pathology of endocrine glands - iii

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Pathology of Endocrine Glands - III. Adrenals. Endocrine Pancreas. Jaroslava Dukov Inst. Pathol. 1st Med. Fac. Charles Univ. Prague. Adrenals. cortex definitive fetal (90% regression by 6 months of age) neonate 8g (3,5kg) 0,002 - PowerPoint PPT Presentation

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  • Pathology of Endocrine Glands - IIIAdrenalsJaroslava Dukov Inst. Pathol. 1st Med. Fac. Charles Univ. PragueEndocrine Pancreas

  • Adrenals cortex definitivefetal (90% regression by 6 months of age)

    neonate 8g (3,5kg) 0,002 healthy adult 9g (70kg) 0,0001 zones G,F,R20x medulla

  • normhypoplasia congenitalis

  • Adrenals - syndromes hypofunction - panhypocorticalism acute chronic Adison peripheral central hyperfunction AGS Cushing hyperaldosteronism Conn, Bartter

  • m Adison: peripheral central

  • Adrenals - syndromes hypofunction - panhypocorticalismus acute chronic Adison periferic central hyperfunction Cushing hyperaldosteronism Conn, Bartter AGS

  • WHO Clasification of Tumours of the Adrenal Gland (WHO 2004) Adrenal cortical tumours Adrenal cortical carcinoma Adrenal cortical adenoma Adrenal medullary tumours malignant phaeochromocytoma benign phaeochromocytoma composite phaeochromocytoma/paraganglioma

    Extra-adrenal paragangliomacarotid bodyjugulotympanicvagallaryngealaorticopulmonarycauda equina..

  • Adrenal Cortical Carcinoma M8370/3two peaks of incidence middle to old age & preschool children mostly hormonally active androgens onlyandrogens + glucocorticoidsandrogens + glucocorticoids+mineralocorticoidsestrogens (exceptional)

  • Adrenal Cortical Carcinoma M8370/3Malignancy criteriahigh nuclear grade (Fuhrman)mitoses incl. atypicaldiffuse architecturenecrosisinvasion into veins, capsule

  • m. Cushing periph.hypothalamic

  • m. Cushing paraneopl.15% pituitary C.>50%

  • Androgen overproductionAGS

  • WHO Clasification of Tumours of the Adrenal Gland (WHO 2004) Adrenal cortical tumours Adrenal cortical carcinoma Adrenal cortical adenoma Adrenal medullary tumours malignant phaeochromocytoma benign phaeochromocytoma composite phaeochromocytoma/paraganglioma

    Extra-adrenal paragangliomacarotid bodyjugulotympanicvagallaryngealaorticopulmonarycauda equina..

  • PheochromocytomaDef.:benign tumour deriving from chromaffin cells(intraadrenal paraganglioma) Clin.: resistence, hypertensionMacro: whittish, solid, regressive changesMicro: solid alveolar (Zellballen)Behaviour: benign (15% bilateral, 10% children,10% malignant)part of MEN II and von Hippel-Lindau disease

  • Biology Behaviour of PheochromocytomaDiffuse growthCentral necrosesHigh cellularityMonotonous FusocellularMitoses >3/10 HPFAtypical mitosesInvasion into fatty tissueInvasion to vesselsTranscapsular invasionPleomorphic cellsNuclear hyperchromasia

    Thompson L.D.R.: Phaeochromocy toma of the Adrenal Gland Scoring Scale (PASS) to separate benign from malignant neoplasms. A clinicopathologic and immunophenotypic study of 100 cases. Am. J. Surg. Pathol. 26(5), 2002, 551-566PASS score222222221111PASS score

  • Neuroblastoma (WHO: Neuroblastic tumours of adrenal gland and sympathetic nervous system)Def.: childhood embryonal tumours of migrating neuroectodermal cells derived from the neural crest and destined for the adrenal medulla and sympathetic nervous system Age /sex 96% in the 1st decade , no sex predilectionIncidence: most common solid extracranial malignant tumours during the first two years of lifeHistogenesis: see definitionClinic: palpable mass (retroperit, abd., cervical), X-ray - thoracicMacro: soft gray-tan mass, regressive changesMicro: undiff. + differentiating neuroblasts

    Variants: neuroblastoma (undiff.), ganglioneuroblastoma intermixed, ganglioneuroblastoma nodular, ganglioneuromaBehaviour: malignant, dependent on age and histology variant

  • Islets of Langerhans (1869) adults 100 000 -1000 000 cell types:B - insulinA - glucagonD somatostatinPP pancreatic polypeptideD vasoactive intestinal polypeptide

  • Islets of Langerhans - regressive changes fibrosis (postinflamm.) - DM I mucoviscidosis DM frequency 10x hyalinosis, amyloidosis

  • Islets of Langerhans - progressive changes hyperplasia diabetic embryopathy nesidioblastosis

  • Islets of Langerhans - tumours nesidioma ( event. in MEN I) insulinoma, glucagonoma, somatostatinoma,VIPoma, PP-oma, G cells -gastrinoma, EC serotonin - carcinoid neuroendocrine carcinoma

  • New classification of GastroEnteroPancreatic NeuroEndocrine Neoplasms GEP NEN (2010) NeuroEndocrine Tumour NET NET G1 (carcinoid) - M8240/3if hormonally active insulinoma, gastrinoma, glucagonoma, somatostatinoma, VIPoma /Mitoses 20%/

    NeuroEndocrine Carcinoma NEClarge cell NEC - M8013/3small cell NEC - M8041/3Mixed AdenoNeuroEndocrine Carcinoma MANEC M8244/3Exceptions: Tubular appendical carcinoid M8245/1; L-cell NET PP/PYY M8152/1

  • Islets of Langerhans - syndromes hyperfunction - insulinomahypoglycemia (weekness , sweating, tremor, coma)

    Zollinger-Ellison, Werner Morrison, glucagonoma

    hypofunction absolute or relative insulin lack ( DMI/II or glucagonoma)hyperglycemia acute : polydipsia, ketoacidosis, coma, liver steatosis , brain edema

    chronic: diabetes mellitus: microangiopathy, macroangiopathy, neuropathy, retinopathy, embryopathy

  • Diabetes mellitusDef.: group of disorders with glucose intolerance in common---------------Chronic hyperglycemia and disturbances of carbohydrate, protein, and fat metabolism.

  • Diabetes mellitus - types DM I IDDM juvenile DM II NIDDM -(+MODY)

    Other secondary- pancreatic disease, drugs, chemicalsGestational - GDM

  • Diabetes - detailed classificationType I Diabetesbeta cell destructionType II- Diabetes beta +insulin resistenceGenetic Defects of Beta Cell Function Maturity Onset Diabetes of the Young (type 1-6 with known mutations) Maternaly inherited diabetes and deafness due to mitochondrial mutations Defects in proinsulin conversionInsulin gene mutationInsulin Receptor MutationsExocrine Pancreas DiseasesChronic pancreatitisPancreatectomyNeoplasiaCystic fibrosisHemochromatosisFibrocalculous pancreatopathy

    EndocrinopatiesAcromegalyCushing syndromeHyperthyroidismPheochromocytomaGlucagonomaInfectionsCMVCoxsackie BCongenital rubellaDrugsGlucocorticoidsThysroid hormonesBeta-adremergic agonistsGenetic Syndromes Associates with DiabetesDown syndromeKlinefelter syndromeTurner syndrome Gestational Diabetes

  • Diabetes mellitus - complications acutehypoglycemia (DM I and insulin treatment)diabetic ketoacidosis : lack of insulin increased release of fatty acids increased ketone formation metabolic acidosischronic AGE Advanced Glycosylation End-products diabetic micro- and macroangiopathy neuropathy infection

  • Pekov M., Hvi R., Dukov, J. Malignant somatostatinoma (brief overview and a case review) Rozhl Chir. 2007 Dec;86(12):643-7. Czech. Male 73

  • well differentiated endocrine carcinoma of pancreas headmetastasizing into peripancreatic lymph nodes.T 99 M 81503Dg.:

  • Case Reportwoman 26 yrsN 571/92

  • History - 1.mononucleosis in the childhood2 yrs prior to death during her 9th week of pregnancy repetitive amentia statuses, hospitalized in the Psychiatry Clinichypoglycemia 1,2mmol/l foundtransferred to General Medicine Clinictwo weeks later gravidity interruption

  • History - 2.explorative laparotomy - tumour of the pancreas with liver metastases no tumour in the biopsy sample takennext two monts cycles of chemotherapy, the hormonal activity of the neoplasm dissappearedprogression of the neoplasm with the gastric wall infiltration

  • History - 3.hormonal activity of the neoplasm reappearedcytostatics administered into a. hepaticadeath two years from the onset of the disease

  • C- peptide proteolytic phragment of proinsulin secreted (equimollar quantities) by beta-cells of Langerhans islets

  • DiagnosisMorbus principalis Carcinoma neuroendocrinum parvocellulare pancreatis ad parietem ventriculi et reproperitoneum progressumComplicationesMetastases carcinomatosae lnn. mesentericorum, hepaticorum, iliacorum. Hyperinsulinismus.Causa mortis Generalisatio carcinomatis

  • Hyperinsulinism due to pancreatic tumour mostly B-cell NESIDIOMA (insulinoma) - BENIGN rare in pregnancy malignant B-cell tumourneuroendocrine carcinoma / nesidioblastoma extremely rareparaneoplastic hypoglycemia mesenchymal retroperitoneal, adrenocortical, GIT tumours

    kong. hypopl. primary X chrom linked only in boys, secondary in Hyth-hypof pathology

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