Adrenal Flashcards

Question

3 Beta hydroxysteroid dehydrogenase deficiency

Answer 1

Increased 17-hydroxypregnenelone and hydroepiandrosterone (DHEA)

Answer 2

YES Measure amniotic fluid for 17 hydroxyprogesterone (or during chorionic villus sampling) Treat mother with dexamethasone, which suppresses fetal production of ACTH, which prevents virilization of the genitalia

Answer 3

46XY Bilateral testes Female appearing genitalia Absence of mullerian derivates

Answer 4

Male undervirilization 46XY Penoscrotal hypospadias or markedly ambiguous genitalia Elevated testosterone, low DHT

Answer 5

**Overnight low-dose (1-mg) dexamethasone suppression test (OST)** Late-night salivary cortisol test (SCT) 24-hour urinary-free cortisol evaluation (UFC) To perform the OST, a dose of 1 mg dexamethasone is given at 23:00 hours followed by measurement of serum cortisol and dexamethasone the following morning at 08:00. A putative diagnosis of Cushing Syndrome is made if the serum cortisol level is **\> 5 micrograms/dL** following an OST. An **ACTH leve**l can be checked to ascertain whether it is ACTH-independent or -dependent. A l**ow/suppressed ACTH or DHEA-sulfate support an adrenal** source.

Answer 6

Any patient with sustained blood pressure above 150/100 on three separate measurements taken on different days Hypertension resistant to 3 antihypertensives Hypertension controlled with four or more medications Hypertension and low potassium Hypertension and a newly diagnosed adrenal incidentaloma Hypertension and concomitant sleep apnea Hypertension and a family history of early onset hypertension or stroke before age 40 All first-degree relatives of patients with a diagnosis of primary aldosteronism

Answer 7

A positive ARR screen should prompt a confirmatory 24-hour urine study with salt loading to assess for primary aldosteronism. It is also important to note that positive testing for aldosterone hypersecretion does not guarantee a unilateral source, and therefore, adrenal venous sampling is recommended prior to surgical intervention.

Answer 8

Investigation for familial syndromes with genetic testing is warranted, with emphasis on mutation of the RET proto-oncogene (multiple endocrine neoplasia type 2), VHL (von Hippel-Lindau disease), or succinate dehydrogenase genes

Answer 9

An adrenal protocol CT scan is a triphasic study. The first phase of the study is without contrast. This is followed by imaging immediately after intravenous administration of contrast. The final phase is imaging after 10 to 15 minutes. Adrenal washout can be presented as absolute percentage contrast-washout ((enhanced HU – 15-min delayed HU)/(enhanced HU – unenhanced HU) x 100%) or as relative percentage contrast washout ((enhanced HU – 15-min delayed HU)/enhanced HU x 100%). The 2017 ACR guidelines denote thresholds of \> 60% absolute percentage washout or \> 40% relative percentage washout as characteristic of benign incidentalomas.

Answer 10

Pheochromocytomas measure greater than 10 HU on unenhanced CT scans and will enhance to more than 100 HU on contrast studies, often exhibiting a well-circumscribed appearance with or without necrotic or cystic elements. Recent data suggest that if HU are \<10 then biochemical testing for pheochromocytoma is not necessary. The classic “light bulb” signal on T2-weighted MRI may be helpful, but recent studies suggest that this sign is less sensitive and specific than previously thought. Additional imaging, such as meta-iodobenzylguanidine (MIBG) scanning may also be warranted in this setting, especially if assessing for an extra-adrenal, metastatic, or recurrent pheochromocytoma.

Answer 11

Adrenocortical carcinomas typically are larger than 4 cm and exhibit a heterogeneous appearance on CT, with calcifications and necrosis often present. HU measurement on non-contrast CT are typically \>25 HU with \<50% washout demonstrated at 10 minutes post-contrast imaging. Adrenal metastasis may also have a similar appearance, which belies the importance of clinical history in this setting. Lipid-poor adenomas may have attenuation values of 20-40 HU, but a washout of \>50% is believed to distinguish these lesions from adrenal carcinomas.

Answer 12

Lipid-poor adenomas may have attenuation values of 20-40 HU, but a washout of \>50% is believed to distinguish these lesions from adrenal carcinomas.

Answer 13

There is some controversy over the management of masses between 4-6 cm. The American Association of Clinical Endocrinologists/American Association of Endocrine Surgeons (AACE/AAES) guidelines recommend removal of all masses over 4 cm regardless of functional status or imaging characteristics, unless it is clearly a myelolipoma.

Answer 14

Patients with excessive glucocorticoid production are at risk for development of the metabolic syndrome, including hypertension, medical issues related to obesity, cardiovascular disease and diabetes. If a patient is not a candidate for adrenalectomy, medical treatment such as -aminoglutethimide -metyrapone -ketoconazole should be initiated while simultaneously monitoring for adrenal insufficiency. For patients with sub-clinical Cushing syndrome, surgical resection is reserved for those with worsening hypertension, dyslipidemia, abnormal glucose tolerance, or osteoporosis.

Answer 15

For those patients with primary aldosteronism, excess plasma aldosterone is harmful and can lead to myocardial fibrosis, left ventricular hypertrophy, cardiac ischemic events, and clotting abnormalities, even if blood pressure is controlled. Therefore, adrenalectomy is preferred unless the patient is not an operative candidate. Adrenal vein sampling is nearly universally performed prior to surgery to identify laterality, even in the case of micronodularity or bilateral adrenal masses. However, in the setting of a unilateral hyperdense nodule \> 1cm with a positive aldosteronoma screen, unilateral adrenalectomy is indicated.

Answer 16

Prior to surgery, patients are given - A mineralocorticoid receptor antagonist (epleronone, spironolactone) - Antihypertensive - Potassium repletion Following surgery, antihypertensives are weaned slowly. Almost all patients will exhibit improvement in hypertension following adrenalectomy with complete normalization seen in approximately 60%.

Answer 17

AACE/AAES recommendd radiographic re-evaluation at 3-6 months and then annually for 1-2 years for all adrenal incidentalomas, even in the setting of benign radiographic characteristics initially. Hormonal evaluation should be performed at time of diagnosis and annually for 5 years. If the lesion size increases by ≥ 1 cm or develops functionality, surgery should be considered. However, 75-95% of incidentalomas remain stable in size while 2-8% of previously non-functioning lesions develop functionality, with hypersecretion of cortisol being the most common finding. CONTROVERSIAL However, in 2016 the European Society of Endocrinology and European Network for the Study of Adrenal Tumors guidelines recommended no further follow-up imaging for an incidentaloma that showed benign imaging (non-contrast HU \<10) and was under 4 cm in size. For tumors greater than 4 cm or with indeterminate imaging characteristics, additional imaging was recommended at 6-12 months. Similar recommendations were put forth in 2017 by the American College of Radiology, which recommended against follow-up imaging for incidentalomas with unequivocally benign characteristics, regardless of size on initial imaging including the presence of macroscopic fat, low CT density (non-contrast HU \<10), cystic features, hemorrhage, absence of enhancement, and loss of MRI signal between in-phase and opposed-phase images on chemical shift MRI. However, for masses measuring 1-4 cm with indeterminate radiographic features, additional risk-stratified imaging follow-up vs. advanced imaging (e.g. PET-CT), biopsy, or extirpation are recommended accordingly.

Answer 18

The most common sites of pheochromocytoma spread are bone, liver, regional lymph nodes, lung, and peritoneum.

Answer 19

Most metachronous metastases will present within the first 5 years of diagnosis; however, lifelong annual biochemical follow-up is recommended as recurrences have been noted to occur more than 15 years following surgery.

Answer 20

Alpha-adrenergic blockade, typically with phenoxybenzamine (an irreversible non-selective alpha blocker), is started 7-14 days prior to surgery and titrated to a blood pressure of 120-130/80mmHg. This is followed by beta blockade with atenolol or metoprolol if tachycardia or arrhythmias develop. Use of metyrosine to block catecholamine synthesis is not commonly needed but can be used in combination with alpha-blockade. Calcium channel blockers have been advocated by some as an adjunct to or in lieu of traditional alpha blockade. Intra-vascular volume expansion is also necessary due to the chronically vasoconstricted state. This can be achieved through preoperative and intraoperative hydration and a high salt diet. This should be initiated 7-14 days prior to surgery.

Answer 21

Primary Tumor (T) pTx Primary tumor cannot be assessed pT0 No evidence of primary tumor pT1 Tumor ≤ 5cm in greatest dimension, no extra-adrenal invasion pT2 Tumor \> 5cm in greatest dimension, no extra-adrenal invasion pT3 Tumor of any size with extra-adrenal invasion, but not invading adjacent organs pT4 Tumor of any size with invasion of adjacent organs (kidney, diaphragm, spleen, liver, etc) Regional Lymph Nodes (N) Nx Regional lymph nodes cannot be assessed N0 No regional lymph node metastasis N1 Metastases in regional lymph node(s) (retroperitoneal) Distant Metastasis (M) Mx Distant metastasis cannot be assessed M0 No distant metastasis M1 Distant metastasis

Answer 22

Stage T N M Stage I pT1 N0 M0 Stage II pT2 N0 M0 Stage III pT1-2 N1 M0 pT3-4 N0-1 M0 Stage IV Any T Any N M1

Answer 23

The reported 5-year survival rates are 30-45%, 13-57%, 5-18%, and 0% for stages I through IV, respectively. The most common sites for metastases are liver (48%), lung (45%), lymph nodes (29%), and bone (13%). Local invasion into the kidney (26%) and IVC (9-19%) can also occur.

Answer 24

Cisplatin + etoposide +/- doxorubicin +/- mitotane Just say "Resection; mitotane +/- cytotoxic chemotherapy"

Answer 25

headache palpitations sweating easy bruisability muscular weakness recent weight loss or gain high blood pressure flushing

Answer 26

central obesity moon face buffalo hump abdominal striae virilization BP body weight (BMI)

Answer 27

non-functional adenoma functional adenoma Pheochromocytoma Aldosteronoma (Conn's) Cortisoloma (Cushing's) Adrenal Cancer (ACC) Myelolipoma Adrenal mets from another cancer Adrenal hyperplasia Adrenal infection (Tb) Sarcoma Oncocytoma Adrenal Cyst Infarct

Answer 28

ACTH dependent → excessive secretion of ACTH from pituitary (70%), ectopic ACTH production (10-15%), ectopic production CRH (bronchogenic carcinoma \< 1%) ACTH independent → cortisol-secreting adenomas of adrenal gland or ACC (10%)

Answer 29

1. Heat CT/MRI 2. Inferior petrosal vein sampling for ACTH (if elevated, NSG) 3. Trans-sphenoidal hypophysectomy for pituitary adenoma

Answer 30

excess cortisol secretion, suppressed HPA axis → adrenocortical insufficiency Administer glucocorticoid replacement, hydrocortisone 100 mg IV or dexamethasone 4 mg IV \*\*Mineralocorticoid replacement is not necessary in acute setting

Answer 31

CT re-evaluation at 3-6 months and then annually for 1-2 years \* even in the setting of benign radiographic characteristics initially Hormonal evaluation should be performed annually for 5 years If the lesion size increases by ≥ 1 cm or develops functionality, surgery should be considered.

Answer 32

Place a drain can close pancreatic capsule with non-absorbable sutures distal tail w/ductal injury → resection of distal portion of pancreas with suture ligate of duct

Answer 33

Splenectomy splenic artery ligated at superior border of pancreas splenocolic ligaments dissected Elevate spleen and ligate segmental splenic artery/veins close to capsule short gastric vessels dissected cut retroperitoneal attachments remove spleen \*\*POST OP Vax pneumococcus/meningococcus/HIB small tears to capsule → fulgurate/argon beam, gelfoam/fibrin glue minor → surgicel/avitene moderate → close with bolster and surgicel/avitene

Answer 34

Adrenal adenoma on Ct washout Benign have \< 10 HU on non-contrast CT \>60% absolute washout or \>40% relative washout Malignant tumors and functional tumors have lesser washout, hold on to contrast non-contrast MRI, masses that demonstrate loss of signal intensity on out of phase sequences are adenomas

Answer 35

severe coagulopathy poor cardiopulmonary performance

Answer 36

Minimal manipulation of mass “no touch” Ligate or clip adrenal arteries Ligate adrenal vein Inspect adrenal fossa for bleeding Perform regional LND (if suspect ACC) No drain necessary Deliver carefully via lap bag

Answer 37

inferior phrenic artery aorta renal artery

Answer 38

this is normal replace volume with NS pressors rarely needed

Answer 39

Compression Suction Proximal and distal control Oversew hole with 4-0 Prolene

Answer 40

proximal and distal control oppose caval edges ("satinsky") oversew injury 4-0 Prolene

Answer 41

Insert Robinson catheter into injury purse string with 3-0 suture evacuate air from pleura by suction or water seal remove tube quickly under neg pressure and tie purse string

Answer 42

\>15% Chest tube \<15% w/o distress, no shift, manage expectantly, O2

Answer 43

lacerations repaired in multiple layers, 4-0 silk, omental wrap, NGT hemotomas observed, identify and control bleeding

Answer 44

liver lac → mattress sutures, surgicel bolster deep lac with bile leak → drained significant hemorrhage → pringle maneuver (cross clamp porta hepatis until stable) call GS

Answer 45

renin-mediated htn RAS cirrhosis heart failure reninoma/juxtaglomerular cell tumor

Answer 46

headache palpitations sweating sustained HTN pallor hyperglycemia paroxysmal HTN chronic weakness, tiredness anxiety/panic

Answer 47

adrenal medulla (75-90%) extra-adrenal (10-25%) = paragangliomas (neural crest) head, neck, thorax, abdomen, pelvis can arise between aortic bifurcation and root of IMA (organ of Zuckerkandl)

Answer 48

CHF (catecholamine induce cardiomyopathy) malignant htn tachyarrhythmia CVA hypertensive encephalopathy renal failure/renal infarction tumor hemorrhage

Answer 49

ALSO aggressive peri-operative hydration