Pituitary Pathology - SRS Flashcards

1
Q

What are the key cells of the anterior pitutitary?

What do each produce?

A
  1. Somatotrophs, producing growth hormone (GH)
  2. Mammosomatotrophs, producing GH and prolactin (PRL)
  3. Lactotrophs, producing PRL
  4. Corticotrophs, producing adrenocorticotropic hormone (ACTH) and pro-opiomelanocortin (POMC), melanocyte-stimulating hormone (MSH)
  5. Thyrotrophs, producing thyroid-stimulating hormone (TSH), and
  6. Gonadotrophs, producing follicle-stimulating hormone (FSH) and luteinizing hormone (LH).
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2
Q

What hormones does the posterior pituitary produce?

A

Oxytocin and antidiuretic hormone (ADH, also called vasopressin)

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3
Q

You notice that your nipples are leaking, and you suddenly find that your visual field looks like the image attached.

What is going on?

A

Prolactinoma - causing galactorrhea through prolactin secretion, and bitemporal hemianopia through mass effect on the optic chiasm,

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4
Q

What is shown here?

A

Papilledema

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5
Q

What should you use to identify a pituitary adenoma?

A

MRI

(X-ray will just show degredation of the sella turcica)

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6
Q

What is the most common cause of hyperpituititarism?

A

Adenoma arising in the anterior lobe

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7
Q

What whould a patient with a lactotroph adenoma likely present with?

A

Galactorrhea

Amenorrhea (in females)

Sexual dysfunction (impotence)

Infertility

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8
Q

Pituitary adenomas are usually found in adults and the peak incidence is from 35 to 60 years of age. What is the size cut off which differentiates microadenomae from macroadenomae?

A

Microadenomas if they are less than 1 cm in diameter and macroadenomas if they exceed 1 cm in diameter

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9
Q

What is the most common activating mutation associated with the condition seen in this patient

A

GNAS

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10
Q

Is a functional pituitary adenoma more likely to be large or small?

A

Small. Large more commonly will be silent hormonally but present with mass effects.

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11
Q

Attached is a sample of a pituitary adenoma. Describe the findings seen here in the classic way we will see in exams!

A

Monomorphic (monotonous) cell appearance and an absence of the reticulin network.

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12
Q

What is the most common type of functioning adenoma of the pituitary?

A

Prolactin-secreting lactotroph adenomas

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13
Q

On EM of a lactotroph adenoma (or really any other pituitary adenoma that is functional) what will you see?

A

Dense core granules (as is common with neuroendocrine tumors)

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14
Q

What are four examples of mass effects that a pituitary adenoma may present with?

A
  1. Radiographic abnormalities of the sella turcica
  2. visual field abnormalities
  3. elevated intracranial pressure (papilledema)
  4. hypopituitarism (less common)
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15
Q

What is sometimes associated with acute hemorrhage into an adenoma?

A

Pituitary apoplexy

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16
Q

In what patient group is a prolactinoma more readily identified?

A

Females, d/t amenorrhea

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17
Q

Somatotroph adenomas can secrete GH, what determines the phenotype observed?

A

Age at which the tumor develops

If as a child, prior to closure of the epiphyseal plate, will present with gigantism.

If as an adult after closure of the plates, will see acromegaly.

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18
Q

What impact do the persistently elevated levels of GH have on the liver?

A

Stimulates hepatic secretion of insulin-like growth factor (IGF-1).

19
Q

What happens to a patient’s bone density with a somatotrophic adenoma?

A

Bone density increases

20
Q

If a patient has pronounced prognathism, and claw/sausage like fingers then they may have?

A

Acromegaly d/t a GH secreting pituitary adenoma.

21
Q

What are three ways we can test for somatotroph adenomas?

Which is the most sensitive?

A
  1. failure to suppress GH production in response to an oral load of glucose is one of the most sensitive tests for acromegaly
  2. elevated serum GH
  3. Elevated serum IGF-1
22
Q

Excess production of ACTH by functioning corticotroph adenomas leads to adrenal hypersecretion of cortisol and the development of hypercortisolism, also known as?

A

Cushing Disease

23
Q

What is Nelson syndrome and how does it arise

A
  • Post surgical removal of the adrenal glands, the development of a large destructive pituitary adenoma is known as Nelson syndrome
  • most commonly d/t the loss of corticosteroids on a preexisting corticotroph microadenoma.
24
Q

What would the skin of a patient with Nelson syndrome look like?

A

Hyperpigmented, d/t the impact of ACTH on melanocytes.

25
Q

How are gonadotroph adenomas frequently recognized?

A

Typically d/t mass effects, as they do not typically secrete hormones and a distinct clinical syndrome is uncommon.

Also, it may cause other pituitary hormone deficiencies - most commonly impaired secretion of LH = decreased energy and libido in men, and amenorrhea in premenopausal women.

26
Q

Nonfunctioning pituitary adenomas are a heterogeneous group that constitutes approximately 25% to 30% of all pituitary tumors. Their lineage can be established by immunohistochemical staining for hormones or by biochemical demonstration of cell type-specific transcription factors.

What have these been referred to in the past as?

What symptoms do these typically present with?

A

Silent variants or Null-cell adenomas

Mass effects

27
Q

In addition to gigantism and acromegaly, what is another significant finding associated with somatotroph adenomas?

A

Impaired glucose tolerance and diabetes mellitus

28
Q

What does a corticotroph adenoma secrete?

How does it present?

A

ACTH - leads to Cushing syndrome and hyperpigmentation

29
Q

What are some clinical manifestations of hypopituitarism? 6

A
  1. Pituitary Dwarfism - GH deficiency
  2. amenorrhea and infertility - LH and FSH deficiency
  3. Hypothyroidism - TSH deficiency
  4. Hypoadrenalism - ACTH deficiency
  5. Failed postpartum lactation - prolactin deficiency
  6. Pallor - Loss of MSH (melanocyte stimulating hormone)
30
Q

What are two important posterior pituitary syndromes we covered?

A

Diabetes insipidus (Central)

Syndrome of inappropriate ADH (SIADH)

31
Q

What goes down in central diabetes insipidus?

A

ADH deficiency causes this condition, which is characterized by excessive urination (polyuria) d/t an inability of the kidney to resorb water properly from the urine.

32
Q

What goes down in SIADH?

A

ADH Excess causes resorption of excessive amounts of free water resulting in hyponatremia

33
Q

What type of cancer is associated with SIADH?

Where is it found? be specific

A

Small cell carcinoma of the lung

found in the central lung regions

34
Q

What size are the nuclei in small cell carcinoma?

A

Large

35
Q

Characterize the following for Diabetes insipidus…

  1. Urine volume
  2. Urine specific gravity
  3. Serum sodium
  4. Serum osmolality
A
  1. Urine volume increased substantially
  2. specific gravity lower than normal
  3. Serum sodium increased
  4. Serum osmolality increased
36
Q

Histology of the image shown is described in the path report as demonstrating characteristic compact, lamellar, “wet” keratin and cords of squamous epithelium with peripheral palisading.

Based on the image and description, what do you suspect is going on?

In what population is this most common?

A

Adamtinomatous craniopharyngioma

Most often observed in children

37
Q

What are the two most commonly implicated hypothalamic suprasellar tumors?

A

Gliomas

Craniopharyngiomas

38
Q

What are craniopharyngiomas thought to arise from?

A

Vestigial remnants of Rathke’s pouch

39
Q

What type of craniopharyngioma is typical in children?

Adults?

A

Children = Adamantinomatous Craniopharyngioma

Adults = Papillary craniopharyngioma

40
Q

Describe the findings shown in the attached image taken from a hypothalamic suprasellar tumor as if you were a pathologist who knows what is going on!

A

Adimantinomatous craniopharyngioma with characteristic compact, lamellar “wet” keratin (Right half of photo) and cords of squamous epithelium with peripheral palisading on the left.

41
Q

What are two classic findings associated with craniopharyngiomas?

A

Crankcase oil - cholesterol rich, thick brownish yellow fluid.

dystrophic calcification

42
Q

What is the prognosis of a patient with a craniopharyngioma?

A

Excellent - recurrance free and overall survival, especially with those less than 5 cm. Even the larger ones have good outcomes though.

43
Q
A