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Prolactinoma & men syndromes


Published on

by West Medical Ward
King Edward Medical University ,
Lahore, Pakistan.

Published in: Health & Medicine

Prolactinoma & men syndromes

  1. 1. Professor Tariq Waseem Dr. Hina Latif
  2. 2. Fort Munro DG Khan 2013
  3. 3. Case Scenario: 1  A 17 years old boy seeks medical advice for not growing any beard or moustaches. He has isolated himself from class fellows as they make fun of him for having feminine figure and heavy breasts.  On examination: He has smooth skin, Gynaecomastia, absent secondary sex characters and small testis.
  4. 4. Case Scenario: 2  A 16 year old girl is brought to OPD for advice regarding delayed menarche, short stature and depressed mood. She also C/O aches and pains all over body.  On examination: Height 4ft 9 inches Weight 41 Kg Pale and Smooth skin. BP 110/70.
  5. 5. Case Scenario: 3  A 30 years old school teacher married for 6 years C/O unexplained spontaneous expression of milky discharge from her breasts which is quite embarrassing for her.  On inquiry she reports oligomennorhea and has not yet conceived despite regular treatment for herself & her husband from a famous homeopathic clinic for infertility.  She also has dyspareunia, and low back pain and was told to have PID by a local doctor in community and has used Flagyl and Ciprofloxacin.
  6. 6. Case scenario: 4  A 32-year-old man visits OPD with C/O headache, blurring of vision and diplopia for six weeks.  For past six months he feels easily fatigued and attributes it to stress at workplace.  He also has loss of libido and erectile dysfunction.  He is married for 8 years but couple has no baby yet.  Wife says he has put on weight over past 6 months.
  7. 7.  Physical examination:  Weight 82 Kg  Normal predicted height  BP 140/90  Mild gynecomastia  Deficient virilization  Testicular atrophy.
  8. 8. Do you Know ?  Which is the most common hormone secreting tumor of the pituitary gland?
  9. 9.  Prolactinomas are the most common hormone- secreting pituitary tumors
  10. 10. Physiology  Prolactin (PRL), a polypeptide hormone consisting of 199 amino acids, is regulated by hypothalamic factors:  Prolactin-releasing factors (PRFs) TRH, VIP, Peptide Histidine Methionine are major PRFs  Prolactin-inhibitory factors (PIFs). Dopamine (DA) is the principal PIF A balance between the PRFs and PIFs keeps the serum PRL level within a physiologic range.
  11. 11. Prolactin  Its primary function is to enhance breast development in pregnancy and induce lactation.  It binds to specific receptors in gonads, lymphoid cells and liver.  Secretion is pulsatile; it increases with sleep, stress, pregnancy, and chest wall stimulation or trauma.  Blood sample should be drawn after fasting.  Normal Values: Less than 25-30 ng/mL
  12. 12. Regulation of prolactin secretion Predominant inhibitory signalStimulatory signal Renal clearance
  13. 13. Hyperprolactinemia:  PHYSIOLOGICAL:  Pregnancy  Nursing  Exercise  Physical and psychological stress  Sleep.
  14. 14. Hyperprolactinemia  OTHERS: Primary hypothyroidism Chest wall lesion Chronic renal failure Empty sella syndrome.  IDIOPATHIC  TUMOURS: Prolactinoma, Pituitary stalk compression by tumor( Hook effect) Craniophyrangiomas.
  15. 15. Hyperprolactinemia:  PHARMACOLOGICAL:  Estrogens  Metoclopramide  Verapamil  SSRI  Methyldopa  Opioids.
  16. 16. A Prolactinoma is classified as: Microprolactinoma (< 10 mm diameter) OR Macroprolactinoma (>10 mm diameter).
  17. 17. Do You Know Why?  60% of the Men present with macroprolactinomas.  90% of the females present with microprolactinomas.
  18. 18. It’s a fact…  Men often present much later for clinical evaluation of hypogonadism than  Women for clinical evaluation of amenorrhea.
  19. 19. Clinical manifestations:  Hormonal Effect :  Women : infertility, oligomenorrhea, amenorrhea or rarely galactorrhea .  Men : decreased libido, impotence, infertility, gynecomastia, very rarely galactorrhea .
  20. 20. Clinical presentation of hyperprolactinemia Premenopausal women 31 < PRL < 50 g/L 51 < PRL < 75 g/L 100 g/L < PRL  Hypogonadism  Galactorrhea  Amenorrhea  Oligomenorrhea Short luteal phase  Decreased libido  Infertility  Increased body weight – associated with prolactin- secreting tumor  Osteopenia – patients with associated hypogonadism  Degree of bone loss – related to duration and severity of hypogonadism
  21. 21. Clinical Manifestations  Mass Effect : headache ,CSF rhinorrhea , compression of optic chiasma & cranial nerve .
  22. 22. Female  Galactorrhea  Amenorrhea  Oligomenorrhea  Infertility  History of fracture Male  Low libido  Impotence  Infertility  Gynecomastia  Galactorrhea  History of fracture or osteoporosis Persistent gonadal dysfunction resulting in estrogen or testosterone deficiency from prolonged hyperprolactinemia if left untreated can result in premature osteoporosis in patients of either sex.
  23. 23. Diagnosis and testing:  Based on clinical evaluation, biochemical testing and imaging  History…drugs, amenorrhoea, galactorrhoea  Physical examination….visual field defects, breast discharge.  Laboratory investigations..pregnancy test, TSH, free T4, creatinine, anterior pituitary function assay  MRI of the pituitary…pituitary tumour
  24. 24. Presence of Pituitary mass on MRI:  Serum prolactin level..normal range 5-25ng/ml.  Serum prolactin values above 200 ng/mL usually indicate the presence of a lactotroph adenoma.  Macroprolactinemia….no clinical features of hyperprolactinemia but apparently elevated prolactin level, specific serum immunoassay required.
  25. 25. Imaging : MRI Of Head * Should be performed in a patient with any degree of hyperprolactinemia to look for a mass lesion in the hypothalamic-pituitary region .
  26. 26. Treatment:  Indications for treatment are….  1)Neurological symptoms  2)Hypogonadism and other symptoms  Corner stone of treatment of prolactinomas…. medical therapy.
  27. 27. Objectives of treatment of hyperprolactinemia  Restoration and maintenance of normal gonadal function  Restoration of normal fertility  Prevention of osteoporosis If a pituitary tumor is present:  Correction of visual or neurological abnormalities  Reduction or removal of tumor mass  Preservation of normal pituitary function  Prevention of progression of pituitary or hypothalamic disease
  28. 28. Medical management:  Dopamine agonists decrease prolactin secretion and reduce the size of the lactotroph adenoma in more than 90 % of patients.  Decrease symptoms within days .  Decrease in serum prolactin within 2-3 weeks .  Decrease in size within 6 weeks ... ( 6 month ) .
  29. 29. Dopamine agonists: Agonist Nature Dose Maintenance Bromocriptine ergot 2.5-10 mg/day 7.5 mg/day Lisuride ergot 0.1-0.2 mg/day 0.1 mg/day Quinagolide ergot 25-300 microgram/day 75 microgram/day Cabergoline ergot 0.25-1 mg/TWW 1mg/ week
  30. 30. ORAL CONTRACEPTIVE Estrogen- progestin : can be considered as therapy in women with symptomatic microprolactinomas IF : 1) They can’t tolerate DA 2) Don’t respond to DA 3) Don’t want to become pregnant.
  31. 31. Galactorrhea Elevated prolactin Sellar MRI Normal & Asymptomatic Normal & Symptomatic Microadenoma & Symptomatic Macroadenoma Dopamine agonist therapy Measure other pituitary hormones to exclude associated deficiency or excess Follow-up Measurement of Prolactin Once yearly
  32. 32. Normal & Symptomatic Micro & Symptomatic Dopamine agonist therapy Follow up Normal Prolactin level Reduced prolactin level After 6 months therapy Prolactin level still elevated After 6 months therapy* Asymptomatic Symptomatic Consider pituitary surgery Measure prolactin level Every 4 – 6 months
  33. 33. Tapering Dopamine Agonist  Consider tapering after 2 years in those: who no longer have elevated serum PRL who have no visible tumor remnant on MRI  May be possible to discontinue therapy when menopause occur
  34. 34. Management of Prolactinomas during Pregnancy:  Stop dopamine receptor agonists  Follow patient symptomatically every 3 months  If headache or visual complaints…repeat MRI (non- contrast) and visual field tests  Reinstitute bromocriptine if evidence of tumour enlargement  Monitoring prolactin levels during pregnancy…not indicated
  35. 35. Case scenario:  A 34 yr old female who had a prolonged history of epigastric pain radiating to her back, underwent abdominal surgery after her gastrin level was found markedly elevated but her symptoms recurred and so did serum gastrin levels and she was put on PPI.  Two years later she presented with headache and lethargy, amenorrhea , loss of appetite but no weight loss.  Her father had neck surgery 20yrs ago to treat kidney stones. Her maternal aunt had CA stomach.
  36. 36. Physical Examination  Pale, scanty axillary and pubic hairs, dry skin,  Pulse..62/min  BP…105/65mm Hg  Fundoscopy….revealed slightly pale discs and bi-temporal hemianopia on visual field testing.  Neurological examination….Normal  Normal CBC with sodium level 129mmol/L, LOW TSH & T4…prolactin 4500mu/L  Skull xray …showed enlargement of pituitary fossa  DIAGNOSIS?????
  37. 37. MEN Syndrome:  Multiple endocrine neoplasias ( MEN) …inherited as autosomal dominant disorders
  38. 38. MEN I: Wermer Syndrome  AD familial syndrome characterized by tumors of  Parathyroid glands( 95%),  Endocrine gastroenteropancreatic {GEP} tract (30-80%)  Anterior pituitary (15-80 % cases)  And skin.  Most common endocrine tumours are parthyroid tumours.  Others include gastrinomas, insulinomas, prolactinomas and carcinoid tumours.  Strong family history.
  39. 39. Case scenario:  A 32 years old female has headache and general malaise for 4 months. Over the past 3 weeks, she has developed nausea and epigastric pain after meals. She also gives H/O off and diarrhea for 2 years.  She also describes frequent episodes of restlessness ,palpitations, sweating and flushing but attributes these symptoms to fear of illness.
  40. 40. Examination  Anxious looking, with irregular swelling in front of neck which moves with deglutition.  Heart rate 120/min regular  B.P…180/105 mmHg  Systemic Examination: Unremarkable.
  41. 41.  HB…17g/dl  Calcium…2.8mmol/L  Phosphate…0.6mmol/L,  TSH 3mu/L  Normal RFT’s n serum sodium and potassium  Normal blood glucose  DIAGNOSIS??????
  42. 42. MEN II:  Most commonly involve  Adrenal gland (50%),  Parathyroid gland (20%)  Thyroid gland (almost 100%)  Further divided into MEN IIa and MEN IIb