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Home , Adrenal medulla, Somatostatinoma, VIPoma

10/23/2018

Overview and VHL: The adrenal and the  Definition of a  Adrenal  Adrenal  Pancreas gland LAUREN FISHBEIN MD, PHD  Pancreas hormones and enzymes UNIVERSITY OF COLORADO SCHOOL OF MEDICINE DIVISION OF ENDOCRINOLOGY, AND DIVISION OF BIOMEDICAL INFORMATICS AND PERSONALIZED MEDICINE OCTOBER 20, 2018

Endocrine Master Gland Metabolism What is a hormone?  Organs in our body that make Energy hormones BP/HR

Energy BP Metabolism

Food breakdown and metabolism Signals for energy usage and stores

Female hormones Male hormones

https://s-media-cache-ak0.pinimg.com/736x/5e/eb/a4/5eeba4d996ec972d35980896fe5bb654.jpg

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What is a hormone? Can you name some hormones?

 Hormones are chemical messengers in the body Common examples  Send messages about a particular function from  hormone one cell to another  Estrogen Several other hormones come  Progesterone Pancreas from the adrenal and Liver  Testosterone pancreas glands Hormone Receptor  FSH

Adrenal Histology

1. Capsule 2. aldosterone 3. 4. glucocorticoids 5. medulla androgens

Medulla fight or flight

http://district.bluegrass.kctcs.edu/shirley.whitescarver/BIO139Lab http://cf.ydcdn.net/1.0.1.66/images/main/A5adrenalgland.jpg http://dspace.udel.edu:8080/dspace/bitstream/19716/1826/2/cenamc1.GIF

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Adrenal Medulla hormones

Also called adrenaline

(TH) (PNMT)

Rate limiting step Upregulated by cortisol

•Medulla is like a and releases secretions (messages) directly into blood

http://www.studyinukraine.eu/spinal-cord-spinal--and-autonomic-nervous-system-with-figures/

Adrenaline (metanephrines ) (and )

(pheos)

 Tumors in the adrenal medulla

 Make adrenaline hormones

 About 20% of patients with VHL will develop pheos

Flight or flight response  Petri et al British Journal of Surgery 2009

 Tumors in nerve bundles (ganglia) outside the adrenal gland

 Pheochromocytoma – leads to high , rapid heart  Similar to pheos rate, sweating, headache, anxiety, tremors, increased blood sugar  Rare in patients with VHL  Some people with pheo have no symptoms

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Testing for pheochromocytoma VHL and Pheo

 Blood test

 Plasma free metanephrines • VHL can be associated with bilateral  24hr urine test pheochromocytomas  Urine fractionated metanephrines • “Can I survive with one adrenal gland?”

 If found to have pheo, need pre-op blocking with • What about no adrenal special blood pressure medication called alpha glands? blocker before surgery

 Blocks effects of high adrenaline α-adrenergic  Ex: phenoxybenzamine, prazosin, doxazosin receptor http://cf.ydcdn.net/1.0.1.66/images/main/A5adrenalgland.jpg

Aldosterone Cortisol androgen Adrenal Insufficiency Androgens (Testosterone) cortisol

aldosterone  Caused by having no functional adrenal gland

 Fludrocortisone (Florinef)

 Aldosterone replacement aldosterone

 Salt/water balance hormone – maintains BP and potassium/sodium levels

Salt/water balance Steroid hormone Male physical  Hydrocortisone/prednisone cortisol Control BP Controls immune system, characteristics  Cortisol replacement BP, metabolism, bone health, …  BP/metabolism/blood sugar/other hormone

• Cortical sparing adrenalectomy sometimes used  Extra medication needed when sick or ill or before • We have medications to replace all of these hormones as needed surgery – “sick day rules” androgen

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Pancreatic Neuroendocrine Tumors VHL and the pancreas (PNETs)

 VHL increases risk of several types of  10-15% of all PNETs are associated with genetic pancreatic masses and syndromes  Mass is solid  ~9-17% of patients with VHL develop PNETs  is fluid filled  Most will be benign  Also seen in other syndromes  Multiple Endocrine Neoplasia Type 1 (MEN1)  One type of mass seen is a pancreatic  Neurofibromatosis Type 1 (NF1)  Tuberous Sclerosis Complex (TSC)

Pancreatic Neuroendocrine Tumors Functional (F) vs (PNETs) Non-functional (NF)

 F-PNETS  Guidelines suggest removal of pancreatic masses  Hormone elevated in blood + clinical syndrome over ~3cm in patients with VHL

 NF-PNETS  The vast majority of PNETs in patients with VHL  No clinical syndrome even if hormone detectable are benign and non-functional

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Pancreas Histology of pancreas

Exocrine pancreas

Endocrine pancreas Islet of Langerhans

http://biocrine.com/wp-content/uploads/2011/09/DAD2.png http://quasargroupconsulting.com/anatomy/pancreaseCells.gif

Pancreas Hormones – Exocrine pancreas – Pancreas messengers makes enzymes to digest food

http://quasargroupconsulting.com/anatomy/pancreaseCells.gif http://www.animatedpancreaspatient.com/en-pancreas/view/m501-s4-exocrine-pancreatic-insufficiency-epi-slide-show

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Exocrine pancreas – Pancreas Hormones – Pancreas messengers makes enzymes to digest food

Lack of nutrients for our body

Can cause bloating and

http://quasargroupconsulting.com/anatomy/pancreaseCells.gif http://www.animatedpancreaspatient.com/en-pancreas/view/m501-s4-exocrine-pancreatic-insufficiency-epi-slide-show

Blood Blood sugar sugar Blood sugar Blood Blood sugar sugar Blood Blood sugar sugar Blood Liver Liver Bloodsugar Pancreas Pancreas sugar Blood sugar Insulin Receptor Glucagon Receptor

Muscle

– blood sugar too high causing diabetes, weight loss, blood clots and a specific rash called necrolytic migratory  – low blood sugar, confusion, vision changes, unusual erythema. behavior, rapid heart beat, sweating, shakiness, amnesia, eating every few hours, waking up at night to eat to avoid symptoms.

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glucagon Vasoactive Intestinal insulin Energy Pancreas Polypeptide Pancreas (VIP) Somatostatin Liver Receptor Pancreas glycogen

VIP Receptor

Nerve

Decrease Pancreas acid Slow motility Secrete bicarb pituitary  – results in dysregulation of many hormones endocrine hormones. Lowers insulin leading to  VIPoma – causes huge amounts of very watery diarrhea leading to diabetes. Slows GI motility which can lead to , , low potassium and chloride intolerance to fat in the diet and leads to fatty diarrhea.

Pituitary picture from https://s-media-cache-ak0.pinimg.com/236x/3d/45/12/3d4512b044af3b0e5877a78499114d4e.jpg

Majority of PNETs in VHL are Abnormal functioning pancreas non-functional

 So why am I talking about the hormones?  Insulin is the main hormone of concern when absent  Insulin deficiency causes diabetes mellitus  A pancreas that is not functioning well or is absent  We can replace insulin to control diabetes due to surgery, may make too little hormone and enzymes  We can replace these with medications

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Blood Blood sugar sugar Blood Insulin sugar Abnormal functioning pancreas Blood Blood sugar sugar Blood sugar

Pancreas Liver  Absent exocrine pancreas is another concern Insulin Receptor  Absent enzymes cause bloating and discomfort because food cannot be digested well

Muscle  We can control the symptoms with giving back the enzymes before each meal

Exocrine pancreas – Summary makes enzymes to digest food

 Definition of a hormone  Adrenal gland  Adrenal hormones  Pancreas gland  Pancreas hormones and enzymes

http://cf.ydcdn.net/1.0.1.66/images/main/A5adrenalgland.jpg http://biocrine.com/wp-content/uploads/2011/09/DAD2.png http://www.animatedpancreaspatient.com/en-pancreas/view/m501-s4-exocrine-pancreatic-insufficiency-epi-slide-show http://quasargroupconsulting.com/anatomy/pancreaseCells.gif

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Questions?

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