their secretory granules
  2. Mammosomatotroph - individual cells produce BOTH GH and prolactin
  3. Mixed GH-prolactin adenomas
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27
Q

How do densely granulated somatotrophs differ histologically from sparsely granulated?

A

Dense:

  1. columnar/cuboidal
  2. monomorphic
  3. round-to-oval nuclei
  4. abundant cytoplasmic granules

Sparse:

  1. pleomorphic nuclei
  2. fewer granules
  3. paranuclear keratin filaments (fibrous bodies)
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28
Q

What genetics are associated with GH secreting adenomas?

A

Most occur sporadically, howevern some occur as a component of MEN-1.

MEN1 - 0-37%
Carney’s complex - 10-20%

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29
Q

ACTH producing adenomas are neoplasms composed of cells differentiating as ____________________. The neoplastic cells make ________ in excess.

A

Adenohypophyseal corticotrophs which make excess proopiomelanocortin (POMC).

POMC is a peptide from which ACTH is cleaved.

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30
Q

What age group and sex are ACTH secreting adenomas more common in?

A

Women (8:1)

30-40 years old

31
Q

What is the difference between Cushing’s disease and Cushing’s syndrome?

How can you clinically determine the difference?

A

Disease = the constellation of symptoms due to production of excess ACTH by a pituitary adenoma

Syndrome= constellation of symptoms due to excess cortisol by a NON-PITUITARY SOURCE [such as an adrenal tumor].

The location of the excess can be determined by tested based on the negative feedback control of cortisol.
Pituitary problem will have high serum ACTH
Adrenal problem/anywhere else will have low ACTH because the cortisol will have negatively fed-back on the H-P axis

32
Q

What are the 6 major symptoms of Cushing’s?

A
  1. excess fat in abdomen, neck, supraclavicular
  2. thinning of skin
  3. purple stria on chest/abdomen
  4. insulin resistance
  5. immunocompromised/susceptible to infection
  6. neuropsychiatric symptoms [depression, sleeplessness, emotionally labile]
33
Q

How is the laboratory diagnosis of Cushing’s syndrome established?

A
  1. measuring total cortisol in the urine/24hrs

2. demonstrating loss of circadian rhythm of cortisol secretion

34
Q

What is seen on imaging, grossly, and histologically for ACTH secreting adenomas?

A

Imaging: 80% microadenoma, 5% macro, 15% below detection of imaging

Gross: soft and red. SO SMALL that they are difficult for surgeons to find.

Histology:

  1. monomorphic cells
  2. round-to-oval nuclei
  3. abundant basophilic cytoplasm
  4. immunohistochemically stains for ACTH
35
Q

What is a “silent” ACTH adenoma? How does the clinical course compare to other ACTH adenomas?

A

It is when a pituitary adenoma has immunohistochemically stained for ACTH but there is no clinical presentation/biological signs of hypercortisolism.

These tumors have a MORE aggressive clinical course.

36
Q

What are the genetic components of ACTH secreting adenomas?

A

They are mostly sporadic, but they can occur as a component of MEN-1.

37
Q

What is the definition of a “null cell adenoma”?

A

It is a pituitary adenoma with cells differentiating as adenohypophyseal cells with NO immunohistochemical differentiation.

[rarely, they will express FSH or LH]

38
Q

What gender and age group are null cell adenomas most common in?

A

There is no gender specificity.

Null cell adenomas are common in OLDER patients and do not occur younger than 40

39
Q

Null cell adenomas show no signs/symptoms related to excess hormone secretion except for what?
What symptoms are shown will null cell adenomas?

A

Prolactin could be elevated due to stalk effect [loss of dopamine inhibition of prolactin secreting cells]

Mass effect symptoms:

  • headache
  • visual disturbances
  • loss of normal pituitary function
40
Q

How do null cell adenomas appear on imaging? Grossly?

Histologically?

A

Imaging: large and invade cavernous sinuses or extend into hypothalamus or nasal cavity. They may contain CYSTS filled with hemorrhage or clear fluid

Gross: yellow-tan, soft

Histologically:

  1. columnar, polygonal
  2. round/oval nuclei
  3. sheet-like architecture
41
Q

What is the genetic association of null cell adenoma?

A

There is none.

[no definite association with MEN-1 or Carney’s complex]

42
Q

What is pituitary apoplexy?

What kind of tumor does it occur most often with?

A

Sudden enlargement of an adenoma due to hemorrhage, often with accompanying infarction.

Occurs most frequently with large, non-secreting adenomas [like null cell]

43
Q

A patient presents with subarachnoid hemorrhage, increased intracranial pressure, headache, visual symptoms, and worsened hypopituitarism. What is the likely cause?

A

Pituitary apoplexy

44
Q

What are the 5 symptoms of pituitary apoplexy?

A
  1. subarachnoid hemorrhage
  2. hypopituitarism
  3. visual disturbance
  4. increased intracranial pressure
  5. headache
45
Q

What is a pituicytoma?

What part of the pituitary gland does it affect?

A

It is a neoplasm composed of neurohypophyseal pituicytes in the posterior pituitary.

Pituicytes are supporting glial cells of the post. pituitary.

46
Q

What grade are pituicytomas?

What age patient is mostly affected by them?

A

WHO 1 = non-infiltrative, expanding mass

Adults 40-60 are most affected

47
Q

How do pituicytomas look on imaging, grossly, and histologically?

A

Imaging: well-circumscribed ANYWHERE in the posterior pituitary so can be suprasellar or intrasellar

Gross: tan and rubbery

Histologically:

  • elongated, bipolar glial cells
  • monomorphic eliptical nuclei
  • architecture is intersecting fascicles
48
Q

What are the 3 pituitary/sellar neoplasms most frequently found in children?

A
  1. craniopharyngioma
  2. pilocytic astrocytoma
  3. germ cell tumors
49
Q

What are the 3 most common sellar region neoplasms in adults?

A
  1. pituitary adenoma
  2. meningioma
  3. craniopharyngioma
50
Q

What cells make up craniopharyngiomas? How do they develop?

What are the two histologic variants?

A

Craniopharyngiomas are squamous epithelial tumors of the sellar region that develop due to proliferation of cells in Rathke’s pouch.

  1. papillary [only 40-55]
  2. adamantinomatous {more common 5-15 and 45-60}
51
Q

What is the age distribution for craniopharyngiomas?

What histologic type is present in each age group?

A 
5-15 = adamantinonmatous
45-60 = adamantinomatous

40-55 = papillary

52
Q

What is the grade of craniopharyngiomas?

A

WHO 1 = well differentiated, non-invasive, expanding mass

53
Q

What are the signs and symptoms of craniopharyngioma in adults? Children?
Who is more likely to get diabetes insipitus?
When would elevated intracranial pressure present?

A

Adults = visual disturbances

Children = endocrine disturbances

Diabetes insipitus in 15% kids, 30% adults

Elevated intracranial pressure if the third ventricle is involved

54
Q

What is seen on imaging for craniopharyngiomas?

A

There will be well-circumscribed masses usually in the suprasellar region

55
Q

What is seen grossly for craniopharyngiomas?

How do adamantinomatous and papillary differ?

A

Adamantinomatous have variegated with:

  1. cystic areas - dark greenish-brown fluid
  2. fibrotic areas
  3. calcification
  4. cells clustered near vessels or nerves or brain

Papillary do NOT have cysts or calcifications

56
Q

Describe the histology of adamantinomatous variant of craniopharyngioma.

How does it differ from papillary?

A
  1. basaloid cells at interface with brain
  2. loose connective tissue
  3. cords/lobules of well-differentiated squamous epithelial cells
  4. nodules of keratin
  5. cysts fill with squamous debris

Papillary does NOT have basaloid layer or nodules of keratin

57
Q

What mutations are associated with craniopharyngiomas?

A

Adamantinomatous are associated with B-catenin mutations.

Papillary are not associated with mutations

58
Q

What is the prognosis of craniopharyngiomas?

What is the key factor for recurrence?

A

10 year recurrence free and 10 year survival are 60-90%

The key factor for recurrence is the extent of resection [tumors >5cm have worse prognosis]

59
Q

What is the most common glioma in children? What is the grade of this tumor?
What is the age and sex distribution?

A

Pilocytic astrocytoma - slow growing astrocytoma

WHO1 = well-circumscribed, non-infiltrative, space expanding

Less than 20years old with no sex preference.

60
Q

Where are pilocytic astrocytomas located?

A

Throughout the CNS most frequently in:

  1. cerebellum
  2. thalamus, hypothalamus, optic pathways
61
Q

A child presents with headache, nausea, vomiting and clumsiness. What tumor are you concerned about and in what location?

A

Pilocytic astrocytoma in the cerebellum

62
Q

If a child patient presents with hemiparesis, what tumor are you concerned about and what location would it be in?

A

Pilocytic astroctroma in the thalamic region because the hemiparesis is from impingement on internal capsule

63
Q

How do pilocytic astrocytomas appear on imaging?

A
  1. well-circumscribed
  2. contrast enhancing
  3. cystic
64
Q

How do pilocytic astrocytomas look grossly? Histologically?

A

Grossly - well demarcated grey, soft, tan, cystic

Histologically:

  1. astrocytic cells have hair-like cytoplasmic processes
  2. biphasic architecture with:
    - packed, overlapping bipolar astrocytes
    - loosely arranged stellate astrocytes
  3. Rosenthal fibers (eosinophilic corkscrew)
  4. eosinophilic granular bodies
65
Q

What genetics are associated with pilocytic astrocytomas?

A
  1. 15% of patients with NF1 (neurofibromatosis type 1) develop pilocytic astrocytoma in the optic nerves
  2. Sporadic = loss of 17q (NF1 suppressor gene)
66
Q

What determines prognosis for pilocytic astrocytomas?

A
  1. location
  2. extent of resection
  3. presence/absence of NF1
67
Q

Where are Rathke’s cleft cysts found?

What size must they be to become symptomatic?

A

It is located between the anterior and posterior pituitary.

If the cyst is >1cm it can extend into the sella and suprasellar region and become symptomatic

68
Q

Describe the histology of Rathke’s cysts.

A
  1. single layer of columnar epithelium on thin layer of collagenous connective tissue
  2. goblet cells
  3. ciliated cells
  4. cells surround watery/mucoid material

[can have squamous metaplasia! the opp. of Barrett’s]

69
Q

What is lymphocytic hypophysitis?

A

Inflammatory infiltrate in the anterior pituitiary as a result of an autoimmune disorder.
It leads to pancreatic insufficiency.

70
Q

Who is usually affected by lymphocytic hypophysitis?

A

women in late pregnancy and sometimes after delivery.

71
Q

How does lymphocytic hypophysitis appear grossly and microscopically?

A

Gross: areas of the gland affected are firm

Histologic:

  1. B and T lymphocytes
  2. plasma cells
  3. histiocytes
  4. lymphoid follicles with germinal centers
72
Q

What other endocrine organs are frequently affected in lymphocytic hypophysitis?

A
  1. adrenal gland

2. thyroid

73
Q

What is treatment for lymphocytic hypophysitis?

A
  1. corticosteroids
  2. hormone replacement
  3. surgical decompression of the pituitary gland

Endocrine and Reproductive Systems (27 decks)

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