Endocrine Flashcards
MEN 1
Wermer’s Syndrome
Deletion 11q12-13
3 Ps
Parathyroid hyperplasia (90%) Pancreatic (and duodenal) islet cell tumors (50%) Pituitary adenomas (25%) - prolactinoma = #1
MEN 2A
Sipple’s Syndrome
RET oncogene mutation on chrom 10q11.2
Missense mutations on chrom 1
Medullary thyroid carcinoma (100%) - 20% of all medullary cancers are from MEN
Pheo (33%) - usually b/l
Parathyroid hyperplasia (50%)
MEN 2B
Mucosal neuroma may be earliest sign (100%) - hypertrophied lips, thickened eyelids
Medullary thyroid carcinoma (85%)
Pheo (50%)
Marfanoid habitus - skeletal abnormalities of spine (kyphosis), pectus excavatum
Tx for MEN syndromes
Perform subtotal or total parathyrodectomy with autotransplantation for parathyroid hyperplasia (MEN 1 and MEN 2A)
Perform total thyroidectomy for medullary thyroid cancer (MEN 2). May require nodal dissection if palpable nodes present
Thyroid development
Thyroid develops at base of tongue btw first pair of pharyngeal pouches, in area called foramen cecum
Thyroid gland descends down midline to its final location and develops bilobed organ with isthmus btw lobes
Remains connected to floor of pharynx via thyroglossal duct, which obliterates around month 2. May fail to go away and form a cyst or fistula instead. Usually in kids and should be removed with surg
A pyramidal lobe can be seen in 50-80% of population and represents remnant of distal thyroglossal tract. It extends superiorly from isthmus
Thyroid anatomical relationships
Anterior = strap muscles (sternohyoid, sternothyroid, thyrohyoid, omohyoid)
Posterior = trachea
Posterolateral = common carotids, IJ veins, vagus nerves
Parathyroid glands on posterior surface of thyroid and may be IN the capsule
Lymphatic drainage of thyroid
To IJ nodes
Intraglandular lymphatics connect both lobes, explaining the high frequency of multifocal tumors in thyroid
Vasculature of thyroid
Arteries:
1) Superior thyroid (each side)
- first branch of external carotid at level of carotid bifurc
2) Inferior thyroid (each side)
- from thyrocervical trunk of subclavian artery
3) Ima (sometimes)
- from aortic arch or innominate artery
Venous:
1) Superior thyroid vein (each side)
- drains into IJ
2) Middle thyroid vein (each side)
- Drains into IJ
3) Inferior thyroid vein (each side)
- drains to brachiocephalic vein
Innervation of thyroid
***1) R recurrent laryngeal nerve branches from R vagus nerve under R subclavian artery, and ascends (posterior to thyroid) to the larynx btw the trachea and esophagus
L recurrent larygneal nerve branches from L vagus, loops under aortic arch and ascends along tracheoesophageal groove to larynx.
Both innervate muscles of the true vocal cords
2) Sympathetic - superior and middle cervical sympathetic ganglia
3) Parasympathetic - from vagus nerves via branches of laryngeal nerves
Clinical importance of recurrent laryngeal nerve
It innervates all the intrinsic muscles of larynx except the cricothyroid (superior laryngeal nerve) and provides sensory innervation to mucous membranes below the vocal cords
It can be damaged during thyroid surgery so surgeon needs to know its course.
Damage produces ipsilateral vocal cord paralysis and results in hoarseness or sometimes SOB due to narrowed airway
TSH function
Causes increased formation of TH
Release of TH into circ within 30mins
Increased TH level in blood then feeds back to pit and causes decreased TSH secretion
Assessment and function of thyroid hormones
If T4 production goes up, both total T4 and free T4 go up
If T4 production drops, total and free T4 drop
If amount of thyroid-binding globulin changes, only the free T4 changes (not the total)
How much hormone do thyroid follicles store?
enough to last 2-3 months
Thus, there is no need to worry about postop hypothyroid patient who is NPO…they can resume taking their synthroid when they begin PO diet
Congenital anomalies of thyroid
Persistent sinus tract remnant of developing gland = thyroglossal cyst - may occur anywhere along course as a midline structure with thyroid epithelium, usually between isthmus and hyoid bone
- # 1 congenital anomaly
- few symptoms but may be infected
- Easier to see when tongue is sticking out
- surgical treatment - excise the duct remnat and central part of hyoid bone (Sistrunk’s operation)
Complete failure to develop
Incomplete descent = lingual thyroid or subhyoid position (if gland enlarges, patient will have earlier respiratory symptoms)
- before surgery to remove it make sure patient has other functioning thyroid tissue (70% of lingual thyroids are the only functioning thyroid)
Excessive descent = substernal thyroid
Malformation of branchial pouch
Causes of hyperthyroidism
1) Grave’s
2) Toxic nodular goiter
3) Toxic thyroid adenoma
4) Functional metastatic thyroid cancer
5) Struma ovarii (abnormal thyroid tissue in ovary)
Grave’s disease def
1 cause of hyperthyroidism in USA
Autoimmune that causes excess of TH to be produced due to presence of thyroid-stimulating immunoglobulins that stimulate production of TSH
2% of american women; 6x more common in women
Onset 20-40
Families with Graves have higher risk of other AI conditions (diabetes, Addison’s) and other thyroid disorders too
Signs of Graves
Nervousness, increased sweating, tachy, goiter, pretibial myxedema, tremor (90%)
Heat intolerance, palpitation, AFib refractory to treatment, weight loss, fatigue, dyspnea, weakness, increased appetite, exophthalmos, thyroid bruit (50-90%)
Amenorrhea, low libidio and fertility
Dx of Graves
TFTs - high T3 and/or T4 and low TSH (neg feedback of high hormone levels)
Radioactive iodide uptake test (RAIU) - scan shows diffusely increased uptake
Tx of Graves
Antithyroid drugs
Radioiodide ablation with I-131
Subtotal or total thyroidectomy
Choosing a Tx for Graves
1) Consider age, severity, size, surg risk, treatment side effects and comorbidities
2) Radioablation is #1 choice
- indicated for small or medium goiters if med therapy has failed, or if other options are contraindicated
- Most patients become euthyroid within 2 months
- Most ultimately require thyroid hormone replacement (Levo)
- Complications include exacerbation of thyroid storm initially
- Contraindicated in pregnancy, women of childbearing age and newborns
3) Surgery is indicated when radioablation is contraindicated or if medical management cannot be used
- Patients should be euthyroid prior to excision
- advantage over radioablation is immediate cure
4) Medical therapy
- B blockers for symptomatic relief
- Antithyroid drugs (PTU, methimazole) inhibit hormone production and peripheral conversion of T4 to T3
- KI reduces hormone production, used to shrink gland prior to surgery
- High recurrence rate with medical tx
- may cause side effects like rash, fever, peripheral neuritis
- Patients relapse if meds are D/C’d
- Check TFTs after any treatment
Risks of thyroid surgery
Recurrent laryngeal nerve injury
Hypoparathyroidism
Persistent hyperthyroidism (with subtotal thyroidectomy)
Toxic nodular goiter
“Plummer’s Disease”
Causes hyperthyroidism but without the extrathyroidal symptoms
Treatment is surgical since medical therapy and radioablation has a high failure rate
Solitary nodule = lobectomy
Multinodular goiter = subtotal thyroidectomy
Thyroid storm (thyrotoxicosis)
Life-threatening extreme exacerbation of hyperthyroidism precipitated by surgery on an inadequately prepared patient (incomplete B blockade and noneuthyroid patient), infections, labor, iodide administration or recent radioablation
Fever, tachy, muscle stiffness, disorientation/AMS
50% with thyroid storm develop CHF
20-40% mortality
Best way to treat is by avoiding it. ppx = achieving euthyroid state preop
Tx = fluids, antithyroid meds, B-block, corticosteroids, sodium iodide or Lugol’s solution (KI) and a cooling blanket
Causes of hypothyroidism
Autoimmune thyroiditis
Iatrogenic: s/p thyroidectomy, s/p radioablation, 2/2 antithyroid meds
Iodine deficiency
Signs of hypothyroidism
Infants/peds: down’s like facies, failure to thrive, mental retardation immediate tx with thyroid hormone will minimize neuro and intellectual effects
Adolescents/Adults: (particularly when due to AI)
- 80% female
- brady, low CO, hypotension, SOB 2/2 effusions
- fatigue, weight gain, cold intolerance, constipation, menorrhagia, low libido and fertility
Less common = yellowish skin, hair loss, tongue enlargement
Dx of Tx of hypothyroidism
H&P
Low T4, T3
High TSH if primary
Low TSH if secondary
Confirm with TRH challenge - TSH will not respond in secondary hypo
Thyroid autoantibodies in AI
Low Hct
ECG may show low voltage or flat/inverted T waves
Tx = thyroxine PO or IV emergently if patient presents in myxedema coma
Thyroiditis (Acute)
Infectious etiology = strep pyogenes, staph aureus, pneumococcus (usually via lymphatics from local infection)
Risk = female sex, goiter, thyroglossal duct
Signs = unilateral neck pain and fever, euthyroid state, dysphagia
Tx = IV ABx and surgical drainage
Thyroiditis ( Subacute/de Quervain’s)
Post viral- URI
Risk = female
Signs = fatigue, depression, neck pain, fever, unilateral swelling of thyroid with overlying erythema, firm, and tender thyroid, transient hyperthyroidism usually preceding hypothyroid phase
Dx = made by H&P
Tx = usually self-limiting (within 6w)
= manage pain with NSAIDs
10% become permanently hypothyroid
Thyroiditis (Chronic/Hashimoto)
Autoimmune
Risk = down’s syndrome, Turner, familial Alzheimer’s, hx of radiation therapy as child
Signs = painless enlargement of thyroid, neck tightness, presence of other AI diseases
Dx = H&P + labs
- Circulating antibodies against microsomal thyroid cell, thyroid hormone, T3, T4, or TSH receptor
Path = firm, symmetrical enlargement; follicular and Hurthle cell hyperplasia; lymphocytic and plasma cell infiltrates
Tx = thyroid hormone (usually results in regression of goiter). With failure of medical tx, partial thyroidectomy is indicated
20% present with hypothyroidism at time of dx. A euthyroid state is more common
Riedel’s Fibrosing thyroiditis
Rare
Fibrosis replaces both lobes and isthmus
Risk = other fibrosing conditions like retroperitoneal fibrosis or sclerosing cholangitis
Signs = usually remain euthyroid; neck pain, possible airway compromise; firm, nontender enlarged thyroid
Dx = often bx required to rule out carcinoma or lymphoma
Path = dense, invasive fibrosis of both lobes and isthmus. May also involve adjacent structures
Tx = with airway compromise: Isthmectomy
Without: medical treatment with steroids
Workup of thyroid nodule
1) Get TSH/T4
- Low TSH, High T4? Hyperthyroid
- Normal? Euthyroid
2) If hyperthyroid:
- it’s prob a functioning adenoma and NOT cancer
- Get RAIU
- Hot = functioning adenoma (I2 ablation, surgery, medical management)
- Cold = ? (Get US guided FNA)
3) If euthyroid:
- Get US guided FNA
4) the FNA
- Benign
- ? (get RAIU, if functioning then benign. not not then cancer)
- Cancer (surgery and I2 ablation)
- Get a staging workup if cancer
Most common thyroid cancer
Papillary (80-85%) - it’s 75% of peds thyroid cancer
Risk factors for the dif thyroid cancers
Papillary = radiation
Follicular = dyshormonogenesis
Medullary = MEN II in 30-40%
Anaplastic = Prior diagnosis of well-differentiated thyroid cancer. Iodine deficiency
Age groups for each thyroid cancer
Papillary = 30-40
Follicular = 5-20
Medullary = 50-60
Anaplastic = 60-70
Sex ratios for each thyroid cancer (F/M)
papillary = 2/1
Follicular = 3/1
Medullary = 1.5/1
Anaplastic = 1.5/1
Signs of papillary thyroid cancer
Painless mass
Dysphagia
Dyspnea
Hoarseness
Euthyroid
Signs of follicular thyroid cancer
Painless mass
Rarely hyperfunctional
Signs of medullary thyroid cancer
Painful mass
Palpable LN (15-20%)
Dysphonia
Dyspnea
Hoarseness
Signs of Anaplastic thyroid cancer
Rapidly enlarging neck mass (large mass at presentation)
Neck pain
Dysphagia
Hard, fixed LN (50%)
Diagnosis of each thyroid cancer
Papillary = FNA, CT/MRI to assess local invasion
Follicular = FNA, CT/MRI to assess local invasion
Medullary = FNA, presence of amyloid is diagnostic. Check for calcitonin
Anaplastic = FNA
Mets for each thyroid cancer
Papillary = lymphatic (5% at time of prez)
Follicular = hematogenous
Medullary = lymphatic (local neck and mediastinal nodes). Local (into trachea and esophagus)
Anaplastic = Aggressive local disease. 30-50% have synchronous pulmonary mets at time of dx
Tx of papillary thyroid
1) Minimal cancer ( 1.5cm
3) For + LN, modified radical neck dissection
4) I-131 ablation or thyroid suppression (with thyroid hormone) for patients with residual thyroid tissue or LN mets
Tx for follicular thyroid
1) Minimal cancer ( 4cm
3) For +LN, modified radical neck dissection
4) I-131 ablation for patients with residual thyroid tissue or LN mets
Tx for medullary thyroid
1) Sporadic (80%): total thyroidectomy
2) Familial (20%): Total thyroidectomy and central neck node dissection
3) No value for I-131 ablation
4) Follow patients with calcitonin levels
Tx for anaplastic thyroid
1) Debulking resection of thyroid gland and adjacent structures
2) XRT
3) Doxorubicin-based chemo
Prognosis of papillary thyroid
worse for older patients and those with distant mets
Presence of +LN not strongly correlated with overall survival
10yr = 74-93%
Prognosis of follicular thyroid
Worse for older patients, distant mets, tumor > 4cm, high grade tumors
Presence of +LN not strongly linked with survival
10yr = 60-80%
Prognosis of medullary thyroid
10yr = 70-80%
Prognosis for anaplastic thyroid
Poor
Median = 4-5months
Position of parathyroids
Superior glands always next to superior lobes of thyroid (from 4th pharyngeal pouch)
Inferior glands more variable (posterior/lateral to thyroid and below inf thyroid artery) - from 3rd pouch
Not uncommon to have a “missing” inferior gland
Most common location for inferiors = thymus. Others could be intravagal, groove in carotid sheath
Histology of parathyroids
Chief cells - produce PTH
Function of parathyroids
Regulate calcium and P metabolism by PTH, Vit D, and calcitonin
Primary organ systems involved = GI, bone, kidney
PTH
Serum calcium levels regulate secretion of cleaved PTH by negative feedback
1) Bone
- stimulates osteoclasts (more bone resorption)
- Inhibits osteoblasts (less bone production)
- This causes release of Ca and P
2) Kidney
- More reabsorption of Ca
- More P excretion
3) GI
- Stimulates hydroxylation of 25-OH D to 1,25 OH D
- 1,25 OH D increases intestinal absorption of dietary calcium (from duodenum mainly) and P
- Promotes mineralization
- Enhances PTH’s effect on bone
Hypercalcemic crisis
Calcium > 13 and symptomatic
Treat with saline, furosemide, bisphosphonates, and if needed antiarrhythmic agents
Calcitonin
Secreted by thyroid C cells
Inhibits bone resorption (inhibits calcium release)
Increases urinary excretion of Ca and P
Works as counterregulatory hormone to PTH
Hyperparathyroidism (Primary) definition
Due to overproduction of PTH, causing increased absorption of calcum from intestines, increased vit d3 production, and decreased renal calcium excretion. This raises overall serum Ca and lowers P
Hyperpara (primary) incidence and risk
1/4000
MEN I
MEN IIA
history of radiation
Signs of hyperpara (primary)
Stones, bones, groans, moans
Kidney stones
Bone pain, pathologic fractures, subperiosteal resorption
Groans: nausea, vomit, muscle pain, constipation, pancreatitis
Moans: lethargy, confusion, depression, paranoia
Causes of primary hyperpara
Solitary adenoma (85-90%)
4-gland hyperplasia (10%)
Cancer (
Workup and dx for primary hyperpara
Preop imaging = US, sestamibi scan, CT/MRI, operative exploration
Dx:
1) Elevation of PTH with inappropriately high serum Ca (normally a high Ca should lower PTH)
2) Check urine for calcium to rule out diagnos of familial hypocalciuric hypercalcemia (will be low if FHH, high if primary hyperpara)
Tx for primary hyperpara
1) Solitary adenoma - solitary parathyroidectomy with neck exploration to ID +/- biopsy 3 remaining glands. If preop sestamibi is done to localize area, no need for neck exploration
2) Multiple gland hyperplasia - remove 3 glands, or all 4 with reimplantation of at least 30g of parathyroid tissue in forearm or other site to retain function (makes it easier to resect additional parathyroid gland if hyperparathyroid state persists)
Outcomes:
First op = 90% success rate
Reoperation = 90% success rate if remaining gland is localized preop
Secondary hyperpara info
Increased PTH due to hypocalcemia that is the result of chronic renal failure (Phosphate retained leads to low calcium), GI malabsorption, osteomalacia, or rickets
Signs of secondary hyperpara
Bone pain from renal osteodystrophy and pruritis
Patients often asymptomatic
What other conditions should you consider in a patient with hypercalcemia?
R/O malignancy
ESP colon, lung, breast, prostate, head, neck, MM
Some tumors secrete PTHrP
Dx of secondary hyperpara
Made by labs in asymptomatic patients
usually due to 4 gland hyperplasia
Tx of secondary hyperpara
1) Nonsurgical - In renal failure patients, correct Ca and P.
- Restrict P intake, treat with P-binders and Ca/Vit D supplements. Adjust dialysate to maximize Ca and minimize aluminum
2) Surgical - indicated for intractable bone pain or pruritis, or pathologic fractures, with failure of medical therapy
- No role for parathyroid surg in secondary hyperpara
- Maybe renal transplant
Tertiary hyperpara
Due to persistent hyperparathyroidism after treatment for secondary hyperpara.
Due to autonomously functioning parathyroid glands that are resistant to negative feedback from high Ca levels. Usually s/p renal transplant
Usually a short-lived phenom
If persistent, surgery is indicated (3.5 gland resection)
Familial hyperpara
MEN 1/2A
High recurrence rate
Total resection with forearm reimplantation is indicated to facilitate potential reoperation if HPTH persists
Patients with sporadic 4 gland hyperplasia may undergo total resection with reimplantation or 3 gland excision
Hypopara etiologies
Uncommon
Usually from surgery: Following total thyroidectomy, usually transient and treated if symptoms develop
Could be congenital absence of all 4 glands
Could be DiGeorge - no parathyroid and no thymus
Could be functional - chronic hypoMg
Hypo para signs
Numbness and tingling of circumoral area, fingers, toes
Anxiety, confusion
may progress to tetany, hyperventilation, seizures, heart block
Tx for hypo para
Supplement with PO Ca and Vit D (to help GI absorption)
Pseudohypoparathyroidism = familial disease causing resistance of PTH at target tissue. Patient remain hypoCa and hyperP despite bone resorption from elevated PTH. Tx = Ca and Vit Supplements
Signs of parathyroid cancer
40-50% present with firm, fixed mass that is palpable
Extremely high Ca and PTH. Usually has high levels of hCG (tumor marker)
Neck pain, voice change (due to lesion in recurrent laryngeal nerve)
Tx for parathyroid cancer
En bloc surg resection of mass and surrounding structures, along with i/l thyroid lobectomy and i/l LN dissection
Postop XRT and chemo not usually helpful
Post op complications = recurrent laryngeal nerve damage, severe hypocalcemia (hungry bone syndrome)
5y survival = 70%
Chvostek sign
HypoCa
Contraction of facial muscles when tapping on facial nerve
Trousseau sign
HypoCa
Development of carpal spasm by occluding blood flow to forearm
Adrenal anatomy
Retroperitoneal
T11
Branches of aorta, inferior phrenic and renal arteries
Venous = R side drains to IVC. L drains to L renal vein
Histo of adrenal glands
Cortex
Glomerulosa: aldosterone (salt)
Fasiculate: Steroids, cortisol (sugar)
Reticularis: Androgens/estrogen (sex)
Adrenal adenoma
Most are unilateral
If adrenal gland 6cm, surgically resect due to increased risk of adrenocortical carcinoma
Adrenal cortical carcinoma signs
Very rare
Women more than men, peak
Dx of Adrenal Cortical Carcinoma
24h urine for cortisol, aldosterone, catecholamines, metanephrine, VMA, 17-OH corticosteroids, 17-ketosteroids
CT (lesions > 7mm) or MRI (esp to assess IVC invasion)
CXR to r/o pulm mets
Tx for Adrenal Cortical Carcinoma
1) Radical en block but only 1/3 of adrenal carcinomas are operable
2) If resection not done, debulk to reduce amount of cortisol-releasing tissue
3) Bone mets should be palliated with XRT
4) No role for chemo
5) Monitor steroid hormone levels postop
6) Recurrence also warrants resection
7) recurrence: lungs, LN, liver, peritoneum, bone
- in 10% of b/l cases, patients develop Nelson’s syndome (excess ACTH from pit adenoma. This causes visual issues due to mass effect, hyperpigmentation (more melanocyte-stimulating hormome) and amenorrhea as well)
Prognosis for ACC
70% present in stage 3 or 4
5yr = 40% for complete resection
If local invasion, median = 2-3y
Workup for Cushing’s Syndrome
Low THen High
1) 24h urine cortisol elevated then Low dose (single dose) dexa suppression
If this fails, we have Cushing’s syndrome (too much cortisol being made - we need to find where it’s coming from)
Is this cortisol driven (ACTH independent) or driven by ACTH?
2) get ACTH level
If normal/not high then cortisol itself is driving it.
This is a primary adrenal tumor
Get CT to find it then resect
OR they’re eating cortisol
If ACTH is high then it’s ACTH driven
3) Get High Dose (8mg) Dexa suppression (If ACTH was high)
If it suppresses cortisol then it’s Cushing’s Disease (Ant pit issue. MRI and resect)
If it fails then it’s ectopic ACTH - CT or PET everything to find it
The tests do steroid dose at 11PM and measurement at 8AM (low dose)
What is the most common cause of ectopic ACTH production?
1 = Small cell lung cancer (oat cell carcinoma)
Addison’s Disease def
Adrenal insufficiency
Primary = Due to destruction of adrenal cortex with sparing of medulla
Secondary = Failure due to hypothalamic or pituitary abnormalities
Causes of Primary Addison’s Disease
1) Post-adrenalectomy
2) AI
3) TB
4) Fungal infx
5) AIDS
6) metastatic cancer
7) Familial glucocorticoid deficiency
Causes of secondary Addison’s
1) Exogenous steroids (#1 - longterm for medical use or for illicit use)
2) Craniopharyngioma
3) Pituitary surgery or irradiation
4) Empty sella syndrome
Abruptly stopping longterm steroid use may cause adrenal insufficiency too. 6 months may be required for intrinsic controls to come back online
Addisonian crisis
Acute situation due to some extrinsic stressor like infection or surgery
Signs of Addison’s
Nause, vomiting
Abdominal pain
Tachy
Weight loss
Fatigue
Lethargy
Hyperpigmentation (low levels of cortisol cause increased pit production of proopiomelanocortin - POMC - which is a precursor to ACTH and MSH)
Fever and hypovolemic shock in crisis
Dx of Addison’s
1) HypoNa, HyperK (due to low aldosterone which is normally produced by adrenals)
2) ACTH stimulation test - give ACTH and measure cortisol level after 30mins. If adrenal failure is present, there will be no increase in cortisol
3) Baseline ACTH level is elevated in patients with primary failure due to absence of negative feedback
Tx of Addison’s
1) Glucocorticoid therapy for primary and secondary causes
2) Additional mineralocorticoid therapy for primary cause
3) Addisonian crisis - Volume (D5NS) and IV glucocorticoids
Stress dose steroids perioperatively?
Patients with Addison’s OR anyone who has been on steroids for more than 6 months prior to surgery should get periop stress dose steroids
Causes of hyperaldosteronism
Primary (too much aldosterone secretion) = Conn’s Syndrome
- Aldosterone-secreting tumor (66%)
- Idiopathic adrenocortical hyperplasia (30%)
Secondary (due to high renin leading to high aldo)
- Renal artery stenosis
- Cirrhosis
- CHF
- Normal pregnancy
Signs of hyperaldo
HTN
Muscle weakness and cramping
HA
Polyuria
Polydipsia
HypoK
Dx for hyperaldo
Primary/Conn’s
1) Diastolic HTN without edema
2) High plasma aldo
3) Normal or low plasma renin
4) HypoK, high urineK (off antihypertensive meds)
5) Post-captopril plasma aldo:
- normally results in low aldo
- diagnostic of hyperaldo if ratio > 50
6) Imaging
- CT picks it up if >1cm. If there is an aldosteronoma, opposite adrenal appears atrophied
- Iodocholesterol scan - picks up 90% of aldosteronomas and shows how functional they are. Hyperplasia will present as b/l hyperfunction versus unilateral for tumor
- if all imaging inconclusive, maybe sample adrenal vein for aldo and cortisol pre and post ACTH
- U/l elevation of aldo or ald/cortisol ratio indicated aldo-secreting adenoma
- B/l elevation of aldo is consistent with hyperplasia
Caveat to eval for Conn’s
Make sure patient isn’t just an uncontrolled hypertensive on K wasting diuretics
Tx for hyperaldo
Primary
1) Hyperplasia - medical treatment with spironolactone, nifedipine, amiloride and/or other antihypertensive. NO SURG
2) Adenoma - Lap adrenalectomy
Outcomes: most patients become normotensive and normokalemic with tx. 20-30% have recurrent HTN in 2-3 years
Secondary
1) Treat underlying cause
Hypoaldo
Decreased aldo without a change in cortisol production
Causes = Congenital error of aldo synthesis, failure of glomerulosa (AI), s/p adrenalectomy, drug inhibition
Signs = postural hypotension, persistent and severe hyperK, muscle weakness, arrhythmias
Tx = mineralocorticoid replacement
Neuroblastoma def
A medullary tumor of adrenals
- Embryonal neural crest tumor mainly in children (small round blue cell tumor)
- # 4 most common peds malignancy
- Can occur anywhere along sympathetic chain (50% in adrenal, 25% paraspinal ganglia, 20% thorax, 5% pelvis)
- May spontaneously differentiate and regress
- Aggressive tumor that commonly presents with distant mets. In 50% of infants and 66% of older children (to LN, bone, liver, subQ)
Diseases associated with neuroblastoma
Neurofibromatosis
Beckwith-Wiedermann Syndrome
Trisomy 18
Signs of neuroblastoma
Abdominal or flank mass
Respiratory distress
SubQ blue tumor nodules (blueberry muffin sign)
Dx of neuroblastoma
Imaging CT for staging. MRI
Urinary tumor markers - high 24h levels of homovanillic acid, vanillylmandelic acid and metanephrines
Tx of neuroblastoma
Localized (stage 1/2) - surg resection
Nonlocalized disease (stage 2) - surg resection with chemo +/- radiation
Mets (4) - surg resection with chemo +/- radiation
LN mets warrants XRT
prognosis of neuroblastoma
Mortality lower when diagnosed within first year of life
5y survival = 90% for disease confined to primary site, 20-40% for disseminated disease
10% rule for pheocrhomocytoma
10% malignant 10% familial 10% extra-adrenal (morel likely to be malignant. Often at organ of Zuckerkandl to left of aortic bifurcation at IMA) 10% bilateral (more likely familial) 10% in children
Pheo definition
Chromaffin cell tumor usually in adrenal medulla (90%) but can be anywhere along sympathetic chain.
Erroneous production of Epi
Pheo risk factors
MEN IIA/2B (usually cause bilateral)
VHL
Neurofibromatosis
FHx
5 Ps of Pheo
Paroxysms (comes and goes - since Epi is released in pulses)
Pressure (HTN)
Pain (HA)
Palpitations (sympathetic overdrive)
Perspire
Dx of Pheo
24h urine VMA and metanephrine (elevated)
Tx of Pheo
Alpha block first
THEN B block
THEN resect
A before B bc we don’t want the B block to cause unopposed alpha activity which would raise BP even more
If you resect first you’ll release catecholamines everywhere and make patient stroke out and die ( for same reason, ligate veins before resection)
parts of pit gland
Anterior = Adenohypophysis
Posterior = Neurohypophysis
Sheehans signs
Postpartum infarction and necrosis of pit leading to hormonal failure
Pit ischemia due to hemorrhage, hypovolemic shock, pit portal venous thrombosis
Failure of lactation
Amenorrhea
Progressive decreased adrenal function and thyroid function
Posterior pit disorders
SIADH and DI
SIADH
15% of hospitalized patients
Impaired water secretion
Hypersecretion of ADH results in increased urinary sodium with high urine osmolality
Causes = CNS injury, cancer, trauma, drugs
DI
Decreased ADH secretion
Impaired water conservation; large volumes of urine, leads to increased plasma osmolality and thirst
1/3 idiopathic
2/3 due to tumor or trauma
Signs of pit macroadenoma
> 1cm
Visual loss (b/l hemianopsia), hypopituitarism, HA, hyperprolactinema due to compression of stalk
- compression prevents dopamine from going from hypothal to post pit where it would normally inhibit prolactin production
This causes hyperprolactinemia and nipple discharge
Signs of pit microadenoma
Prolactinoma (#1) = secondary amenorrhea, galactorrhea
GH - Gigantism or acromegaly depending on age; course facial features, thick finger and heel pads, cardiomegaly, hepatomegaly, enlarged mandible, increased teeth spaces, neuropathy, arthropathy, osteoporosis, HTN, DM, goiter
ACTH - Cushings
Complications of pit surgery
Death (direct hypothal injury
Treatment for pit adenomas
PreOp: complete endocrine assessment, lytes to look for borderline DI
Surg:
- Transphenoidal vs transcranial vs perioperative
- PostOp XRT for large lesions
Primary Radiation:
- consider when surg contraindicated for other reasons in nonfunctioning tumor as primary therapy may worsen preexisting hypopituitarism
Medical
- For prolactin - Bromocriptine
- For GH - somatostatin which shrinks tumor size in 20-50%, normalizes GH in 50%, and normalizes IGF-1 in 40-80%
