Pituitary adenomas, prolactinoma, and empty sella

Question 1

pituitary disorders

Answer 1

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

Question 2

hypothalamic-pituitary lesions

Answer 2

-Patients present with
-Combination of symptoms or signs of a mass lesion:
-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)

Question 3

pituitary or hypothalamic tumor

Answer 3

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

Question 4

pituitary adenomas

Answer 4

-Benign neoplasms of the anterior pituitary
-Symptoms
-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

Question 5

pituitary adenomas: classification

Answer 5

-Macroadenoma - >10mm - either increased hormones or enlarged and not functioning
-Microadenoma - <10mm
-Secretory vs. nonfunctional
-Secretory tumors:
-Prolactin-secreting adenomas
-GH secreting adenomas
-Corticotrophin-secreting adenomas
-Thyrotropin-secreting adenomas

Question 6

pituitary adenomas: nonfunctional adenomas OR non-secreting adenomas

Answer 6

-especially Gonadotropin-secreting*– little or no clinical effect
-Or may not secrete any detectable hormone
-Generally present as macroadenomas with headache, visual disturbances, or hypopituitarism
-Some adenomas are capable of secreting both prolactin and GH

-Therapy for pituitary adenomas:
Transphenoidal surgery
Pituitary radiation
Pharmacotherapy

Question 7

pituitary adenomas: corticotropin secreting pituitary adenoma

Answer 7

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

Question 8

pituitary adenomas: thyrotropin-secreting pituitary adenoma

Answer 8

-Produces thyrotoxicosis
-Goiter may be present
-Visual impairment can occur

Question 9

pituitary adenomas: GH secreting pituitary adenoma

Answer 9

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

Question 10

adenoma symptoms

Answer 10

-From macroadenomas -usually due to mass effect
-Headache and visual field deficits
-From microadenomas - usually due to effects of aberrant hormone levels
-Patient may report infertility, impotence, or dyspareunia (painful intercourse due to decreased vaginal secretions) (FSH, LH)
-Hypopituitarism and hypogonadism due to destruction of pituitary gland
-Pathologic fractures due to osteoporosis

Question 11

signs macroadenomas

Answer 11

-examine for signs of hypopituitarism:
-Pallor
-Hypotension
-Visual field defects
-Prolactin-secreting tumors:
-Galactorrhea
-Hypogonadism
-Features of osteoporosis

Question 12

signs of all the tumors

Answer 12

-GH-secreting tumors: Signs of acromegaly

-Thyrotropin-secreting pituitary adenoma:
-Thyrotoxicosis
-Goiter
-Visual impairment

-Corticotropin -secreting pituitary adenoma:
-Truncal obesity
-Round face
-Dorsocervical fat pad (buffalo hump)
-Hirsutism
-Acne
-Menstrual disorders
-Hypertension
-Striae
-Bruising
-Thin skin

Question 13

adenoma DX

Answer 13

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

-Clinical presentation must be consistent with a syndrome caused by a pituitary tumor
-Blood tests and MRI
-Pregnancy test - r/o

Question 14

treatment of goals of adenomas

Answer 14

-eliminate- eliminate effects due to the mass of tumor
-reduce- reduce elevated pituitary hormone levels to normal
-ameliorate (improve)- ameliorate end organ effects of elevated pituitary hormone levels
-avoid- avoid damage to remaining normal hypothalamic or pituitary functinon
-minimize- minimize other potential adverse effects of therapy

Question 15

other pituitary tumors: MEN syndromes

Answer 15

-Syndromes of MEN
-autosomal dominant traits
-cause a predisposition to the development of tumors of 2 or more different endocrine glands
-Genetic testing should be done
-MEN 1: tumors of the parathyroid glands, pancreas and pituitary,
-MEN 2A : medullary thyroid cancers, pheochromocytoma, hyperparathyroidism -> RET mutation

Question 16

multiple endocrine neoplasia (MEN) 1

Answer 16

-Autosomal dominant syndrome
-characterized by a genetic predisposition to parathyroid, pancreatic islet, and pituitary adenomas
-About 1/2 of affected pts develop prolactinomas -> Acromegaly and Cushing’s are less commonly encountered
-Carney syndrome - spotty skin pigmentation, myxomas, and endocrine tumors including testicular, adrenal, and pituitary adenomas -> Acromegaly – 20%

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

-Familial acromegaly: rare disorder in which family members may manifest either acromegaly or gigantism

Question 17

pituitary metastases

Answer 17

-Occur in ~3% of cancer pts
-Blood-borne metastatic deposits are found almost exclusively in posterior pituitary
-diabetes insipidus can be presenting feature of lung, GI, breast, and other pituitary metastases
-1/2 of pituitary metastases originate from breast cancer; about 25% of patients with metastatic breast cancer have such deposits
-rarely, pituitary stalk involvement results in anterior pituitary insufficiency.
-MRI dx of metastatic lesion may be difficult to distinguish from aggressive pituitary adenoma; [histologic examination]
-Primary or metastatic lymphoma, leukemias, and plasmacytomas also occur within the sella

Question 18

prolactinoma

Answer 18

-Intrasellar tumor of the anterior part of the pituitary gland, which secretes prolactin
-Treatment depends on size and encroachment on optic chiasm
-women present early bc galactorrhea, absent periods etc.
-men present later -> when tumor is large -> diff tx
-Treatment:
-Medical, surgical, or radio-therapeutic
-Dopamine agonists - preferred option
-Recurrence rates are high with surgery

Question 19

prolactinoma etiology

Answer 19

-physiologic
-nipple stimulation/suckling
-pregnancy
-postpartum period
-stress
-food ingestion
-sexual intercourse in some women
-sleep
-hypoglycemia

-hypothalamic disorders*
-hypothalamic tumors
-infiltration- sarcoidosis, TB, langerhans cell histiocytosis (Hand-Schüller-Chrisitan disease)
-post-encephalitis
-idiopathic galactorrhea
-head trauma

-pharmacologic causes*
-amphetamines, nicotine, TCA, verapamil, estrogens**

-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

Question 20

features of prolactinoma

Answer 20

-MC pituitary tumor
-results in hypeprolactinemia
-features in women*:
-oligomenorrhea
-amenorrhea
-infertility
-galactorrhea
-hypopituitarism- rare
-microadenoma- usually

Question 21

features of prolactinoma: men

Answer 21

-Hypogonadism; decreased libido and erectile dysfunction; infertility;
-Decreased or softer beard and body hair;
-Fatty breast enlargement
-Macroadenoma - usually
-Galactorrhea (one-third of cases)
-Neurologic effects- Headaches due to raised intracranial pressure
-Visual loss from optic nerve compression*
-Bitemporal hemianopsia*!!
-Altered vision – blurring

Question 22

visual fields

Answer 22

-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

Question 23

prolactinoma symptoms: :(

Answer 23

-apathy in 63% (DDx of depressive illness) in men as well as women)

Question 24

prolactinoma: symptoms and signs

Answer 24

-Ophthalmoplegia - Squint
-Double vision
-Hemorrhage into tumor -> May rarely cause hypopituitarism requiring immediate evaluation and treatment
-Uncommonly severe headache with prostration
-Seizures:
-If large tumors extend into temporal lobe
-Rare

Question 25

prolactinoma signs

Answer 25

-Vary with age, sex, duration of hyperprolactinemia, and tumor size
-Often chance radiologic discovery with no signs
-Signs can be due to mass effects of the tumor or to hyperprolactinemia itself
-*In women
-Signs of prolonged estrogen DEFICIENCY - atrophic vaginitis, thinning of pubic hair, loss of labial fat pads, reduction in breast size

Question 26

prolactinoma tests

Answer 26

-Medications*
-TSH*
-Serum prolactin levels!- Prolactin secretion – episodic; stress may raise prolactin concentrations
-Low or borderline level should be confirmed by obtaining several samples
-Normal <20mcg/L
-A single prolactin measurement may be sufficient to diagnose a prolactinoma if the value is >200mcg/L
-MRI**

Question 27

prolactinoma- rx

Answer 27

-indications for tx of a prolactinoma are: restoration of fertility and presence of a macroadenoma
-goals of therapy:
-Return hormonal hypersecretion to normal
Reduce tumor size
Correct visual or cranial nerve abnormalities
Restore any abnormal pituitary function

-clinical tx of choice dopamine agonist*** (unless visual disturbance -> surgery)
-trans-sphenoidal resection- preferred surgical tx
-long term follow up necessary
-high recurrence rates

Question 28

prolactinoma: tx

Answer 28

-tx is not required in certain cases:
-When fertility is not an issue
Microprolactinomas have a benign natural history
If regular menses, no therapy is necessary

-dopamine agonists:
-cabergoline
-bromocriptine

-surgery:
-surgical resection of a microadenoma offers the greatest potential for cure

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

Question 29

prolactinoma: efficacy of therapies

Answer 29

-natural hx of prolactinoma- not known
-most grow slowly over years
-if tx stopped- recurrence high
-surgery indicated when medical therapy fails
-some pts require more than 1 type of tx to correct prolactin levels and decrease tumor size

Question 30

clinical pearls

Answer 30

-Likelihood of blindness is great because, in the absence of endocrine symptoms, visual loss is the usual initial manifestation
-The most common visual abnormality is bitemporal hemianopsia
-Lateral extension of the tumor into the cavernous sinus:
-can cause impaired Oculomotor function involving cranial nerves III, IV, and VI, and divisions of cranial nerve V, either singly or in combination

Question 31

empty sella syndrome

Answer 31

-The sella appears empty
-It is filled with CSF, which flattens the pituitary gland against the wall of the sella
-Syndrome may be congenital, primary, or secondary to injury (such as ischemia after childbirth, surgery, head trauma, or radiation therapy)
-Typical patient is:
-female (> 80%), obese (about 75%), and hypertensive (30%) and may have idiopathic intracranial hypertension (10%) or spinal fluid rhinorrhea (10%)
-pituitary function frequently normal
-hypopituitarism may occur
-headaches and visual field defects +/-
-occasionally, small coexisting pituitary tumors that secrete growth hormone (GH), prolactin, or ACTH
-dx can be confirmed by CT or MRI
-no specific therapy is needed for an empty sella alone