pituitary tumors n.
Download
Skip this Video
Loading SlideShow in 5 Seconds..
PITUITARY TUMORS PowerPoint Presentation
Download Presentation
PITUITARY TUMORS

Loading in 2 Seconds...

play fullscreen
1 / 89

PITUITARY TUMORS - PowerPoint PPT Presentation


  • 291 Views
  • Uploaded on

PITUITARY TUMORS. PITUITARY TUMORS. Account for 10-25 % of brain tumors Medium age at debut: between 20-50 years Children rarely have pituitary adenomas. Most tumor in children are craniphariogiomas and are associated with growth failure and diabetes insipidus.

loader
I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.
capcha
Download Presentation

PowerPoint Slideshow about 'PITUITARY TUMORS' - kishi


An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.


- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -
Presentation Transcript
pituitary tumors1
PITUITARY TUMORS
  • Account for 10-25 % of brain tumors
  • Medium age at debut: between 20-50 years
  • Children rarely have pituitary adenomas. Most tumor in children are craniphariogiomas and are associated with growth failure and diabetes insipidus.
  • Most pituitary adenomas in children are prolactinomas
  • Prolactinomas, Gh secreting adenomas and ACTH-secreting adenomas are more frequent in women. GH secreting adenomas are more frequent in men.
pituitary tumors classification
PITUITARY TUMORS - CLASSIFICATION

According to their size:

  • Microadenomas:have less than 1 cm, do not modify the shape of sella turcica and do not produce pituitary tumor syndrome
  • Macroadenomas:have nore tahn 1 cm. and according to the direction they develop produce “the syndrome of pituitary tumors”

According to their degree of aggression

  • Benign adenomas
  • Invasive adenomas
  • Carcinamas: less then 1 % of pituitary tumors
histogene sis of pituitary tumors
HISTOGENESISOF PITUITARY TUMORS

Two hit hypothesis:

Pituitary adenomas are monoclonal tumors

Polyclonal adenomas may result from excessive stimulation of pituitary by specific releasing hormones

Pituitary cells have a genetic protective factor against tumor proliferation. Lost of one protective allelle - first hit is not associated with tumor transformation, a point mutation of the second allelle – second hit results in tumor proliferation . Tumor occurs only if both protective factors are lost

histogene sis of pituitary tumors1
HISTOGENESISOF PITUITARY TUMORS

Anotherpathogenic hypothesis is an activating mutation of alpha subunit of GTP-binding protein which activates cAMP and stimulates cell proliferation

În MEN 1– Multiple Endocrine Neoplasia type 1 there is an autosomal dominant deletion of a protective gene MENINE encoded on chromosome 11 (11q13)and multiple tumors simultaneous or successive occur:

- multiple parathyroid adenoams with primary hyperparathyroidism

- gastro-entero-pancreatic tumors: gastrinoma, insulinoma, glucagonoma

- carcinoid tumors

- adrenal adenomas

- lipomas

- facial angiofibromas

pituitary tumor s y ndrom e
PITUITARY TUMOR SYNDROME
  • NEUROLOGIC SYMPTOMS:
    • Headache
    • Nerves III, IV and VI which cross the cavernous sinus
    • Temporal seizures
    • Other seizures
    • Meningeal signs
  • OPHTALMOLOGICSIGNS
    • Decreased visual acuity
    • Reduction of visual field according to tumor extension
    • Exophtalmos : rare
  • RADIOLOGICAL SYGNS
    • Enlarged surface of sella turcica
    • Radiologic signs specific for some pituitary adenomas: acromegaly
diagnosi s of pituitary adenomas
DIAGNOSIS OF PITUITARY ADENOMAS
  • Clinical suspicion
  • Assessment of pituitary hormones to determine hormonal secretion of adenomas and level of other pituitary hormones in case if pituitary is partially dystroyed.
  • Radiograph of sella turcica: useful in case of macroadenomas
  • CT orMRI of hypothalamic-pituitary area
  • Inhibitory tests, biochemical markers for some adenomas
tr e atment of pituitary adenomas
TREATMENT of PITUITARY ADENOMAS
  • SURGERY
  • RADIOTHERAPY
  • PHARMACOTHERAPY
surgical treatment of pituitary adenomas
SURGICAL TREATMENT OF PITUITARY ADENOMAS
  • First intention therapy for all adenomas with exception of those which have a proven beneficial pharmacological treatment
  • Immediately indicated in tumors which exert compression over structures from the proximity and involve a risk for sight loss or have intracranial hypertension.
  • Is an emergency treatment for pituitary apoplexy – pituitary infarct.
  • May be delayed until pharmacological treatment may reduce tumor volume and make the tumor more accessible to surgery in some responsive cases
surgical treatment of pituitary adenomas1
SURGICAL TREATMENT OF PITUITARY ADENOMAS

Aim of surgery:

  • To reduce mass effect produced by large tumors over adiacent structures
  • To inhibit hormone secretion in pituitary secreting adenomas
  • To preserve morphologic and functional integrity of the pituitary
surgical treatment of pituitary adenomas2
SURGICAL TREATMENT OF PITUITARY ADENOMAS

Approach of the pituitary during surgery:

  • Transcranial approach: in large tumors with extra selar extension. The aim is to reduce tumor volume and has greater number of complications
  • Transphenoidal approach – is used in most adenomas with medium and small size. This treatment have no complications in a skillful hand and preserves the pituitary function if it was not previously affected.
  • Complete cured: 90 % of microadenomas
  • Tumor reduction without complete cure in larger tumors
surgical treatment of pituitary adenomas3
SURGICAL TREATMENT OF PITUITARY ADENOMAS

Complication of pituitary surgery depend of the size of the tumor and quality of surgery:

  • Death by carotid injury
  • Severe complication due to injury of cavernosal sinus and nerves III,IV and VI
  • Brain injuries
  • Chiasma injury with complete sight loss
  • Infections: meningitis, enchephalitis
  • Cerebro-spinal flud fistula
  • Diabetes insipidus: permanent 5 % of (frequently transitory condition – some weeks)
  • Syndrome of inapropriate vasopressine secretion 10 %
  • Hypopituitarism 5-10 % in large tumors
i r radi ation in pituitary adenomas
IRRADIATION IN PITUITARY ADENOMAS

Convenţional irradiation:

The tumor is irradiated based on a computerized program which includes CT and MRI in order to spare the proximal regions with CT/IRM 4000 – 5000 cGy, in fractionated doses of 180 – 200 cGy per day, 5 days per week

Succes:

  • 80 % in acromegaly, but full effect appear variably in time until 8 years and even more
  • 55 – 60 % in ACTH-secreting tumors , in a shorter time
  • In prolactinomas the response rate is less important because tumor secretion may be successfully controlled with dopamine agonists
i r radi ation in pituitary adenomas1
IRRADIATION IN PITUITARY ADENOMAS

Complications of conventional irradiation:

  • Hypopituitarism in 50-60 % of cases in 8-10 years
  • Optic nerve injury
  • Brain radio necrosis
  • Occurrence of other neoplasia of the brain favor by previous irradiation

Gamma knife delivers in one MRI –guided the entire dose of irradiation on a very small field

The effects of irradiation are more rapid – until 4 years

Only in tumors which are more distant of the optic chiasm: at least

4 mm.

Until the cure obtained by irradiation the tumor secretion and growth must be controlled by pharmacotherapy

prolactinom as and hyperprolactinemia
Prolactinomas and hyperprolactinemia

Prolactin excess inhibits gonadotropins secretion

In women:

  • Secondary amenorrhea, oligomenorrhea, infertility
  • Galactorhea
  • Hirsutism
  • Signs of estrogen deficiency with genital atrophy
  • Osteoporosis
  • Pituitary failure in large prolactinomas

Most prolactinomas in women are microadenomas.

prolactinom as and hyperprolactinemia1
Prolactinomas and hyperprolactinemia

In men: decreased testosterone secretion with:

  • Decreased libido
  • Erectile dysfunction
  • Infertility
  • rare: gynecomastia şi galactorhea
  • Pituitary failure

In men most prolactinomas are macroprolactinomas and are associated with “pituitary tumor syndrome”

slide37

Microprolactinoma

Macroprolactinoma

other causes of hyperprolactinemia
OTHER CAUSES OF HYPERPROLACTINEMIA
  • Physiological: breast feeding, sexual activity, sleep, stimulation of mamary gland
  • Interruption of conection between hypothalamus and pituitary and inhibitory control of the hypothalamus over pituitary, stalk section, stalk compression by other tumors, hypothalamic tumors
  • Empty sella syndrome
  • Drugs which inhibit dopamine: psychotropes, antidepressives, l-DOPA, 5HT2 inhibitors, estrogeni, oral contraceptives
  • Hypothalamic diseases: sarcoidosis, hysticytosis
  • Polycystic ovarian disease, acromegaly, hipothyroidism, kidney failure, liver cirrhosis
  • Torax unjuries
assessement oh h y perprolactinemi a
ASSESSEMENT OH HYPERPROLACTINEMIA
  • Prolactin values
  • Prolactin levels correlates with tumor size
    • Normal prolactin levels: 9-25 ng/ ml
    • 50 ng/ ml functional hyperprolactinemia
    • between 50-100 ng/ ml microprolactinomas
    • over 100 ng/ ml macroprolactinomas
  • Bromocriptine test:
    • 2,5 mg bromocriptinemust reduce prolactin levels
  • Assessement of lesions: CT, IRM
treatment of prolactinomas
TREATMENT OF PROLACTINOMAS

Pharmacotherapy – dopamine agonists

  • First choice treatment in microprolactinomas and pre treatment in macroprolactinomas in order to reduce tumor size and facilitate surgery
    • Bromocriptine: 2,5 – 20 mg /day
    • Cabergolină: 0,5 – 3,5 mg /week
    • quinagolid
  • Effects of pharmacotherapy:

- menses occur again

- fertility is restored

- during pregnancy the treatment may be stoped

- during pregnancy the tumor is followed by assessing the visual field

treatment of prolactinomas1
TREATMENT OF PROLACTINOMAS

SSurgery

  • For large tumors with compressive symptoms
  • May be done after previous pharmacotherapy
  • Effects of surgery:

- in best cases gonadotropin secretion occurs again

- risks and complications are similar to other pituitary tumors submited to surgery

- residual disease may be controlled with dopamine agonists

C. External irradiation is rarely needed

acromegal y
ACROMEGALY

PREVALENCE:

  • 40 – 60 cases / 1 milion /year
  • 3-2 new cases per year
  • 1 / 15.000 person
acromegal y1
ACROMEGALY

Causes :

  • sporadic:
    • Adenoama pure high granulated, sparse granulate
    • Mixed GH and prolactin secreting adenomas
    • Acidofilic adenoams with stem cells
    • Ectopic adenomas
    • GH secreting carcinoma
    • Mc Cune-Albright syndrome
  • Familial forms: izolated, MEN 1, Carney complex, FIPA -
  • Hypothalamic GH.RH excess: harmartoms, gangliocytoma, glyoma,
  • Extrahypothalamic GH-RH secretion
    • Pancreatic carcinoids, bronchial carcinoma MTC,
slide47

Development of the disease is insidious and graduated during years, the disease being recognized 10 years after real debut

acromegaly signs and symptoms
Acromegaly – signs and symptoms
  • Signs and symptoms of the disease are determined by the effects of GH and IGF1 over target tissues after the epiphyseal growth plates are closed. In case of a precocious debut gigantism occurs
  • Short and flat bones are more affected,
  • GH and IGF1 excess produce
    • Hypertrophy of all structures containig connective tissue and bone
    • Metabolic abnormalities
acromegal y signs and symptoms
ACROMEGALY SIGNS AND SYMPTOMS

SIGNS AND SYMPTOMS AT THE DEBUT:

  • Headache
  • Joint and bone pain
  • Dental problems
  • Amenorhea, galactorhea, loss of libido
  • Diabetes mellitus
  • Hyperhydrosis
  • Carpal tunell syndrome
  • Sleep apnea
  • HTA, cardiomyopathy
  • Colonic poliposis
acromegal y signs and symptoms1
ACROMEGALY signs and symptoms
  • Pituitary tumor syndrome:
  • Narrowing of the visual field,
  • Decreased visual acuity
  • Facial abnormalities:
    • Prominent frontal boses
    • Prominent occipital bone
    • Enlargement of low jaw
    • Dental : spaces between tees
    • Large tongue
acromegalia signs and symtpoms
ACROMEGALIA signs and symtpoms
  • Abnormalities of hands and feet:
    • Thicknening of the fingers
    • Carpal tunell syndrome
  • Joints and spine:
    • Spondilosis
    • Osteoarthritis
    • skin: hyperhidrosis
    • Cutis giratta
    • Moluscum pendulum
    • Skin spots
acromegal y metabolic problems
ACROMEGALY – metabolic problems
  • Lypolisis
  • Insulinoresistence
  • Diabetesmellitus
  • Hypercalciuria, hypercalciuria
  • Sodium and water retention
acromegal y complica tion
ACROMEGALy – Complication
  • Hearth :
    • Increased cardiac volume and systolic volume
    • Interstitial fibrosis
    • Systolic and dyastolic dysfunction
    • Ventricular dillatation
    • Hearth failure
  • Lung:
    • Laringeal hypertrophy
    • Respiratory dysfunction
    • Sleep apnea
  • Digestive : colonic polyposis
  • Other tumors
acromegalia diagnostic
ACROMEGALIA – diagnostic

GH increased in multiple determinations. IGF-1 increased

GH during OTTG

GH below< 1 ng/ml

GH not inhibited during OTTG

Imagery CT, IRM

Fundus of the eyeVF

Nu este acromegalie

slide68

OCTREOSCAN – Indium labeled Somatostatin scintigraphy allows to detect somatostatin receptors and predicts the response of tumors to somatostatin analoques

acromegal y tr e atment
ACROMEGALY TREATMENT
  • Surgery: transphenoidal, transcranial
  • Radioterapy
  • Long-acting somatostain analoques
  • Inhibitors of GH receptor
acromegal y surgical treatment
ACROMEGALYSURGICAL TREATMENT
  • In emergency if there are symptoms of pituitary apoplexy
  • Guided by MRI and computer-assisted navigation
  • Transphenoidal approach is most frequent
  • Criteria for cure: GH during OTTG <0.30 ng/ ml, Partial response medium GH per 24 h les than 2.5 ng/mL
  • Normal IGF
treatment of complications
Treatment of complications
  • Osteoarthritis
  • Osteoporosis
  • Hypercalciuria
  • Hyperparatiroidism in MEN1
  • Treatment of sleep apnea
  • Monitorig Hb A1c, triglycerides
  • Treatment of hearth complications
  • Monitoring for colonic polyposis and colonic cancer
acth secreting adenoma
ACTH- SECRETING ADENOMA
  • Small or very small size
  • Clinically manifested by Cushing’s disease
  • Diagnosis: cortisol, Dexametasone inhibition test,
  • Tretament: surgery and /or gamma knife