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Common Types of Pituitary Tumors

Common Types of Pituitary Tumors. Laura Knecht MD. Medical Director of the Barrow Pituitary Center. Introduction. Pituitary tumors comprise 10-15% of all intracranial tumors Gliomas – Meningiomas - Pituitary adenomas 10% of all surgically resected tumors

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Common Types of Pituitary Tumors

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  1. Common Types of Pituitary Tumors Laura Knecht MD Medical Director of the Barrow Pituitary Center

  2. Introduction • Pituitary tumors comprise 10-15% of all intracranial tumors • Gliomas – Meningiomas - Pituitary adenomas • 10% of all surgically resected tumors • Affects up to 20% population (1 in 5) • Majority arise from anterior pituitary gland

  3. Pituitary Adenomas • Classified based on size, secretory abilities, histology • <10mm-microadenomas • >10mm-macroadenomas • Beyond sella-giant adenomas

  4. M:F • Females • Present with micoadenomas • Age 20-30 • Hormone dysfunction • Menstral irregularities • Infertility • Males • Present with macroadenomas • Age 40-50 • Mass effect • HA • Visual compromise

  5. FSH/LH-Gonadotroph adenomas • Nonfunctioning, rarely functions • Prolactin-Prolactinomas • Most common • TSH-TSHomas • Rare cause of hyperthyroidism, <1% pituitary adenomas

  6. GH-Acromegaly • Can be cosecretors with GH/prolactin • ACTH-Cushing’s Disease • 2/3 of causes of Cushing’s Syndrome

  7. Nonfunctioning Pituitary Tumors

  8. Nonfunctioning Pituitary Tumors • 30% of all pituitary tumors • No evidence of hormonal hypersecretion • Large at presentation • Mass effects • HA • Visual field deficits • Hypopituitarism

  9. Mass Effects • Hypopituitarism • GHRH – FSH/LH/prolactin – TSH - ACTH • Elevated prolactin from stalk effect • Compression of optic chiasm • Bilateral superior temporal hemianopsia • Lateral growth into cavernous sinus • Cranial nerve palsies

  10. Mass Effects • Headache • Pressure on dura • Blood products • Cystic components • Pressure on frontal/temporal lobes • Hydrocephalus • Memory issues

  11. Hormonal Testing • ACTH, 8am cortisol • TSH, free T4, free T3 • Prolactin • With 1:100 dilution if macroadenoma • GH, IGF-1 • FSH, LH, total testosterone/estradiol • Alpha subunit

  12. MRI • Pituitary protocol • Gadolinium • 3T • Dynamic protocol • Experience of center

  13. Neuro-ophthalmology Testing • Formal visual fields • Visual acuity • Health of optic nerves

  14. Prolactinomas

  15. Prolactinomas • Most common hormonally active tumor • F>>M • Microadenomas • Benign, regress spontaneously, can have no growth • Macroadenomas • Present w/ pressure symptoms, increase in size, rarely disappear

  16. Prolactinomas • Clinical features dependent on prolactin level, mass effects, hypopituitarism • Gender, age, tumor size • Prolactin stimulates milk in Estrogen-primed breast • High prolactin inhibits GnRH which decreases FSH/LH which decreases testosterone/estrogen

  17. When to Treat • Infertility • Menstral disturbances • Bothersome galactorrhea • Enlarging tumor • Apoplexy w/ headache

  18. Treatment • Medications • 1st line treatment regardless of size • Surgery • In resistant prolactinomas • Intolerance to both dopamine agonists • Radiation • For residual/recurrent tumor

  19. Cabergoline • Better tolerated • Fewer side effects • More likely to normalize level • No increased risks in pregnancy • ½ life-2-3days • Effective dose 1-1.5mg twice a week, resistant prolactinomas 7-12mg/week

  20. Cabergoline • May be fast metabolizers • Change to every day or every other day • Valvulopathy • Mitral valve stenosis • May be reversible • ? role of echocardiogram

  21. Bromocriptine • Cheaper • ½ life-8hrs • Should be 2-3 times daily • Common dose up to10mg every night or 5mg twice a day • Doses >20-40mg not more efficacious • Preferred agent in pregnancy • No risk of valvulopathy

  22. Side Effects of Dopamine Agonists • Nausea • Lightheadedness • Mental fog • Worsening of depression • Psychotic reaction • Minimize if take at night, start low, go slow, take w/ snack

  23. Use of Hormone Replacement in Prolactinomas (Estrogen) • Possible growth of tumor • In combo w/ dopamine agonists-safe • No prospective studies when used alone • Would not use if chiasmopathy • Monitor prolactin regularly

  24. Pregnancy • Risk of micro growing-1-3% • Risk of macro growing-<15% • Stop medication once pregnant • Go thru pregnancy, breastfeeding, restart if amenorrhea, future fertility • Monitor for headache, vision changes • Can get MRI not Gadolinium • May have issues w/ lactation • Role of debulking if macroadenoma, chiasmopathy

  25. Cushing’s Disease

  26. Clinical Manifestations • Degree • Duration • Presence/absence androgen excess • Cause • Tumor related symptoms • Age

  27. Clinical Manifestations • Obesity • Moon facies • Dorsal cervical fat pad • Exophthalmos • Periorbital edema • Conjunctival injection • Chest/facial plethora • Growth retardation • Skin atrophy • Easy bruisability • Striae • Hyperpigmentation • Hirsutism • Acne

  28. Complications • Fungal infections • Cardiovascular complications • Stroke, heart attack • Proximal myopathy • Psychiatric disturbances • Menstrual abnormalities • Osteoporosis • PCOS (Polycystic Ovarian Syndrome) • Diabetes/impaired glucose tolerance

  29. Screening Tests • 1 mg overnight dexamethasone • Take at 11pm • Draw cortisol at 8am • Cortisol < 1.8mg/dL – r/o hypercortisolism • Cortisol > 5mg/dL = hypercortisolism

  30. 24 hour urinary free cortisol • If >3x normal, diagnostic of true Cushing’s • Lesser elevations require confirmation • False elevations of UFC • Physical stress • Exercise • Large volume intake • Medications

  31. Late Night Salivary Cortisol • Bedtime/11pm salivary cortisol (series of 2-3)

  32. Overnight High Dose Dexamethasone Suppression • Dexamethasone 8mg by mouth at 11pm • Serum cortisol at 8am • Will suppress in pituitary source • Cortisol <1.8mg/dL • Cortisol <50% of baseline

  33. Inferior Petrosal Sinus Sampling/IPSS

  34. Surgery • Goal is for cure • Immediate post-op cortisol <2-3mg/dl within 24-72hours • If not cured, consider • Repeat surgery • Radiation treatment

  35. Ketoconazole • Dosing 200-400mg BID-TID • Side effects • HA • Sedation • Nausea/vomiting • Gynecomastia • Decreased libido • Impotence • Life threatening-reversible hepatotoxicity

  36. Mifepristone (Korlym) • Dosing 300mg daily • Maximum dose 1200mg daily • Maximum dose in hyperglycemia 600mg daily • Side effects • Adrenal insufficiency • Peripheral edema • Hypertension • Headache • Hypokalemia • Endometrial hypertrophy • Cannot follow cortisol levels

  37. Pasireotide • Dosing 600-900 mcg subcutaneously twice daily • Decrease in cortisol, ACTH, salivary cortisol • Signs and symptoms improved • Side effects • Hyperglycemia • Diarrhea • Abdominal discomfort • Gallstones

  38. Bilateral Adrenalectomy • Immediate cure • Complication-Nelson’s Syndrome • Vision loss • Progression of pituitary tumor • Dependent on glucocorticoids and mineralicorticoids

  39. Post-op Management • Adrenal insufficiency results • Treat w/ decreasing doses of steroids • Initial dosing – hydrocortisone 40-80mg daily • Wean over 6-24months • Cosyntropin stimulation testing once off to confirm normal axis

  40. Monitoring • Lifelong • Patients usually feel symptoms prior to abnormalities in testing • Yearly cortisol, ACTH • Scheduled MRIs • Consider hypercortisolemia testing • Late night salivary testing • 24 hour urine free cortisol • 1mg overnight dexamethasone suppression

  41. Acromegaly

  42. Acromegaly • M=F • Mean age 42-44 • Usually have diagnosis 7-10 years prior • Premature mortality from cardiovascular disease with risk decreasing when normalize IGF-1, GH

  43. Symptoms • Change in facial features • Enlargement in forehead, mandible, tongue, gap in teeth • Enlargement of hands/feet • Excessive sweating • Dental malocclusions • Sleep apnea

  44. Signs/Symptoms • Diabetes • Hypertension • Colon polyps • Arthralgias • Skin tags • Carpal tunnel

  45. Co-morbidities • Cardiomyopathy/Congestive Heart Failure • Diabetes/Insulin resistance • Hypertension • Obstructive sleep apnea • Precancerous colon polyps • Thyroid nodules

  46. Lab Values • Elevated GH • Elevated IGF-1 • Lack of GH suppression to glucose load

  47. Treatment • Surgery • 1st line treatment by experienced surgeon • Medications • Has been used as adjunctive vs primary medical therapy • Radiation

  48. Surgery • Post op day 1 GH<5 highly predictive of remission • Remission if GH<1 after OGTT • IGF-1 takes weeks to months to decrease because of delayed clearance

  49. Medical Treatment • Somatostatin analogs • Dopamine agonists • GH receptor antagonist

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