pituitary adenoma, prolactinoma, empty sella Flashcards
pituitary disorders
-hypothalamic-pituitary lesions
-pituitary adenoma/tumors
-prolactinoma
-empty sella syndrome
hypothalamic-pituitary lesions presentation
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)
pituitary or hypothalamic tumor
-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
pituitary adenomas
-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
pituitary adenomas: classification
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
Therapy for pituitary adenomas:
Transphenoidal surgery
Pituitary radiation
Pharmacotherapy
Nonfunctional adenomas OR non-secreting adenomas
*Generally present as MACROadenomas with headache, visual disturbances, or hypopituitarism
corticotropin secreting pituitary adenoma
-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
pituitary adenomas: thyrotropin-secreting pituitary adenoma
-Produces thyrotoxicosis
-Goiter
-Visual impairment
pituitary adenomas: GH secreting pituitary adenoma
Mostly MACROadenomas with little or no clinical effect
-May present with visual field deficits
-Gigantism or acromegaly
-Hypertension
-Hyperglycemia
-Osteoarthritis
-Signs of hypopituitarism ±
symptoms: macroadenomas vs microadenomas
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
With macroadenoma examine for signs of: _________ such as
Examine for signs of hypopituitarism:
-Pallor
-Hypotension
-Visual field defects
-Hypogonadism
-Features of osteoporosis
Diagnosing pituitary adenomas
-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
treatment of goals of adenomas
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”
What are MEN syndromes?
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
What are the men syndrome classifications
MEN 1:
-parathyroid glands
-pancreas
- pituitary
MEN 2 (MEN 2A):
- medullary thyroid cancers
- pheochromocytoma
- Hirschsprung disease
MEN 3
MEN 4
multiple endocrine neoplasia (MEN) 1
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
What syndromes are associated with MEN1 tumors?
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
pituitary metastases
Occur in ~3% of cancer pts
-Blood-borne metastatic deposits ALWAYS prefer posterior pituitary*
- 50% of pituitary metastases = breast cancer
MRI dx of metastatic lesion may be difficult to distinguish from _________. what test do you need to perform?
-MRI dx of metastatic lesion may be difficult to distinguish from:
- aggressive pituitary adenoma
Need to do:
- tissue bx: histologic examination
Rarely, pituitary stalk involvement in metastases results in _________.
anterior pituitary insufficiency
presenting feature of lung, GI, breast, and other pituitary metastases
-diabetes insipidus: posterior pituitary metastases
prolactinoma description
Intrasellar tumor of the anterior part of the pituitary gland, which secretes prolactin
- MC pituitary tumor
Prolactinoma tx
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
prolactinoma: men vs women presentation
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*
Signs of Prolactinoma
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
prolactinoma etiology
-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
visual fields
-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
With prolactinoma, what are the signs of prolonged estrogen deficiency in women….
atrophic vaginitis
thinning of pubic hair
loss of labial fat pads
reduction in breast size
What tests should be checked in prolactinoma?
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
prolactinoma- indications for tx
Indications for tx of a prolactinoma are:
-restoration of fertility
- presence of a macroadenoma
Clinical TX of choice for prolactinoma
Dopamine agonist*** (unless visual disturbance -> surgery)
*Cabergoline
*Bromocriptine
- recurrence high if tx stopped
surgery indicated when medical therapy fails
surgical and radiotherapy tx for prolactinomas
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
Goals of prolactinoma therapy
Return hormonal hypersecretion to normal
Reduce tumor size
Correct visual/cranal nerve abnormaltes
Restore abnormal pituitary function
prolactinoma: when is tx not required?
-When fertility is not an issue
-If microprolactinoma have a benign natural history
-If regular menses
efficacy of therapies: Some pts require more than 1 type of tx to correct: ______ + decrease _______
Some pts require more than 1 type of tx to correct prolactin levels and decrease tumor size
MC visual abnormality of prolactinomas and what are you concerned with
MC visual abnormality: bitemporal hemianopsia *
-Likelihood of blindness is great because, in the absence of endocrine symptoms, visual loss is the usual initial manifestation
Lateral extension of the tumor into the cavernous sinus can cause
-can cause impaired Oculomotor function involving cranial nerves III, IV, and VI, and divisions of cranial nerve V, either singly or in combination
empty sella syndrome: typical pt
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
Empty Sella Syndrome dx and tx
Dx:
- CT or MRI
Tx:
-no specific therapy is needed for an empty sella alone
Empty Sella Syndrome description
Sella Turcica is filled with CSF = Flattens pituitary gland against wall
-Sella appears empty
-pituitary function frequently normal
-hypopituitarism may occur
Empty Sella Syndrome causes
congenital
secondary to:
- injury (head trauma, childbirth)
- surgery/ radiation
