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Pathology- Endocrinology
Pituitary Disorders (T Pierce)
20
Medical
Professional
03/30/2010

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Cards

Term
Adjacent structures to pituitary gland
Definition
  • CN III, IV, and VI
  • ICA and ECA
  • cavernous sinuses
  • optic chiasm
Term
Diaphragma sellae (structure, function)
Definition
  • continuation of dura
  • function
    • covers roof of sella turcica
    • protects pituitary from pressure of CSF in subarachnoid space
Term
Histology of pituitary gland
Definition
  • nests of secretory cells
    • acidophil cells (syn. peptide hormones)
    • basophil cells (syn. glycoprotein hormones)
    • chromophobe cells (too few granules to be IDed as either acidophil or basophil)
Term
embrylolgical development of anterior pituitary
Definition
  • formed by invagination of Rathke's pouch in roof of oral cavity
    • occasionally see cystic remnants of this invagination
    • lined by squamous epithelium
    • tumors can often resemble normal structures of oral cavity
    • ectopic pituitary tissues may be found anywhere along migratory path
Term
pituitary adenoma: clinical presentation, dx
Definition
  • clinical presentation
    • hormonal overproduction (most frequently of PRL, but less of GH, ACTH)
    • in men, initial presentation may be due to mass effects of hyperpituitary function
    • in women, nonfunctional tumors are relatively uncommon
  • dx- image studies
    • if microadenoma, MRI used
    • if macroadenboma, plain films
Term
pituitary adenomas: morphology and classification
Definition
  • gross
    • spherical masses that grow by compressing adjacent pituitary
    • may protrude through diaphragma sellae (suprasellar extension)
    • may see erosion into and through bone with extension into adjacent sinuses
    • may be macroadenomas or microadenomas
  • histology
    • uniform, but pleomorphism may be present
    • cant make dx of malignancy based on histology exam
  • classification
    • size (micro and macro)
    • older schemes may be acidophil, basophil, or chromophobe cells (chromophobe is MAJORITY)
    • hormones present in tumor cells (ex: prolactinemias)
Term
pituitary adenomas: prognosis and tx
Definition
  • prognosis
    • usually slow growing neoplasms
    • course is prolonged even though tumor has extended beyond the pituitary
  • tx
    • for prolactinemia, dopamine agonists (decrease PRL production and reduce size of tumor)
    • if you stop tx, most patients will experience regrowth
    • if secretes GH, ACT or is nonfunctiona, we do surgery, radiation preferred for tumor in combo with medical therapy
      • for ACTH, cyproheptadine
      • for GH, somatostatin analogues
Term
craniopharyngioma: origin, epidemiology, location, clinical presentation, morphology, why is tx difficult
Definition
  • epidemiology
    • mostly in children (arises from embryonic structure) but may be found in any age range
    • less common than adenomas
  • origin- benign tumor derived from Rathke's pouch
  • clinical presentation
    • suprasellar, midline location (but can be within sella or sinuses)
    • usually detected due to mass symptoms or pituitary hypofunction
  • morphology
    • gross- cystic mass composed of structures normally found in oral cavity (squamous epithelium, mass of mineralized material simulating tooth buds)
      • may see calcification on X ray films
    • histologically bening
  • tx is difficult
    • cause fatalities because it is difficult to remove entirely due to location
    • if the keratin in the cysts leak out, it can cause fatal chemical meningitis
Term
infarction: clinical presentation, what it can lead to?
Definition
  • clinical presentation
    • hypotension
    • shock
    • pituitary is enlarged and more vascular than normal
      • ex: pregnancy or pituitary adenoma
  • if there is a pregnancy related hemorrhage, it can lead to hypofunction called Sheehan's syndrome
  • if pituitary adenomas lead to infarction and hemorrhage, its called pituitary apoplexy
    • leads to increase in CSF pressure, followed by coma and death
Term
empty sella syndrome: cause and pathogenesis
Definition
  • pathogenesis
    1. there is a defect in the diaphragma sellae
    2. arachnoid is pushed into the sella by the pressure of the CSF
    3. causes selllar enlargement and compression of pituitary around periphery of sella
  • epidemiology
    • usually middle aged women
    • very common cause of sella enlargement
  • common clinical presentation
    • most are asymptomatic
    • most common symptoms due to increase PRL due to compression of pituitary stalk
  • dx- usually incidental finding during imaging studies or autopsy

Although name says empty, there is usually a normal amount of pituitary tissue surroundin the fluid filled subarachnoid space that produces an empty appearance

Term
Most common modes of presentation of pituitary disease
Definition
  • due to mass efffect
  • due to hormone overproduction
  • due to hormome underproduction
Term
Pituitary disease manifestations: mass effect symptoms
Definition
  • headache
    • look for change in type, frequency, or duration of headaches compared to person's usual headaches
  • vision problems due to compression of optic nerve and/or nerves supplying extra-ocular muscle (all of which pass near sella turcica) present in many patients
    • classic: bitemporal hemianopsia
    • most patients tend to have asymmetric visual field loss
Term
pituitary disease manifestation: hormone overproduction symptoms
Definition
  • if overprod. gonadotropins, we see hypogonadism
    • pulsatile release is needed for normal function of gonads
  • usually due to excess (in desc. order) of
    • PRL
    • GH
    • ACTH
Term
Symptoms of PRL overproduction in men and women
Definition
  • women- fairly dramatic
    • abnormal milk production (galactorrhea)
    • amenorrhea (remember PRL inhibits gonadotropin production)
    • usually, the above two symptoms co-exist
      • lack of normal dev. of follicles can lead to infertility
  • men- majority are asymptomatic
    • impotence and infertility are most common complaints
Term
Most common causes of increase PRL levels
Definition
  • medications (MOST COMMON)
    • OCP's
    • phenothiazines
    • cimetidine
  • pituitary adenoma
    • pituitary stalk compress base of brain (ex: craniopharyngioma, empty sella syndrome)

 

Term
pituitary manifestations: GH overproduction (manifestation in children and adults)
Definition
  • gigantism (in childhood)
    • increase bone length and thickness
  • acromegaly (in adulthood)
    • increase bone thickness (esp. in face)
      • prognathism (protruding lower jaw)
      • big digits (growth in hands, feet)
Term
GH excess: features other than those specific to children and adults
Definition
  • symptoms
    • skin- thickening of soft tissue and overgrowth of sebaceous glands
      • thick, oily skin
    • heart- HTN, interstital fibrosis lead to cardiac damage
    • DM (GH antagonizes insulin)
    • degenerative arthritis (larger bones)
  • dx
    • measure IGF-1 aka somatomedin-C
      • produced in response to GH
      • unline GH, there is no diurinal fluctuation
Term
pituitary disease manifestations: symptoms due to GENERALIZED underproduction (panhypopituitarism) (which hormones are the first to go)
Definition
  • common with pituitary tumors and infarct of tumors to have more than one hormone have some degree of underproduction
  • usually the first one to be underproduced in GH (NO symptoms in adults)
  • PRL underproduction
    • absent milk production in post partum pituitary infarction (otherwise asymptomatic)
  • gonadotropin underpoduction
    • often asymptomatic in men
    • in women between 10 and 45, you will see cessation of menstrual cycles
  • thyrotropin (takes more time to have underpoduction)
    • often presenting symptom in post-menopausal women and men
  • ACTH and ADH underpoduction happens VERY LATE
Term
Effect of GH undeproduction (tx, dx, presentation)
Definition
  • presentation (MOST common underproduced hormone)
    • short stature in kids (look like toddlers)
    • if severe, you will see hypoglycemia
  • dx- lack of response of GH to normal stimuli
    • IGF-I not useful measurement in children under 5
  • tx- GH (prompt increase in height and the earlier the tx, the greater the height that can be reached)
Term
effect of ADH underproduction (etiologies, presentation, commonality, prognosis)
Definition
  • most common isolated pituitary deficiency in adults
  • presentation
    • lesion usually in hypothalmus, but can also be in pituitary or the stalk
    • polydypsia, polyuria (1 L/hr)
    • as long as patients have access to water, their plasma volume remains normal (thirst receptors still in tact)
    • when deprived of water, they become dehydrated (they cant concentrate their urine)
  • common etiologies
    • tumors
    • any space occupying lesions
    • trauma to head
  • prognosis- half of patients with post traumatic DI recover
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