Endocrine

EMBRYOLOGY, ANATOMY, AND PHYSIOLOGY

What embryologic structures does the thyroid originate from?

• The medial thyroid comes from the first and second pharyngeal pouches

• Lateral portions of the thyroid and parafollicular C cells arise from the fourth and fifth pharyngeal pouches

What is the arterial supply of the thyroid?

• Superior thyroid artery (from external carotid artery)

• Inferior thyroid artery (from thyrocervical trunk)

What is the venous drainage of the thyroid?

• Superior and middle thyroid veins (drain into internal jugular vein)

• Inferior thyroid veins (drain into innominate and brachiocephalic veins)

The recurrent laryngeal nerve innervates all of the muscles of the larynx except this muscle?

• Cricothyroid muscle, which is innervated by the external branch of the superior laryngeal nerve

What structures do the recurrent laryngeal nerves wrap around?

• Subclavian artery on the right

• Arch of the aorta on the left

Injury to which nerve results in loss of projection and voice fatigability?

• Superior laryngeal nerve

What is the mechanism of action of propylthiouracil (PTU) and methimazole?

• Both drugs block peroxidase oxidation of iodide to iodine, thereby inhibiting incorporation of iodine into T4 and T3

• PTU also inhibits peripheral conversion of T4 to T3

Which drug crosses the placenta: PTU or methimazole?

• Methimazole

• PTU is the safer choice in pregnancy

When steroids are given in severe or acute hyperthyroid conditions? How do they work?

• Steroids inhibit peripheral conversion of T4 to T3 and lower serum TSH by suppressing the pituitary-thyroid axis

What is the Wolff-Chaikoff effect?

• Large doses of iodine given after an antithyroid medication can inhibit thyroid hormone release by disrupting the coupling of iodide

• This is a transient effect

What is the most common cause of hyperthyroidism?

• Graves disease, also known as diffuse toxic goiter, is the cause of 60% to 80% of hyperthyroidism

What is the etiology of Graves disease?

• Autoantibodies to TSH receptors (also called thyroid-stimulating antibodies or TSAb) bind and stimulate thyroid hormone production

• This leads to thyrotoxicosis, diffuse goiter, pretibial myxedema, ophthalmopathy

What is the treatment for Graves disease?

• Antithyroid medication

• Thyroid ablation

• With/without thyroidectomy

A 55-year-old woman presents with a 3-year history of fatigue and mild, diffuse, nontender thyroid enlargement and 15-lb weight loss. What is the most likely diagnosis?

• Hashimoto thyroiditis

What is the most common cause of hypothyroidism in adults?

• Hashimoto thyroiditis

What is the first-line treatment for Hashimoto thyroiditis?

• Thyroid hormone replacement

What will pathology show in a patient with Hashimoto thyroiditis?

• Lymphocytic infiltrate

A 35-year-old female presents with sudden onset of severe pain and associated swelling and tenderness of her thyroid with fever, chills, and dysphagia following an acute upper respiratory infection. What is the most likely diagnosis?

• Acute suppurative thyroiditis

What is the treatment for acute suppurative thyroiditis?

• Antibiotics

• Occasionally, abscess drainage

A 35-year-old female presents with moderate swelling and tenderness of her thyroid with repeated exacerbations and remissions over several months following an acute upper respiratory infection. What is the most likely diagnosis?

• Subacute (de Quervain) thyroiditis

What is the treatment for subacute (de Quervain) thyroiditis?

• NSAIDs, steroids

A 40-year-old female presents with hypothyroidism and symptoms of tracheal and esophageal compression and is found to have dense fibrosis throughout her thyroid gland. What is the most likely diagnosis?

• Riedel fibrous struma

• Painless, progressive goiter

• Usually euthyroid may become hypothyroid

What is the treatment for Riedel fibrous struma?

• Thyroid hormone replacement and steroids

• Surgery may be necessary to relieve obstructive symptoms

What is the treatment of thyroid storm?

• PTU or methimazole q4 to 6 hours and inorganic iodide to block synthesis and release of thyroid hormones

• Dexamethasone to inhibit peripheral conversion of T4 to T3

• Propanolol

• Fever reduction

• General resuscitation

What is the most common cause of thyroid enlargement?

• Multinodular goiter

What are indications for surgery with a multinodular goiter?

• Inability to rule out cancer

• Compressive symptoms

• Cosmetic deformity

What is the diagnostic test of choice for the evaluation of a thyroid nodule?

• FNA

• It has a sensitivity of 86% and specificity of 91%

• Initial cytology is nondiagnostic in 20% to 25% of cases

• However, a diagnosis of malignancy in FNA is highly accurate, approaching 100%

What is usually the first manifestation of multiple endocrine neoplasia (MEN) 2a and 2b?

• Medullary thyroid carcinoma (MTC)

What are the cytologic features of MTC?

• Amyloid among neoplastic cells

• Immunohistochemistry positive for calcitonin

• Positive staining for carcinoembryonic antigen (CEA) or calcitonin gene-related peptide

• Heterogeneity with polygonal or spindle-shaped cells

What percentage of MTC is sporadic?

• 75% to 80%

• Unable to differentiate familial versus sporadic at presentation—all should be tested for rearranged during transfection (RET) mutation, pheochromocytoma, and hyperparathyroidism

What operation should be done for MTC?

• Total thyroidectomy

• High incidence of multicentric disease

By what age should MEN 2a and MEN 2b patients undergo total thyroidectomy?

• MEN 2a—age 6

• MEN 2b—age 1 to 2

How can you follow patients after thyroidectomy for MTC?

• Calcitonin levels

• History and physical examination

What are the cytologic features indicative of anaplastic thyroid carcinoma?

• Grossly firm and white

• Marked heterogeneity with spindle, polygonal, or multinucleated cells

What is the prognosis for anaplastic thyroid carcinoma?

• Poor—only few survive more than 6 months

Who typically gets anaplastic thyroid carcinoma?

• Elderly patients with a long-standing goiter

What is the treatment for a small anaplastic thyroid carcinoma?

• Total thyroidectomy with or without external beam radiation

• Has small improvement in survival, especially for younger patients

What is the treatment for anaplastic thyroid carcinoma with compressive symptoms?

• Debulking surgery with tracheostomy

Which patient has a higher likelihood of malignancy: the patient with a solid thyroid lesion versus cystic thyroid lesion?

• Solid thyroid lesion

Which patient has a higher likelihood of malignancy: the patient with a solitary thyroid lesion versus multiple thyroid lesions?

• Solitary thyroid lesion

Which patient has a higher likelihood of malignancy: the patient with a hot thyroid lesion versus cold thyroid lesion?

• Cold thyroid lesion

Which type is the most common thyroid cancer and has the best prognosis?

• Papillary thyroid cancer, about 70% to 80% of all thyroid cancers

Which subtypes carry a worse prognosis?

• Insular

• Columnar

• Tall cell

What are the histologic findings for papillary thyroid carcinoma?

• Psammoma bodies

• Orphan Annie nuclei

What is the treatment for papillary thyroid carcinoma?

• High-risk, large (>2 cm), or bilateral tumors—total thyroidectomy

• Low-risk, small (<1 cm), or unilateral tumors—thyroid lobectomy and isthmusectomy

What laboratory test is followed after surgery to monitor recurrence?

• Thyroglobulin

True or False: Positive cervical nodes affect the prognosis of papillary thyroid carcinoma?

• False; positive cervical nodes do not affect the prognosis of papillary thyroid carcinoma as long as disease is resectable.

What are the histologic findings needed to define malignancy in follicular cancer?

• Blood vessel, capsular invasion

What is the most common site of distant metastasis for follicular thyroid carcinoma?

• Bone

• Next most common is lung

• Spread is hematogenous

Which has a worse prognosis: Hurthle cell carcinoma or Follicular carcinoma?

• Hurthle cell carcinoma

• Higher recurrence rate usually to regional lymph nodes

Name the 3 classifications systems specific to papillary thyroid cancer:

• AGES (age, grade of tumor, extent of tumor, size)

• AMES (age, metastasis, extent of tumor, size)

• MACIS (metastasis, age, completeness of resection, local invasion, size)

• High-risk patients by AGES or AMES criteria: >40 years old, male, capsular invasion or extrathyroidal extension, regional or distant metastases, size >4 cm, or poorly differentiated carcinoma

What is the (tumor, node, metastasis) (TNM) stage for a 57-year-old patient who underwent a total thyroidectomy for a 2.5-cm mass that was determined to be papillary thyroid carcinoma? All lymph nodes were free of disease and there was no extrathyroidal disease.

• This patient has T2N0M0 disease

• Because the patient is older than 45 years, this is stage II papillary thyroid cancer

• See Table 12-1

What are the indications for I-131 therapy?

• All stage III or IV disease

• All stage II disease younger than 45 years old

• Most patients 45 years or older with stage II disease

• Stage I disease who have aggressive histologies, nodal metastases, multifocal disease, and extrathyroid or vascular invasion

PARATHYROID EMBRYOLOGY, ANATOMY, AND PHYSIOLOGY

What structure are the superior parathyroid glands embryologically derived from?

• Fourth branchial pouch

What structure are the inferior parathyroid glands embryologically derived from?

• Third branchial pouch

What structure is the thymus embryologically derived from?

• Third branchial pouch

What is the arterial blood supply to the superior parathyroid glands?

• Inferior thyroid artery (occasionally by branches of the superior thyroid artery)

What is the arterial blood supply to the inferior parathyroid glands?

• Inferior thyroid artery

What is the spatial relationship of the inferior parathyroid gland to the recurrent laryngeal nerve and inferior thyroid artery?

• Inferior parathyroid glands are medial to the recurrent laryngeal nerves and located below the inferior thyroid artery

What is the spatial relationship of the superior parathyroid gland to the recurrent laryngeal nerve and inferior thyroid artery?

• Superior parathyroid glands are lateral to the recurrent laryngeal nerves and located above the inferior thyroid artery

Describe the mechanisms by which PTH increases serum calcium concentration:

• Bone—enhances resorption of bone matrix by osteoclasts

• Kidney—increases tubular reabsorption of filtered calcium and decreases tubular reabsorption of filtered phosphate

• Intestine—stimulates renal vitamin D complex synthesis, which increases intestinal absorption of calcium (indirect effect)

What laboratory test is the most sensitive and specific way to diagnose hyperparathyroidism?

• Intact parathyroid hormone level (elevated in >95% of patients with primary hyperparathyroidism)

What is the half-life of parathyroid hormone?

• 2 to 4 minutes

What is the desired decline in the intraoperative parathyroid hormone assay that confirms that the suspected abnormal parathyroid tissue was resected?

• 50% decrease from baseline PTH or a drop of the PTH to the normal range

Describe the process of vitamin D formation and activation:

• 7-dehydrocholesterol undergoes ultraviolet activation to form vitamin D3 (cholecalciferol) → hydroxylated in the liver to form 25-hydroxycholecalciferol → undergoes a second hydroxylation in the kidneys to its most active form 1,25-dihydroxycholecalciferol

What cells secrete the hormone calcitonin?

• Parafollicular (C cells) of the thyroid

Which type(s) of hyperparathyroidism (primary, secondary, or tertiary) is associated with high serum PTH levels and high-normal to elevated serum calcium levels?

• Primary and tertiary hyperparathyroidism

Which type of hyperparathyroidism is associated with high serum PTH levels and low-normal to low serum calcium levels?

• Secondary hyperparathyroidism

Which type of hyperparathyroidism is considered a compensatory response of the parathyroid glands to hypocalcemia?

• Secondary hyperparathyroidism

Which type of hyperparathyroidism occurs when long-standing stimulation of the parathyroid glands by hypocalcemia results in autonomous hyperfunctioning of the parathyroid glands?

• Tertiary hyperparathyroidism

What acid-base disturbance can be seen with primary hyperparathyroidism?

• Hyperchloremic metabolic acidosis

What chloride:phosphate ratio is highly suggestive of primary hyperparathyroidism?

• Chloride:phosphate ratio >33 is highly suggestive of primary hyperparathyroidism

What is the most common cause of primary hyperparathyroidism? Second most common? Third most common?

• Single adenoma (80%)

• Diffuse hyperplasia (15%)

• Multiple adenomas (4%)

What oncogene increases the risk for a parathyroid adenoma?

• PRAD-1

What are the classic gross findings diagnostic of a parathyroid adenoma?

• Single enlarged gland with 3 normal or small remaining glands associated with the histologic finding of hyperplastic tissue

Indications for initial parathyroidectomy for a patient with primary hyperparathyroidism:

Symptomatic:

• Typical bone, renal, gastrointestinal, neuromuscular symptoms

Asymptomatic:

• History of an episode of life-threatening hypercalcemia

• Presence of kidney stones detected by abdominal imaging

• Medical surveillance not desirable/possible

• Serum calcium level >1 mg/dL above upper limit of normal

• Age <50 years

• Osteoporosis (bone density T-score <–2.5 at any site)

• Creatinine clearance decreased by >30%

• Elevated 24-hour urinary calcium excretion (>400 mg/d)

What is the difference between persistent and recurrent hyperparathyroidism?

• Persistent refers to hypercalcemia that remains within 6 months of initial surgery

• Recurrent refers to hypercalcemia that returns after 6 months of initial surgery

What is the most common cause of persistent hyperparathyroidism?

• Missed adenoma

What are the causes for recurrent hyperparathyroidism?

• New adenoma formation or recurrent parathyroid cancer

Indications for reoperative parathyroidectomy for persistent or recurrent disease:

• Ongoing nephrolithiasis

• Worsening renal function

• Worsening bone disease as evidenced by bone mineral density scores

• Associated neuromuscular symptoms

• Associated psychiatric symptoms

• Worrisome progressive hypercalcemia

Indications for parathyroidectomy in patients with secondary hyperparathyroidism:

• Development of open ulcerative skin lesions from calcinosis or calciphylaxis

• Persistent bone pain or pathologic fractures (renal osteodystrophy)

• Ectopic calcifications

• Intractable pruritus

• Worsening disease with failure of medical management

General intraoperative algorithm for searching a "missing" parathyroid gland:

• Open and inspect the thyroid capsule, palpate gland

• Consider intraoperative US

• Dissect superior thymic/paratracheal tissue and complete a cervical thymectomy for missing inferior parathyroid glands

• Mobilize the pharynx and esophagus to look in the parapharyngeal/retropharyngeal/esophageal spaces for missing superior glands

• Open the carotid sheath and expose the common carotid and inspect for potential parathyroid glands

• Ligate the ipsilateral inferior thyroid artery and/or perform a thyroid lobectomy (record the location of all confirmed glands identified)

• End the procedure and follow patient for any evidence of persistent hypercalcemia

• Can reimage patient for evidence of ectopic parathyroid adenoma

What is the most common location for a "missing" parathyroid gland at reoperation?

• Normal anatomic position

Generally what is the recommended period of time that cryopreserved parathyroid tissue should not be transplanted?

• 24 months after freezing

How is parathyroid tissue autotransplanted?

• The resected parathyroid tissue is minced into 1-mm fragments. A pocket is then made in the desired muscle. Ten to 20 1-mm fragments of minced parathyroid tissue are inserted into the muscle pocket, which is closed and tagged with hemoclips/suture (for future identification)

Why is the forearm used preferentially over the sternocleidomastoid for autotransplantation of parathyroid tissue?

• Easier to re-explore if patient develops persistent or recurrent disease from autotransplanted tissue

• Easier to identify PTH gradients with peripheral blood draws in forearm versus neck (can place BP cuff)

Why do some patients experience postoperative hypocalcemia?

• Bone hunger, hypomagnesemia, failure of parathyroid remnant or graft

What imaging study is used for preoperative localization for reoperative parathyroid surgery and for minimally invasive parathyroidectomy?

• Technetium-sestamibi scan

In general, what is the most common cause of hypoparathyroidism?

• Surgical trauma to parathyroid glands during thyroid or parathyroid exploration

What operation is performed for parathyroid carcinoma?

• En bloc tumor resection that includes ipsilateral thyroid lobectomy, and resection of adjacent soft tissues

True or False: A frozen section biopsy for suspected parathyroid cancer should be performed before surgical excision?

• False; capsular rupture may occur with the potential of spreading tumor cells in the neck.

What is the most common location for parathyroid cancer to metastasis?

• Lung

What is the most common cause of hyperparathyroidism in MEN 1?

• Parathyroid hyperplasia (90%)

Which of the following needs to be corrected first in MEN 1—hyperparathyroidism, gastrinoma, or prolactinoma?

• Hyperparathyroidism; need to correct calcium first

Which of the following needs to be corrected first in MEN 2—hyperparathyroidism, pheochromocytoma, or MTC?

• Pheochromocytoma

ANATOMY AND PHYSIOLOGY

What hormones are released from the posterior pituitary?

• ADH and oxytocin

What hormones are released from the anterior pituitary?

• ACTH, FSH, LH, GH, TSH, and prolactin

What gonadotropic hormone promotes spermatogenesis or ovarian follicle maturation?

• FSH

What gonadotropic hormone promotes testicular testosterone production?

• LH

What drug may be given as primary therapy in patients who are not operative candidates with excessive production of GH by a pituitary adenoma?

• Octreotide (decreases serum levels of GH and the downstream growth factor somatomedin C)

What is the size cutoff to determine whether a pituitary lesion is a microadenoma versus macroadenoma?

• Microadenoma <1 cm

• Macroadenoma >1 cm

What imaging study is the gold standard for evaluating the pituitary?

• MRI with gadolinium

What is the most common pituitary adenoma?

• Prolactinoma

What is the treatment for prolactinoma?

• Bromocriptine

• Transsphenoidal resection for failure of medical management

Name the syndrome

Meningococcal sepsis/infection results in adrenal gland hemorrhage leading to adrenal insufficiency:

• Waterhouse-Friderichsen syndrome

Results from arachnoid herniation secondary to a congenital defect in the diaphragma sellae:

• Empty sella syndrome (primary)

A 35-year-old female with 1-week postpartum history of placental hemorrhage who presents with trouble lactating and amenorrhea:

• Sheehan syndrome

Hyperpigmentation and pituitary enlargement (resulting in amenorrhea and visual problems) after bilateral adrenalectomy:

• Nelson syndrome

Calcified cyst that is remnant of Rathke pouch and can present with endocrine abnormalities, headache, hydrocephalus, and visual disturbances:

• Craniopharyngioma

ADRENAL ANATOMY AND PHYSIOLOGY

What are signs/symptoms of Cushing syndrome?

• Acne, buffalo hump, depression, diabetes, easy bruising, hirsutism, hypertension, moon facies, myopathy, purple striae, truncal obesity, weakness

What enzyme is present almost exclusively in the adrenal medulla and organ of Zuckerkandl? What does it do?

• Phenylethanolamine-N-methyltransferase (PNMT)

• Converts norepinephrine to epinephrine via methylation

What enzyme is involved in the rate-limiting step of catecholamine synthesis?

• Tyrosine hydroxylase (hydroxylates tyrosine to dihydroxyphenylalanine [DOPA])

What are the intermediate substrates involved in the synthesis of epinephrine from tyrosine?

• Tyrosine→DOPA→Dopamine→Norepinephrine→Epinephrine

Which artery does the superior adrenal artery originate from?

• Inferior phrenic artery

Which artery does the middle adrenal artery originate from?

• Aorta

Which artery does the inferior adrenal artery originate from?

• Renal artery

The left adrenal vein empties into which vein?

• Left renal vein

The right adrenal vein empties into which vein?

• Inferior vena cava

What does the zona glomerulosa of the adrenal cortex produce?

• Aldosterone

What does the zona fasciculata of the adrenal cortex produce?

• Glucocorticoids

What does the zona reticularis of the adrenal cortex produce?

• Androgens/estrogens

What is the most common deficiency seen with congenital adrenal hyperplasia?

• 21-hydroxylase deficiency (90%)

What are the indications for surgical resection of an adrenal mass?

• More than 4 to 6 cm, enlarging (0.5 cm over 6 months), functioning, ominous characteristics (nonhomogenous) on imaging

What laboratory tests should be ordered in the workup of an adrenal mass?

• Urine metanephrines/vanillylmandelic acid (VMA)/catecholamines, urine hydroxycorticosteroids, serum potassium

• If decreased serum potassium or HTN, then obtain plasma renin and aldosterone

Primary hyperaldosteronism accounts for what percentage of all cases of HTN?

• 1%

Classic Conn syndrome is characterized by:

• HTN, hypokalemia, polyuria

What test can differentiate primary hyperaldosteronism from secondary hyperaldosteronism?

• Plasma renin level (suppressed with primary and not suppressed with secondary)

What is the #1 cause of primary hyperaldosteronism?

• Adrenal adenoma

What is the aldosterone:renin ratio that is suggestive of an aldosteronoma?

• 20 is diagnostic for primary hyperaldosteronism

What is the aldosterone suppression test?

• Give a saline infusion; if the urinary aldosterone level is elevated (>12 μg/d), the diagnosis of primary hyperaldosteronism is confirmed

What is the best imaging modality to distinguish aldosteronoma from bilateral hyperplasia?

• CT scan of the abdomen with thin cuts (3–5 mm) through the adrenal glands

What acid-base disturbance will be seen with primary hyperaldosteronism?

• Hypochloremic metabolic alkalosis

What test can be performed to lateralize an aldosterone producing hyperfunctioning adrenal gland in the setting of adrenal hypertrophy, bilateral nodules, or absence of lesions?

• Selective venous catheterization for aldosterone sampling

What is the adrenal vein aldosterone-to-cortisol ratio required to lateralize an aldosteronoma/unilateral adrenal hyperplasia?

• At least 5 times higher on the affected side; if ratios from both sides are similar, then suspect bilateral hyperplasia

If the captopril test is performed in a patient with an aldosterone-secreting tumor, what will happen to serum aldosterone levels?

• No change

• If the patient has bilateral adrenal hyperplasia, a captopril test will cause decreased aldosterone levels

What competitive inhibitor of aldosterone is usually given to control HTN and normalize potassium levels in a patient with an aldosteronoma/unilateral hyperplasia before surgery?

• Spironolactone

What is the best treatment approach for an aldosteronoma or unilateral hyperplasia?

• Laparoscopic adrenalectomy

What is the treatment for hyperaldosteronism from adrenal hyperplasia?

• Medical therapy with calcium channel blockers, potassium, and spironolactone. If refractory hypokalemia, then bilateral adrenalectomy, steroids, and fludrocortisone

What is the most sensitive and specific test for making the diagnosis of Cushing syndrome?

• 24-hour urinary free cortisol

What is the most common site of ectopic ACTH-producing tumor? Second most common site?

• #1 Small cell lung carcinoma

• #2 Carcinoid

What ACTH level signifies an ACTH-independent cause of endogenous glucocorticoid hypersecretion?

• <5 pg/mL (low)

What ACTH level signifies an ACTH-dependent cause of endogenous glucocorticoid hypersecretion?

• 15 pg/mL (high)

How is the low-dose overnight dexamethasone suppression test performed?

• Administer dexamethasone 1 mg PO at 11 PM; check plasma cortisol at 8 AM the next day. Cortisol level will be markedly suppressed (<1.8 μg/dL) in normal individuals. If cortisol level is not suppressed, the patient has Cushing syndrome.

How is the high-dose overnight dexamethasone suppression test performed?

• Obtain baseline plasma cortisol; administer dexamethasone 8 mg PO at 11 PM; check 8 AM plasma cortisol. Suppression occurs if there is >50% reduction in plasma cortisol, which indicates Cushing syndrome from a pituitary source (if not suppressed, then from an adrenal or ectopic cause).

What happens to the ACTH level during a high-dose dexamethasone suppression test with a responsible pituitary tumor?

• ACTH level is suppressed

What happens to the ACTH level during a high-dose dexamethasone suppression test with a responsible ectopic source?

• ACTH level is not suppressed

How is the corticotropin-releasing hormone (CRH) stimulation test performed?

• Check baseline serum ACTH and cortisol. Give 1 mcg/kg CRH IV. Draw serum ACTH and cortisol 15 minutes after administering CRH.

How does the CRH stimulation test distinguish between pituitary versus adrenal tumor versus ectopic ACTH causes of endogenous glucocorticoid hypersecretion?

• ACTH increases at a minimum of 35% above baseline with pituitary lesions only (others do not)

What laboratory tests establish the diagnosis of a virilizing tumor?

• Increased levels of serum dehydroepiandrostenedione (DHEA-S), serum testosterone, and 24-hour urine 7-hydroxysteroids and 17-ketosteroids

How does a dexamethasone suppression test differentiate between an adrenal versus ovarian cause for a virilizing tumor?

• 24-hour ketosteroids and serum androgens will be suppressed with ovarian tumors but not adrenal tumors

What happens to gadolinium on MRI for a malignant adrenal tumor? For an adrenal adenoma?

• Gadolinium persists in a malignant tumor; gadolinium washes out rapidly from an adenoma

What is the #1 cause of adrenal insufficiency?

• Withdrawal of exogenous steroids

What electrolyte abnormalities are seen in adrenal insufficiency?

• Hyponatremia, hyperkalemia

What test can be performed to diagnose adrenal insufficiency?

• ACTH stimulation test

What is one of the first signs for the loss of hypothalamic regulation of cortisol secretion?

• Loss of diurnal variation of cortisol levels

What is the safest operation for patients with suspected adrenocortical carcinoma?

• Open adrenalectomy

What medication is commonly used for adjuvant therapy with residual, recurrent, or metastatic adrenocortical carcinoma?

• Mitotane, a synthetic derivative of the insecticide dichlorodiphenyltrichloroethane (DDT) that directly suppresses the adrenal cortex and modifies the peripheral metabolism of steroids

What follow-up is needed for a patient with adrenal tumor <4 cm or 4 to 6 cm treated nonoperatively?

• Repeat abdominal imaging Q6 months to a year and annual screening for cortisol and catecholamines for 5 years

What is the treatment for a patient with adrenal mass >6 cm?

• Adrenalectomy; most likely malignant

What disease process needs to be ruled out before performing a fine-needle aspiration of a solitary adrenal mass?

• Pheochromocytoma; inadvertent biopsy can be potentially lethal

What laboratory test is the most sensitive for the detection of pheochromocytoma?

• Plasma metanephrines (96%)

What is the rule of 10's for pheochromocytoma?

• 10% bilateral, 10% malignant, 10% extra-adrenal, 10% hereditary, 10% in children

What ectopic sites can a pheochromocytoma be found?

• Organ of Zuckerkandl, thorax/mediastinum, bladder, scrotum

What familial syndromes are associated with pheochromocytoma?

• MEN type 2, Sturge-Weber syndrome, tuberous sclerosis, von Hippel-Lindau disease, neurofibromatosis type I

What is usually seen with a hypertensive "spell," the hallmark clinical presentation of pheochromocytoma?

• Diaphoresis, headache, palpitations, and paroxysmal HTN

True or False: Paragangliomas and pheochromocytomas located in the organ of Zuckerkandl can use PNMT to metabolize norepinephrine to epinephrine?

• True; paragangliomas and pheochromocytomas in the organ of Zuckerkandl contain PNMT.

What medications should be given preoperatively for pheochromocytoma?

• Phenoxybenzamine 10 mg BID and increase by 20 mg/d until BP and symptoms controlled; add β-blocker 3 days prior to surgery (inderal 10 mg TID); IVF hydration starting 2 days prior to surgery (typically patients are volume contracted)

What rapid-acting agents should be available in the operating room when operating on a pheochromocytoma?

• Neosynephrine, nitroprusside, lidocaine, esmolol, phentolamine

What follow-up is recommended after adrenalectomy for pheochromocytoma?

• Yearly 24-hour urinary metanephrines/catecholamines

What is the localizing test of choice for adrenal pheochromocytoma?

• CT scan of the abdomen with thin cuts (3–5 mm) through the adrenal glands

What is the approximate percentage of MEN 2 patients with pheochromocytoma?

• 40%

What is the surgical treatment for pheochromocytoma in MEN 2?

• Bilateral adrenalectomy; trend now is to remove the affected side and observe the contralateral side until there is radiographic/biochemical evidence of disease

What is the most common cancer to metastasize to the adrenal gland?

• Breast cancer