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Transcript of Pathology of the Endocrine Organs - I Pituitary Jaroslava Dukov Inst. Pathol. 1st Med. Fac. Charles...
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Pathology of the Endocrine Organs - I
Pituitary
Jaroslava Dušková Inst. Pathol. 1st Med. Fac. Charles Univ. Prague http://www1.lf1.cuni.cz/~jdusk/
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Pituitary - history - I
Galenos (2nd cent.) lat. pituita = gr. phlegm = moisture, mucus
A. Vesalius (16th cent.)
glandula pituitam cerebri excepiens
R. Lower (17th cent.) Dissertatio de origine catarrhi – incretion
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Pituitary - history - II
H. Rathke (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
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1. formation of the Rathke´s pouch & proc. infundibularis
2. splitting of the Rathke´s pouch
3. mature formation
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Neuroimmunoendocrine Regulation Messengers
Neurotransmitters Interleukins Hormons
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Pituitary - regulation
Higher neural centra
limbic system reticular system
hypothalamus
peripheral endocrine glands
adenohypophysis
tissues
liberins & statins
trophic hormons of the adenohypophysis
hormons of periph. glands
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Pituitary – weight
Rasmussen, ATAm.J. Anat. 1928 a 1934
Females505 - 1002 mg(average 660 mg)pregnant women560 - 1220 mg(average 762 mg)
Males400 - 855 mg(average 570 mg)
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Pituitary - architecture
adenohypophysis pars infundibularis (tuberalis) pars intermedia neurohypophysis
hypophysis pharyngea (+ hidden islets of pit. cells in the os sphenoides)
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Pituitary - cell inclusions
squamous epithelium Rathke´s pouch between AH and NH salivary glands - NH, often with
oncocytes
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Pituitary - parts - function
Adenohypophysis secretion of tropins Pars infundibularis (tuberalis)
modulation of AH secretion Pars intermedia Neurohypophysis secretion of neuropeptides hypophysis pharyngea possible secretion
of tropins
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Pituitary – cell populations acidophil somatotrophs, lactotrophs basophil corticotrophs, gonadotrophs,
thyreotrophs chromofobe (transitional. +
folliculostellate) oncocytes (or preoncocytes) mesenchymal pituicytes (macroglia) secretion neurons (tractus supraoptico-
et tuberohypophyseus)
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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
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Pituitary - cell population & hormonal production
Hormonal production mostly mixed
(e.g. ACTH+FSH,LH,TSH,PRL)
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Pituitary - cell population &hormonal production
Individual producents able of interconversion
following stimulation (e.g. PRL-GH)
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L e p t i n 7th chromosome
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
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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
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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
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Pituitary - necrosis
incidence 1- 8 % large autopt.
series pathogenesis intracranial
hypertensionischemiavasospasmusatherosclerosisthrombistalk lesion
healing scar focal regenerates possible
meaning – hypofunction only in
case of more than 3/4 of volume destruction
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Necrosis hypophysis intra partum
sy. Sheehani
• hyperplasia • shock• ischaemia• necrosis• panhypo- pituitarismus
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Pituitary - inflammation
non specific (peri)hypophysitis
purulentanon purulenta
septicpyemicmicroabscesses
lymphocytic– autoimmune
specific tbc
hematogenous dissemination
solitary tuberculoma
lues inbornacquired
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Pituitary syndromes
Hypofunctional–panhypopituitarismus–selective hypofunction
Hyperfunctional–monohormonal–combined
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Hypopituitarismus
Total >90% AH destroyed
Syndromes: Simmonds Sheehan Falta Lorain
Partialmonohormonalcombined
Regulatory hypofunction
peripheral glands ectopic
production iatrogenous
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Petzold S, Keller A, Keller E, Meigen C, Hirsch W, Kratzsch JK, Kiess W, Pfäffle R.
A prismatic case: A 31-year old man who did not miss his pituitary. lab. panhypopituitarismus – dg. at the age of 13 MRI- empty sella growth retardation correction, cryptorchidism, hypoglycemie, He felt better without hormonal therapy, hypogenitalism, normal
professional life & mental status
Hormones (Athens). 2008 Jul-Sep;7(3):203-4.
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Pituitary Adenomas
no galactorea-amenorea acromegaly hypercortisolism chiasma opticum compression hypopituitarism
Clin. symptomes 9%9%
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Pituitary Adenomas
1. chiasma opticum compression 432. acromegaly 233. galactorea-amenorea 74. hypopituitarism 7
Clin. symptomes in surgically treated (n=80)
54%28%
9%9%
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Pituitary Adenomas
Guanine nucleotide- binding protein (G protein) mutations - signal transduction- uncontrolled proliferation (40% GH adenomas and som ACTH have GNAS I gene mutations)
Familiar pituitary adenomas: MEN I, CDKN I B, PRKAR I A, AIP
TP 53 mutations – aggressive behaviour
pathogenesis 9%9%
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The WHO Classificationof Adenohypophysial Neoplasms .
A proposed five-tier scheme
1. endocrine activity2. imaging/ surgery3. histology4. immunohistochemistry5. ultrastructure
Kovacs, K., Scheithauer, B., Horvath Eva, Lloyd, R Cancer 1996, 78,502-10
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Pituitary Adenomas
acidophillic basophillic chromophobe mixed
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Acidophillic adenomas
somatotroph lactotroph mixed somatotroph and lactotroph somatolactotroph densely granulated
onkocytic
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Basophillic adenomas
corticotrophic thyreotrophic gonadotrophic densely granulated
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m. Cushing paraneopl. pituitary ad.
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m. Cushing periph. hypothalamic
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Chromophobe adenomas
all types of hormonal productionsscarcely
granulated
null cell
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HyperpituitarismusMonohormonal
Syndromes:1. gigantismus/acromegaly2. hyperprolactinemia
(galaktorea, amenorea)
3. Cushing4. hyperthyreosis5. (hypergonadotropinismus)
Combined1+2, 1+3, 1+4, 2+3, 3+4
Regulatory hyperfunctionperiph. glandsectopic productioniatrogenous
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Plurihormonal and PlurifunctionalPituitary Adenoma with Acromegaly Syndroma
doc. MUDr Jaroslava Dušková, CSc*,prof. MUDr Josef Marek, DrSc**, prof. MUDr Ctibor Povýšil,DrSc*
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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
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Plurihormonal and PlurifunctionalPituitary Adenoma
with Acromegaly
STH +++ Prl ++ ACTH ++
TSH +++FSH (beta)+LH (beta)+
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Pituitary INCIDENTALOMA- algorithm of Investigation and treatment
m e d ik a m e n t . th .b r o m o c r ip t in
p r o la k t in o m
o p e r a c e
j in ý
h y p e r f .
k o n e c s le d .
b e ze z m ě n y
o p a k o v a t N M R z a 1 ,2 ,5 ,le t
< 1 cm
o p e r a c e
r ů st tu m o rup o r u ch a z o r . p o le
h y p o p itu ita rism u s)
o p a k o v a t N M R z a 0 ,5 1 ,2 ,5 ,le t
z o r n é p o lev y l . h y p o p itu ita r ism u s
> 1 c m
F u n k c e h y p o fý z yPituitary function
other
operation
repeat NMR in1,2,5 yrs
no change
visual field, excl. hypopituit.
repeat NMR in 0.5,1,2,5 yrs
end check- ups
operation
neoplasm growth, visual field def., hypopituitarism
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Other Pituitary Tumours
craniopharyngeoma metastases
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WHO Histological Typing of Tumours of the CNS
TUMOURS OF THE SELLA REGION & TUMOUR LIKE LESIONS
– craniopharyngeoma– Rathke´s cyst– epidermoid cyst– dermoid cyst– colloid cyst of 3rd ventricle– enterogenous cyst, pituicytoma, nasal glioma
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WHO Histological Typing of Tumours of the CNS
TUMOUR LIKE LESIONS OF THE SELLA REGION – diff. dg.!!!
– Rathke´s cyst– epidermoid cyst– dermoid cyst– colloid cyst of 3rd ventricle– enterogenous cyst
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Other Pituitary Tumours
craniopharyngeoma metastases