The document summarizes key information about the pituitary gland and adrenal glands. It discusses the anatomy and physiology of the pituitary gland and its role in controlling the anterior pituitary via hormones from the hypothalamus. It also discusses specific pituitary tumors like prolactinomas and acromegaly. It then covers the anatomy and physiology of the adrenal glands, including disorders like Cushing's syndrome, Conn's syndrome, adrenal insufficiency, and adrenal tumors. Evaluation and treatment of various pituitary and adrenal pathologies are summarized.

1. Pituitary and
Adrenals
ABSITE STUDY

2. Pituitary Gland
 Anterior has no direct
blood supply. There is a
portal system with blood
passing through the
posterior pituitary first
 Located in the sella turcica

3. Hypothalamus
 Releases TRH, CRH, GnRH and dopamine into median eminence
 Hypothalamus  Posterior Pituitary  Anterior Pituitary
 Dopamine inhibits prolactin secretion

4. Posterior Pituitary (neurohypohysis)
 Axons from the supraoptic and paraventricular nuclei of the hypothalamus to the
posterior pituitary
 Secretes 2 hormones
 ADH
 Release is mostly controlled by the supraoptic nuclei
 Regulated by osmolar receptors in the hypothalamus released in response to high osmolarity
 Causes increased water absorption in the renal collecting ducts
 Oxytocin
 Release is mostly controlled by the supraoptic nuclei
 Mainly functions in uterine contraction and lactation
*Posterior pituitary does NOT contain cell bodies

5. Anterior Pituitary (adenohypohysis)
 Comprises 80% of the pituitary gland mass
 Releases ACTH, TSH, GH, LH, FSH, and prolactin
 Bi-temporal hemianopia occurs from compression of CN II
by a pituitary mass at the optic chiasm
 Bromocriptine can be used to treat all endocrine secreting
pituitary tumors EXCEPT an ACTH secreting tumor
 Trans-sphenoid resection is an option, but is
contraindicated with supra-cellar extension (dumbbell-
shaped) and massive lateral extension

6. Prolactinoma
 Most common pituitary tumor (most are micro-adenomas)
 Symptoms: amenorrhea, galactorrhea, infertility, poor libido, visual problems
 Dx: Elevated prolactin (>150), MRI and visual field testing
 Tx: Most will not require surgery
 Asymptomatic AND micro (≤1cm)= follow with MRI
 Symptomatoc OR macro (>1cm)=
 Bromocryptine or cabergoline (dopamine agonist)- 85% success (safe with pregnancy)
 If failed medical Tx, hemorrhage, significant visual loss, young female wanting pregnancy, or
CSF leak then trans-sphenoid surgery- 85% success (15% recurrence)

7. Acromegaly
 Excessive GH, which stimulates secretion of IGF-1
 Most are macro-adenomas
 Symptoms: jaw enlargement, macroglossia, headaches, HTN, amenorrhea,
DM, giganticism and visual problems
 Can have cardiac issues (valvular or cardiomyopathy)
 Dx: Elevated IGF-1 (random GH is NOT useful), MRI
 Tx: Trans-sphenoid resection is first choice if no invasion to surrounding
tissue
 Octreotide and bromocriptine can shrink tumor and improve symptoms
 Pegvisomant (GH receptor antagonist)

8. Pituitary Potpourri
 Sheehan’s Syndrome
 Pituitary insufficiency in mother after delivery
 Caused by pituitary ischemia 2/2 hypovolemia/shock following childbirth
 Symptoms: Most common initial sign in difficulty with lactation; can also have amenorrhea, adrenal
insufficiency or hypothyroid
 Posterior pituitary is usually unaffected 2/2 direct blood supply
 Tx- exogenous replacement of deficiency
 Craniopharyngioma
 Most commonly occur in children 5-10yo and are benign
 Calcified cyst near anterior pituitary, remants of Rathke’s pouch (diverticulum arising from the embryonic
buccal cavity, from which the anterior pituitary gland develops)
 Symptoms: headaches, growth failure, visual problems, endocrine abnormalities, hydrocephalus
 Tx: Surgery if symptomatic (DI is a common post-op complication)
* Keep metastatic disease in mind (women with hx of breast CA and men with hx of lung CA)

9. Adrenal Anatomy
 Weighs 4-6 grams
 Arterial Supply – Inferior Phrenic, Unnamed Aorta, Renal
A. Branches
 Anterior and posterior surface are avascular
 Venous Drainage
 L Adrenal
 Drains from the lower pole into the L Renal V
 R Adrenal
 Drains the anterior surface of the gland into the posteriolateral
IVC; short
 Innervation
 Cortex= none
 Medulla= splanchnic

10. Adrenal Anatomy
 Cortex
 Originates from the mesoderm. From the adrenocortical ridge near the gonads during the 5th week
of gestation. Can have adrenal nests in the ovaries, testes or kidneys
 3 zones
 Glomerulosa (salt)– Aldosterone
 Fasciculata (sugar)– Cortisol
 Reticularis (sex)– Testosterone/estrogen
 Medulla
 Originates from the ectoderm from the neural crest
 Epinephrine (20%) – Requires PMNT – medulla or zuckerkandl (paraganglia near aortic bifrication)
 NorEpi (80%)

11. Physiology
 Renin-angiotensin-aldosterone pathway
 Decrease Na and volume stimulate renin secretion from the JXA
 Renin converts angiotensinogen to angiotensin I
 Angiotensin I is converted to angiotensin II by ACE in the lungs
 Angiotensin II then causes vasoconstriction and aldosterone secretion
 Aldosterone causes sodium retention and potassium excretion in the renal tubules
 Cortisol pathway
 Hypothalamus releases CRH
 CRH stimulates ACTH from the anterior pituitary
 ACTH stimulates cortisol synthesis and release from adrenal
 Cortisol acts in negative feedback to inhibit CRH/ACTH and also has effects on hepatic gluconeogenesis,
glycogen synthesis, protein catabolism, lipolysis
 Also causes hyperglycemia, impaired wound healing, collagen and bone loss

12. Primary Hyperaldosteronism
Conn’s Syndrome
 HTN, Hypokalemia, Polyuria, Polydipsia, Muscle Weakness
 Adenoma (60%), Bilateral Hyperplasia (40%)
 Elevated Aldosterone, Low Renin (Ratio >20:1)
 Elevated urine aldosterone following a salt loading test
 CT – adenoma vs hyperplasia
 If microadenoma (<1cm) or no mass then adrenal venous sampling to distinguish unilateral
adenoma vs B/L hyperplasia
 If macroadenoma (>1cm)- resect
 Adenoma- Resect (preop with spironolactone to normalize potassium)
 18-hydroxycorticosterone elevated (>100)with adenomas and low in hyperplasia
 Hyperplasia – Medical Tx – Spironolactone, Ca2+Blockers, Potassium

13. Hyperaldosteronism
 Secondary
 Secondary to renovascular HTN
 Elevated aldosterone AND renin
 Tertiary
 From diuretic use

14. Hypercortisolism
Cushing’s Syndrome
 Cushing’s Syndrome – Adrenal Hyperplasia, adenoma, carcinoma or ectopic ACTH or
coticotropin releasing factor (small cell carcinoma)
 Cushing’s Disease – ACTH Hypersecretion Pituitary Adenoma (60-70%)
 Weight gain, HTN, DM, Centrpetal obesity, hirsutism, striae, osteopenia
 24H Urinary/Cortisol (not serum cortisol)
 Low Dose Dexamethasone – 1mg
 Low Plasma ACTH – adrenal adenoma, hyperplasia, carcinoma
 High Plasma ACTH – Pituitary adenoma or Ectopic
 High Dose Dex – 2mg Q6h for 48H
 Etopic – Will not be suppressed
 Pituitary adenoma – Will be suppressed

15. Hypercortisolism Work-up

16. Hypercortisolism
 Source
 If adrenal suspected  CT abdomen (adrenal protocol)
 If pituitary suspected  MRI sella turcica
 If ectopic suspected  CT chest (suspected small cell carcinoma)
 Adrenalectomy
 Unilateral for adenoma or carcinoma
Need perioperative steroid coverage
 Bilateral for hyperplasia or in some cases of secondary
hypercortisolism without identification of primary tumor
Will require steroid replacement
*Exogenous steroid use is the MCC of cushingoid appearance

17. Congenital Adrenal Hyperplasia
Deficiency Aldosterone Testosterone
21-Hydroxylase Normal ↑
17-α-Hydroxylase ↑ Normal
11-α-Hydroxylase ↑ ↑
 Aldosterone
 HTN and hypokalemia
 Tertiary
 Virilization

18. Adrenal Insufficiency
 Primary – Most common is autoimmune
 Infection – Histoplasmosis, TB (MC in developing world), Meningococcus (Waterhouse Friderichsen
syndrome)
 Secondary-Iatrogenic; Exogenous Corticosteriods, most common
 Labs
 Elevated ACTH, Hyperkalemmia, Hyponatremia, Hypochloremia, Hypoglycemia, Acidosis
 Cosyntropin Stim Test – 250micrograms IV
 Cortisol level at 30-60 min if <18 is suggestive of insufficiency
 Hydrocortisone will interfere with this; can use dexamethasone
 Tx – Glucocorticoids and hydration
 Addisonian crisis
 Symptoms: hypotension, tachycardia, refractory shock
 Treat immediately. Don’t wait for labs

19. Evaluating Adrenal Mass
 Functioning or Non Functioning
 Majority are non functioning, if functioning resect regardless of size
 Blood/Urine levels – VMA, Catecholamines, Metanephrines
 Benign or Malignant
 Vast Majority are benign
 Carcinomas are rare – 50% stage IV at diagnosis
 Primary or Secondary
 Metastatic lesions from lung, breast, renal cell, melanoma, prostate, colon

20. Adrenal Incidentalomas
 Adrenal Mass discovered during imaging for unrelated reason
 DDx – Cortical adenoma, carcinoma, pheo, ganglioneuroma, cyast,
hemorrhage, fibrosis
 Evaluation
 Plasma Cortisol, Estradiol, testosterone, androstenedione
 24H urinary cortisol
 Low Dose Dexamethasone cortisol suppression test – should suppress pituitary
ACTH yielding decreased levels. No depression means cushing’s
 Renin/Aldosterone ratio
 Urinary catecholamines, metanephrines, VMA

21. Adrenal Incidentaloma
 Benign
 Round, smooth, homogenous
 Increased fat content
 Malignant
Irregular, focal hemorrhage/necrosis
 Percutaneous Bx
 Contraindicated if surgery indicated
 Perform if multiple masses present with primary unknown
 Not appropriate for hormonally active large tumor or if pheo is suspected

22. Indication for Surgery
 Hyperfunctioning – Conn, Cushing, Pheo
 Malignancy – based on CT
 Size
 Non functioning <cm 4 – Do not operate if benign imaging characteristics (smooth,
homogenous, <10 HU, >60% washout on 15min delay phase)
 CT at 3, 6, 12 months, resect if increase in size
 Non functioning 4-6cm – Individualize,
 <40yo resect, elderly observe
 Non Functioning - >6cm – Operate due to increased risk of malignancy unless clearly
benign cyst or myolipoma on imaging
 Begin metastatic workup

23. Adrenal Cortical Carcinoma
 Rare, aggressive, 60% hyperfunctioning
 Women, 40-50yo, Large (90%>6cm)
 Left sided more common
 Can present with liver mets
 Tx – Open Adrenalectomy (concern for tumor spillage), resection en bloc
 5 yr survival 20%
 No benefit to chemo/rads
 If unresectable, consider mitotane (adrenolytic agent that binds mitochondrial
proteins  adrenal atrophy and tumor death)

24. Pheochromocytoma
 Tumor of adrenal medulla or extra adrenal adrenergic tissue (neuroectodermal origin)
 Rule of 10s – 10% familial (MEN II A/B, von Hippel Lindau, Neurofibromatosis), extraadrenal
(secrete NE), multiple, bilateral, pediatric, malignant
 Sx- paroxysmal HTN, tachycardia, HTN, palpitations, flushing, sweating
 Dx – Initial plasma catecholamines/metaneprhines
 24h Urinary catecholamines (dopamine, epi, NE) and metabolites (VMA, metanephrines)
 CT abdomen
 MRI – bright T2
 Pheo Scan – MIBG I131 – concentrates in adrenergic vesicles – identify extra adrenal
 Preop – Alpha blockade first – Phenoxybenzamine (7-10d preop), then beta blockade 48 H
prior, hydrate
 If beta blockade first yields unopposed alpha yields HTN crisis
 Tx: Laparoscopic adrenalectomy

25. MEN
 MEN I
 Pituitary – Prolactinoma
 Initial Tx with bromocriptine; rare surgical excision
 Parathyroid – 4 gland hyperplasia (MC manifestation of MEN I)
 Pancreas – Neuroendocrine (MC non-functional)
 MEN IIA – Autosomal Dominant
 Medullary Thyroid Cancer (100%)
 Pheochromacytoma
 Hyperparathyroid
 MEN IIB – Autosomal Dominant
 Medullary Thyroid Carcinoma (100%)
 Pheochromacytoma
 Mucosal Neuroma/Marfanoid Habitus
Genes:
MEN I-
MEN II-
MTC-
MEN I
RET
RET, NTRK1