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Pathology of the Endocrine Organs - I

Pathology of the Endocrine Organs - I. Pituitary. Adrenals. Jaroslava Dušková Inst. Pathol. 1st Med. Fac. Charles Univ. Prague https://www1.lf1.cuni.cz/~jdusk/. Pituitary - history - I. Galenos ( 2nd cent.)

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Pathology of the Endocrine Organs - I

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  1. Pathology of the Endocrine Organs - I Pituitary Adrenals Jaroslava Dušková Inst. Pathol. 1st Med. Fac. Charles Univ. Prague https://www1.lf1.cuni.cz/~jdusk/

  2. Pituitary - history - I Galenos (2nd cent.) lat. pituita = gr. phlegm = moisture, mucus A. Vesalius(16th cent.) glandula pituitam cerebri excepiens R. Lower(17.stol.) Dissertatio de origine catarrhi – incretion

  3. Pituitary - history - II Rathke, H.: (1838) Über die Entstehung der glandula pituitaria (Arch Anat. Physiol. u. Wiss. Med. , 5, 482-5) W. Haberfeld(1909) Die Rachendachhypophyse, andere Hypophysengangreste und deren Bedeutung für die Pathologie Beiträge zur path. Anat. u. allg. Pathol. , 46, 133-232)

  4. 1. formation of the Rathke´s pouch & proc. infundibularis 2. splitting of the Rathke´s pouch 3. mature formation

  5. Neuroimunoendocrine regulation CRF IL-1 neuronal connections ACTH TNF IL-6 IL-1 n.vagus Glucocorticoids Adrenals Immune cells

  6. Pituitary - regulation Higher neural centra limbic system reticular system hypothalamus liberins & statins adenohypophysis trophic hormons of the adenohypophysis periferal endocrine glands hormons of periph. glands tissues

  7. Neuroimmunoendocrine Regulation Messengers • Neurotransmitters • Interleukins • Hormons

  8. Pituitary – weight Females 505 - 1002 mg (average 660 mg) pregnant women 560 - 1220 mg (average 762 mg) Males 400 - 855 mg (average 570 mg) Rasmussen, AT Am.J. Anat. 1928 a 1934

  9. Pituitary- architecture • adenohypophysis • pars infundibularis (tuberalis) • pars intermedia • neurohypophysis • hypophysis pharyngea (+ hidden islets of pit. cells in the os sphenoides)

  10. Pituitary- cell inclusions • squamous epithelium • Rathke´s pouch between AH and NH • salivary glands- NH, often with oncocytes

  11. Pituitary- parts - function • Adenohypophysis secretion of tropins • Pars infundibularis (tuberalis) modulation of AH secretion • Pars intermedia • Neurohypophysissecretion of neuropeptides • hypophysis pharyngea evtl. secretion of tropins

  12. Pituitary– cell populations • acidophil(somatotrophs, lactotrophs) • basophil(corticotrophs, gonadotrophs, thyreotrophs) • chromofobe(transitional. + foliculostellate) • oncocytes(or preoncocytes) • mesenchymal • pituicytes (macroglie) • secretion neurons(tractus supraoptico- et tuberohypophyseus)

  13. Hypophysis- cell population & hormonal production • acidophil PRL , STH • basophil ACTH, FSH, LH, TSH • chromophobe 0, PRL , STH , ACTH, FSH, LH, TSH • oncocytes 0, PRL , STH , ACTH, FSH, LH, TSH • mesenchymal • pituicytes • secretion neurons oxytocin, vasopresin

  14. Pituitary- cell population & hormonal production Hormonal production mostly mixed (e.g. ACTH+FSH,LH,TSH,PRL)

  15. Pituitary - cell population &hormonal production Individual producents able of interconversion due to stimulation (e.g. PRL-GH)

  16. L e p t i n • adipocytes - blood - CSF -hypothalamus (ncl. arcuatus ) • regulation of energetic homeostasis • correlation with the body fat content • in most obese individuals high levels -resistence? Friedman et al., Nature 1994

  17. Pituitary- regressive changes • Dystrophy(Crooke´s hyaline change) • Atrophy - in aging increased fibrosis, no functional influence • NECROSIS • traumatic (mostly due to the stalk lesion) • ischemic

  18. Pituitary- vascularisation • a. hypophysea sup. (from ACI) • a. trabecularis directly to AH • long portal veins in the stalk • a. hypophysea inf. (from ACI in sinus c.) • short portal veins

  19. incidence 1- 8 % large autopt. series pathogenesis intracranial hypertension ischemia vasospasmus atherosclerosis thrombi stalk lesion healingscar focal regenerates possible meaning hypofunction only in case of more than 3/4 of volume destruction Pituitary- necrosis

  20. non specific (peri)hypophysitis purulenta non purulenta septic pyemic microabscesses lymphocytic autoimmune specific tbc hematogenous dissemination solitary tuberculoma lues inborn acquired Pituitary- inflammation

  21. Pituitary syndromes • Hypofunctional • panhypopituitarismus • selective hypofunction • Hyperfunctional • monohormonal • combined

  22. Total >90% AH destroyed Syndromes: Simmonds Sheehan Falta Lorain Partial monohormonal combined Regulatory hypofunction peripheral glands ectopic production iatrogenous Hypopituitarismus

  23. m. Adisoni centralis • adrenal atrophy • vacuolisation of cardiomyocytes • lack of the skin hyperpigmentation • hypotension • weekness • hyperkalaemia

  24. Pituitary Adenomas 9% • chiasma opticum compression 43 • acromegaly 23 • galactorea-amenorea 7 • hypopituitarismus 7 - most frequent clin. symptomes 9% 54% 28%

  25. The WHO Classificationof Adenohypophysial Neoplasms . A proposed five-tier scheme 1. endocrine activity 2. imaging/ surgery 3. histology 4. immunohistochemistry 5. ultrastructure Kovacs, K., Scheithauer, B., Horvath Eva, Lloyd, R Cancer 1996, 78,502-10

  26. Pituitary Adenomas • acidophillic • basophillic • chromophobe • mixed

  27. Acidophillic adenomas • somatotroph • lactotroph • mixed somatotroph and lactotroph • somatolactotroph densely granulated • onkocytic

  28. Basophillic adenomas • corticotrophic • thyreotrophic • gonadotrophic densely granulated

  29. Chromophobe adenomas • all types of hormonal productions scarcely granulated • null cell

  30. Monohormonal Syndromes: 1. gigantismus/acromegaly 2. hyperprolactinemia (galaktorea, amenorea) 3. Cushing 4. hyperthyreosis 5. (hypergonadotropinismus) Combined 1+2, 1+3, 1+4, 2+3, 3+4 Regulatory hyperfunction periph. glands ectopic production iatrogenous Hyperpituitarismus

  31. Plurihormonal and PlurifunctionalPituitary Adenoma with Acromegaly Syndroma doc. MUDr Jaroslava Dušková, CSc*, prof. MUDr Josef Marek, DrSc**, prof. MUDr Ctibor Povýšil,DrSc*

  32. F 75 yrs • 30 yrs lasting acromegaly • refused surgery • Symptomatic therapy • hyperfunction thyr . – Carbimazol • cardiomegaly - cardiotonica • 5 yrs prior tu death ca coli – surg. removed • 6 yrs prior tu death corticoid substitution • death cardial failure

  33. STH +++ Prl ++ ACTH ++ TSH +++ FSH (beta)+ LH (beta)+ Plurihormonal and PlurifunctionalPituitary Adenoma with Acromegaly

  34. Pituitary INCIDENTALOMA- algorithm of Investigation and treatment

  35. Other Pituitary Tumours • craniopharyngeoma • metastases

  36. Adrenals • cortex • definitive • fetal (90% regression by 6 months of age) • neonate 8g (3,5kg) 0,002 • healthy adult 9g (70kg) 0,0001 • zones G,F,R 20x • medulla

  37. Adrenals - syndromes • hypofunction - panhypocorticalism • acute • chronic – Adison • peripheral • central • hyperfunction • AGS • Cushing • hyperaldosteronismus Conn,Bartter

  38. norm hypoplasia congenitalis

  39. Adrenals - syndromes • hypofunction - panhypocorticalism • acute • chronic – Adison • peripheral • central • hyperfunction • AGS • Cushing • hyperaldosteronismus Conn,Bartter

  40. m Adison: peripheral central

  41. Adrenals - syndromes • hypofunction - panhypocorticalismus • acute • chronic – Adison • periferic • central • hyperfunction • AGS • Cushing • hyperaldosteronismus Conn,Bartter

  42. m. Cushing periph. hypothalamic

  43. m. Cushing paraneopl. pituitary C.

  44. Androgen overproduction AGS

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