Case presentation

Urology Tuesday meeting

Anas Hindawi PGY3 Urology ResidentMakassed General Hospital

Beirut Arab University

14/4/2015

• 24 y.o female previously healthy

• Presentation for gastroenteritis with associated abdominal pain

• P.E : normal • PMH : none• PSH : none• Allergy : none

• IV hydration and workup started

• CBCD : Hg/Hct 13.7/40.2 ,WBC 10.4 ,Neut 62 ,Plt 267• Chem 6 : BUN/Cr 0.55/26 ,138/4/105/21• Glucose : 93• LFTS and lipid panel are all normal • CRP : 1• FT3 3.7 ,FT4 13.97 ,TSH 1.91• H pylori IGg normal • U/A : PH 5.5 ,wbc 7-8 ,rbc 2-3

• Chest X ray normal

Patient gets improved except for incidental finding on ultrasound abdomen pelvis

•Parasplenic nodule 3*4 cm corresponding to adrenal gland with left iliac adenomegaly corresponding to the left kidney

Next

CT abdomen and pelvis with IV contrast

• Left parasplenic heterogenous mass found to have 11 up to 18 HU with no remarking enhancement

Next

• Plasma Metanephrine 37 ng/L (ref. range <90)• Plasma Normetanephrine 228 ng/L (ref. range <129)• Cortisol 3.69 micg/dL (5-25)

• 24 hrs Urine collection 1750 mL• Urine Metanephrine 140 micg/24hr (<350)

Next

MRI with gadolinium

Next

Laparoscopic Left Adrenalectomy

Review of Assessment and Management of an Adrenal Mass in Urological Practice

AUA Update Series 2014Lesson 19 Volume 33

Division of Urologic Oncology, Department of Surgical Oncology

Fox Chase Cancer Center-Temple University Health System

Philadelphia, Pennsylvania

ADRENAL ANATOMY AND PHYSIOLOGY

• Adrenal glands are located deep in the retroperitoneum and are essential to life

• Laparoscopic approaches to the adrenal extremely advantageous because of equidistant from all body surfaces

• The ‘‘capper’’ adrenal glands are located superior to the kidney ,while the ‘‘cradlers’’ lie medial to the kidney and straddle the renal vessels

• The adrenal glands are almost always found in their orthotopic location in cases of renal agenesis or renal malposition

• A devastating surgical error is to confuse the tail of the pancreas for the left adrenal gland, as the tissue of these organs intraoperatively can look surprisingly similar

• Given the endocrine function of the glands, blood flow to the adrenal glands is rich

• Approximately 60 separate small arteries enter each gland and stem from 3 main sources

• Generally a single vein drains each adrenal gland

• The adrenal glands are best conceptualized as 2 distinct organs comprising the adrenal cortex and the adrenal medulla.

• The medulla lies at the center of the adrenal, is innervated by sympathetic fibers and secretes epinephrine (80%), norepinephrine (19%) and dopamine (1%)

• The adrenal cortex is subdivided into 3 regions known as zona glomerulosa, zona fasiculata and zona reticularis

• The hypothalamic-pituitary-adrenal axis regulates cortisol and adrenal androgen secretion, while the renin-angiotensin-aldosterone system controls secretion of aldosterone

ADRENAL MASS• By age 70 years up to 7% of individuals will harbor an

adrenal mass ,while much more rare in youth ~0.5%

• Defined as a lesion >1 cm from either the cortex or the medulla

• Although the majority of masses are benign and metabolically silent, during every evaluation of an adrenal mass the urologist must risk stratify for presence of malignancy and assess for metabolic activity

• Cross-sectional imaging with MRI or CT affords excellent visualization of the gland

• The linchpin of adrenal imaging is quantification of intracytoplasmic lipid content of a given tumor, since adrenal adenomas generally contain much larger quantities of intracellular lipid than other adrenal lesions

• Lipid poor adenomas can usually be distinguished from other adrenal pathologies such as pheochromocytomas, carcinoma or metastases using the CT washout study

• Standard post-contrast CT (1 minute after an iodinated contrast bolus), does not provide meaningful information regarding lipid content of adrenal masses

• MRI does not allow for washout testing, as gadolinium based contrast agents do not harbor dose dependent signal fall-off properties as do iodinated CT contrast agents

• FDG-PET better used for staging non MEN 2 cases with pheochromocytoma than as initial adrenal mass characterization

Size of adrenal mass• Analysis of large retrospective cohorts of incidentally

discovered adrenal lesions revealed that chances of harboring a malignant adrenal mass are strongly linked to tumor size

• Large upper pole kidney tumors leave the renal unit in its orthotopic location, large adrenal masses often displace the kidney

• Current recommendations advise resection of masses >6 cm that are not radiographically consistent with myelolipoma, since nearly a third of these tumors prove malignant upon resection

• Meanwhile, the 4 cm cutoff is generally reserved for the young and patients without comorbidities

• No improvement in survival for patients with ACC has been noted in the last 2 decades

• Sound clinical judgment that integrates not only tumor size, but also patient age, comorbidity status, tumor growth kinetics and surgical risks should inform clinical decision making to proceed with adrenalectomy

Adrenal mass growth kinetics

• Expert consensus advises reimaging of adrenal masses at 6, 12 and 24 months after diagnosis

• Up to 9% of adrenal incidentalomas will grow >1 cm during the first few years of follow-up

Adrenal biopsy• Rarely necessary

• Differentiation between adrenocortical carcinoma and adenoma is rarely possible

• Needle track seeding and hemorrhage have been reported with the latter potentially complicating future adrenalectomy

• Therefore, adrenal biopsy should be pursued only when limitations of imaging and metabolic evaluation have been reached and when the clinical team is certain that the result of biopsy will influence management

• More than 10% of adrenal incidentalomas are metabolically active, and all adrenal masses >1 cm should be tested for cortisol and catecholamine hypersecretion

• To rule out hypercortisolemia or Cushing syndrome, the low dose dexamethasone suppression test is used by most practitioners

• Plasma-free metanephrines or 24-hour total urinary fractionated metanephrines are largely equally adequate for initial screening for pheochromocytoma

Metabolic evaluation

• Only patients with an adrenal mass and hypertension require screening for hyperaldosteronism, and Conn syndrome is found in <1% of patients with adrenal incidentaloma

• Adrenal sex steroids testing in the form of dehydroepiandrosterone sulfate, 17-OH progesterone, androstenedione, testosterone and 17-estradiol levels is only necessary when adrenocortical carcinoma is strongly suspected

Metabolic evaluation

Management• Metabolically silent adrenal lesions <3 cm are unlikely to

exhibit hormonal hypersecretion at follow-up

• Up to 20% of patients with adrenal lesions >3 cm can have hormonal hypersecretion upon further testing

• Metabolically silent adrenal lesions require an annual metabolic evaluation for 3 to 4 years after the initial diagnosis

• Reimaging generally should occur at 6, 12 and 24 months after diagnosis to establish tumor growth kinetics