pituitary adenoma, prolactinoma, empty sella Flashcards

1
Q

pituitary disorders

A

-hypothalamic-pituitary lesions
-pituitary adenoma/tumors
-prolactinoma
-empty sella syndrome

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2
Q

hypothalamic-pituitary lesions presentation

A

Patient presentation:
-Headaches
-Visual field defects
-Bitemporal hemianopia-lateral vision loss -> due to size of tumor
-Hemifield slide phenomenon [images drifting apart]
-Altered appetite
-Thirst

Imaging evidence of a mass lesion as an incidental finding
-Hypersecretion or
-Hyposecretion of one or more pituitary hormones (compression)

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3
Q

pituitary or hypothalamic tumor

A

-MC cause of hypopituitary or hyperpituitary secretion is pituitary or hypothalamic tumor
-Pituitary tumor tends to produce an enlarged sella turcica (erodes the bone)
-Enlarged sella may represent empty sella syndrome (increase CSF -> makes it look empty but its not)- usually asymptomatic

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4
Q

pituitary adenomas

A

-Benign neoplasms of the anterior pituitary

Symptomatic because:
-Due to excess secretion of pituitary hormones
-Dysfunction in the compressed areas of the pituitary
-Due to mass effect of the tumor impinging on local structures

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5
Q

pituitary adenomas: classification

A

Macroadenoma: > 10 mm
- Sx: usually due to mass effects - headaches, visual field defects

Microadenoma: < 10 mm
- Sx: usually due to effects of aberrant hormone levels

Secretory tumors:
-Prolactin-secreting adenomas
-GH secreting adenomas
-Corticotrophin-secreting adenomas
-Thyrotropin-secreting adenomas

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6
Q

Therapy for pituitary adenomas:

A

Transphenoidal surgery
Pituitary radiation
Pharmacotherapy

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7
Q

Nonfunctional adenomas OR non-secreting adenomas

A

*Generally present as MACROadenomas with headache, visual disturbances, or hypopituitarism

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8
Q

corticotropin secreting pituitary adenoma

A

-Round face, truncal obesity, and a dorsocervical fat pad (buffalo hump)
-Hirsutism (excess hair growth)
-acne, striae, thin skin, bruising
-Menstrual disorders
-Hypertension and hyperglycemia

aka: cushing ds
Diagnostic Test: Dexamethasone Suppression Test
- Normal: Cortisol decreases with Dexamethasone

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9
Q

pituitary adenomas: thyrotropin-secreting pituitary adenoma

A

-Produces thyrotoxicosis
-Goiter
-Visual impairment

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10
Q

pituitary adenomas: GH secreting pituitary adenoma

A

Mostly MACROadenomas with little or no clinical effect

-May present with visual field deficits
-Gigantism or acromegaly
-Hypertension
-Hyperglycemia
-Osteoarthritis
-Signs of hypopituitarism ±

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11
Q

symptoms: macroadenomas vs microadenomas

A

Macroadenomas sx: due to mass effect
- compression of local structures -> headache and visual field deficits

Microadenomas: due to effects of aberrant hormone levels
-Patient may report infertility, impotence, or dyspareunia (painful intercourse due to decreased vaginal secretions; low FSH, LH)
-Hypopituitarism and hypogonadism due to destruction of pituitary gland
-Pathologic fractures due to osteoporosis

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12
Q

With macroadenoma examine for signs of: _________ such as

A

Examine for signs of hypopituitarism:
-Pallor
-Hypotension
-Visual field defects
-Hypogonadism
-Features of osteoporosis

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13
Q

Diagnosing pituitary adenomas

A

-Clinical presentation must be consistent with a syndrome caused by a pituitary tumor
-Blood tests and MRI
-Pregnancy test: prolactinomas elevate prolactin levels -> galactorrhea

-suppress ovulation
-> first thing you think is pregnancy then tumor

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14
Q

treatment of goals of adenomas

A

Eliminate (effects due to mass of tumor, w radiation for ex)

Reduce (elevated hormone levels)

Ameliorate (end organ effects of elevated pituitary hormone levels)

Avoid (Damage to remaining normal hypothalamic/ pituitary function)

Minimize- potential adverse effects of therapy

“ERA AM”

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15
Q

What are MEN syndromes?

A

inherited as autosomal dominant traits that cause a predisposition to the development of tumors of two or more different endocrine glands
-Genetic testing should be done

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16
Q

What are the men syndrome classifications

A

MEN 1:
-parathyroid glands
-pancreas
- pituitary
MEN 2 (MEN 2A):
- medullary thyroid cancers
- pheochromocytoma
- Hirschsprung disease
MEN 3
MEN 4

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17
Q

multiple endocrine neoplasia (MEN) 1

A

Autosomal dominant syndrome
-characterized by a genetic predisposition to parathyroid, pancreatic, and pituitary adenomas (PPP)
-About 1/2 of affected pts develop prolactinomas*
- Acromegaly and Cushing’s are less commonly encountered

18
Q

What syndromes are associated with MEN1 tumors?

A

Carney syndrome
- spotty skin pigmentation
- myxomas,
- endocrine tumors: testicular, adrenal, and pituitary adenomas
- Acromegaly – 20%

McCune-Albright syndrome
-Polyostotic fibrous dysplasia
- pigmented skin patches
- endocrine disorders: GH-secreting pituitary tumors, adrenal adenomas, and autonomous ovarian function

Familial acromegaly:
-rare

19
Q

pituitary metastases

A

Occur in ~3% of cancer pts
-Blood-borne metastatic deposits ALWAYS prefer posterior pituitary*
- 50% of pituitary metastases = breast cancer

20
Q

MRI dx of metastatic lesion may be difficult to distinguish from _________. what test do you need to perform?

A

-MRI dx of metastatic lesion may be difficult to distinguish from:
- aggressive pituitary adenoma

Need to do:
- tissue bx: histologic examination

21
Q

Rarely, pituitary stalk involvement in metastases results in _________.

A

anterior pituitary insufficiency

22
Q

presenting feature of lung, GI, breast, and other pituitary metastases

A

-diabetes insipidus: posterior pituitary metastases

23
Q

prolactinoma description

A

Intrasellar tumor of the anterior part of the pituitary gland, which secretes prolactin
- MC pituitary tumor

24
Q

Prolactinoma tx

A

Treatment depends on size and encroachment on optic chiasm

Options for tx:
-Medical, surgical, or radio-therapeutic
-Dopamine agonists - preferred option *
-Recurrence rates are high with surgery

25
Q

prolactinoma: men vs women presentation

A

Women: EARLY presentation
- galactorrhea
-absent periods
- Infertility
- Galactorrhea
- Hypopituitarism (rare)
- MICROadenoma (common)
- APATHY*

Men present later -> when tumor is large -> different tx
-Hypogonadism: Decreased libido, ED, Infertility, Decreased body/beard hair
-Fatty breast enlargement
- Galactorrhea
- Neuro effects- ICP
- VISUAL**OPTIC NERVE COMPRESSION= Bitemporal hemianopsia, blurring
-usually MACROadenoma
- APATHY*

26
Q

Signs of Prolactinoma

A

Apathy**
- Prolactinoma is antagonist of dopamine
Ophthalmoplegia = Squint
- May also present with double vision
May cause hemorrhage into tumor = Hypopituitarism
May cause seizures if tumor extend into temporal lobe

27
Q

prolactinoma etiology

A

-hypothalamic disorders*
-pharmacologic causes*
-amphetamines, nicotine, TCA, verapamil, estrogens**
-physiologic
-nipple stimulation/suckling
-pregnancy
-postpartum period
-stress
-food ingestion
-sexual intercourse in some women
-sleep
- hypoglycemia
-hypothalamic tumors
-infiltration- sarcoidosis, TB, langerhans cell histiocytosis (Hand-Schüller-Chrisitan disease)
-post-encephalitis
-idiopathic galactorrhea
-head trauma
-other endocrine disrodres
-acromegaly
-cushings
-primary hypothyroidism
-disorders of other systems
-chronic renal failure
-liver disease
-ectopic production of prolactin- bronchogenic carcinoma (mostly small cell undifferentiated)
-hypernephroma

28
Q

visual fields

A

-Screening technique for detection of lesions in the anterior and posterior visual pathway.
-Position yourself about an arm’s length away from the patient.
-Place your hands about 2 feet apart out of the patient’s view, roughly lateral to the patient’s ears.
-While in this position, wiggle your fingers and slowly bring your moving fingers forward into the patient’s center of view.
-Ask the patient to tell you as soon as he or she sees your finger movement.
-Test each clock hour, or at least each quadrant.
-Note any abnormal “field cuts”
-Kinetic red target test

29
Q

With prolactinoma, what are the signs of prolonged estrogen deficiency in women….

A

atrophic vaginitis
thinning of pubic hair
loss of labial fat pads
reduction in breast size

30
Q

What tests should be checked in prolactinoma?

A

Check Medications!!!*
TSH*
Serum prolactin levels
-Normal <20 mcg/L
- Need several sample to ascertain diagnosis
- Single sample can be diagnostic if >200 mcg/L

Imaging: MRI

31
Q

prolactinoma- indications for tx

A

Indications for tx of a prolactinoma are:
-restoration of fertility
- presence of a macroadenoma

32
Q

Clinical TX of choice for prolactinoma

A

Dopamine agonist*** (unless visual disturbance -> surgery)
*Cabergoline
*Bromocriptine
- recurrence high if tx stopped

surgery indicated when medical therapy fails

33
Q

surgical and radiotherapy tx for prolactinomas

A

Trans-sphenoidal resection- preferred surgical tx
-long term follow up necessary
-high recurrence rates
- surgery indicated when medical therapy fials

Surgical resection of a microadenoma = greatest potential for cure

Radiotherapy:
-not effective as primary therapy but may be useful as adjunctive therapy with large tumors or after surgery

34
Q

Goals of prolactinoma therapy

A

Return hormonal hypersecretion to normal
Reduce tumor size
Correct visual/cranal nerve abnormaltes
Restore abnormal pituitary function

35
Q

prolactinoma: when is tx not required?

A

-When fertility is not an issue
-If microprolactinoma have a benign natural history
-If regular menses

36
Q

efficacy of therapies: Some pts require more than 1 type of tx to correct: ______ + decrease _______

A

Some pts require more than 1 type of tx to correct prolactin levels and decrease tumor size

37
Q

MC visual abnormality of prolactinomas and what are you concerned with

A

MC visual abnormality: bitemporal hemianopsia *
-Likelihood of blindness is great because, in the absence of endocrine symptoms, visual loss is the usual initial manifestation

38
Q

Lateral extension of the tumor into the cavernous sinus can cause

A

-can cause impaired Oculomotor function involving cranial nerves III, IV, and VI, and divisions of cranial nerve V, either singly or in combination

39
Q

empty sella syndrome: typical pt

A

Typical patient is:
- Female
- Obese
- Hypertensive
- can have idiopathic intracranial htn or spinal fluid rhinnorhea

Sx:
-headaches and visual field defects +/-
-occasionally, small coexisting pituitary tumors that secrete growth hormone (GH), prolactin, or ACTH

40
Q

Empty Sella Syndrome dx and tx

A

Dx:
- CT or MRI

Tx:
-no specific therapy is needed for an empty sella alone

41
Q

Empty Sella Syndrome description

A

Sella Turcica is filled with CSF = Flattens pituitary gland against wall
-Sella appears empty
-pituitary function frequently normal
-hypopituitarism may occur

42
Q

Empty Sella Syndrome causes

A

congenital
secondary to:
- injury (head trauma, childbirth)
- surgery/ radiation