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Surgical Disease of the Adrenal Gland (Part I)

Surgical Disease of the Adrenal Gland (Part I). Roc McCarthy, D.O. Anatomy Physiology The incidental adrenal mass Fun facts Pheochromocytoma. Adrenal Gland. Cortex - mesoderm Medulla - neuroectoderm Renal agenesis, found in normal anatomic position Size- 5 x 3 x 1 x 5 cm

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Surgical Disease of the Adrenal Gland (Part I)

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  1. Surgical Disease of the Adrenal Gland (Part I) Roc McCarthy, D.O.

  2. Anatomy • Physiology • The incidental adrenal mass • Fun facts • Pheochromocytoma

  3. Adrenal Gland • Cortex - mesoderm • Medulla - neuroectoderm • Renal agenesis, found in normal anatomic position • Size- 5 x 3 x 1 x 5 cm • Retroperitoneal structure, contained in its own sub-compartment w/in Gerota’s fascia

  4. Adrenal Gland • Blood supply Arterial - receives 7cc/gram minute - 3 arterial sources of flow: 1. Inferior phrenic artery 2. Aorta 3. Renal artery Venous – single main vein most important surgical structure - right → post IVC - left → renal vein

  5. Nerve Supply • Medulla - sympathetic branches - epinephrine - norepinephrine • Cortex – none • Lymphatics- lateral aortics (renal artery to diaphragm)

  6. Hypothalamic-Pituitary-Adrenal Axis

  7. Layers of Adrenal Gland

  8. Aldosterone • Primary stimulus for release is angiotensin II • Other: ACTH, low serum Na, elevated K, JGA via low kidney perfusion

  9. Adrenal Medulla • Distinct from cortex embryologically (neuroectoderm) • Secretes catecholamines sympathetic stimulation (mainly epinephrine but also norepinephrine and dopamine) • If pt. has excess of BOTH epi and norepi, the tumor is in the adrenal gland

  10. The Incidental Adrenal Tumor • 0.5 – 5% of abdominal CTs show abnormal adrenal glands • 85% of adrenal masses are nonfunctional and BENIGN • Def. of incidental mass: - >1cm - discovered on exam for non-adrenal cause - absence of signs or symptoms of adrenal disorder

  11. Questions to be asked: • Is the mass functional? • Any physical signs or symptoms? • Is there biochemical evidence of activity: 1. Pheo screen 2. Potassium level 3. Glucocorticoid screen • Is the mass malignant? • Any history of another malignancy? • Is imaging suggestive of malignancy?

  12. Nature of Incidentally Found Adrenal Masses • Review of 2,005 incidentally-discovered adrenal masses: - Nonfunctioning adenoma 82% - Functioning: Cushing’s 5% Pheo 5% Aldosteronoma 1% - Malignancy: Metastasis 3% ACC 4% • Young WF, et al. Endocrinol Clin N Am. 2000

  13. Good to Know Facts! • If the adrenal gland has fat density material, it is by definition a benign myelolipoma • If pt. has a known primary cancer, the adrenal mass with be mets from that site 50% of time • Overall 2-4% of adrenal masses are ACC • If mass >6cm, ACC 65%

  14. Incidental Adrenal Mass Initial Evaluation • History and physical exam • Look for signs of hormonal syndromes • Search for occult malignancy • CXR • Stool for occult blood • Mammogram (women only)

  15. Extent of Endocrine Evaluation? • Serum K (if HTN) → Conn’s • Plasma metanephrines: most sensitive test for pheo • 24-hr urine cortisol (Cushing’s)

  16. Diagnosis: Imaging • Rare for a nonfunctional adenoma to become functional • MRI- Both ACC and pheo are hyper-intense in T2 images (light up from T1 to T2)

  17. CT Adenoma Characteristics • Sharp margins • Smooth, homogenous, lipid rich • Most <10 Hu on noncontrast images • Washout >50% @ 15 min

  18. Incidental Adrenal Mass Management • Hormonally active → surgical removal • > 5 cm → removal (with a scalpel) • < 3 cm observe • Surveillance Recommendations: Old: - CT at 6 months - Annual endocrine eval for 4 years New: If mass stable on scans @ 3m and 1 yr then no further workup

  19. Pheochromocytoma • Incidence and Presentation - symptoms → release of epi/norepi - hypertension present 90% cases - orthostatic hypotension (low plasma vol) - 30% of pheo’s found at autopsy and cause of death cardiovascular disease - micturition syncope

  20. Pheo (cont’d) • Triad: headache, tachycardia, diaphoresis • Other symptoms: Pallor, flushing, palpitations, abd/chest pain, weakness, N/V, psychosis • Small tumors more likely symptomatic • ALL patients regardless of age, have a complete cardiac work-up before surgery

  21. Pheochromocytoma Rule’s of 10 • Bilateral • Familial (non-sporadic) • Pediatric • Malignant • Normotensive • Extra-adrenal • Multiple • *Childhood presentation breaks the rules- 25% bilateral, multiple, extra-adrenal

  22. Pheo- The Diagnosis • Plasma free metanephrines- most sensitive test -seen 99% of patients • 24° urinary catecholamines (2x normal is diagnostic) • VMA • Clonidine suppression test (0.3mg oral, test 3 hrs later) >50% reduction catecholamines NO pheo

  23. MEN I

  24. MEN IIA

  25. MEN IIB

  26. Pre-op Management • Early alpha blockade??? • Goal to control hypertension- phenoxybenzamine • Do NOT use b-blocker before alpha • IV hydration • Prevent cardiac arrhythmias

  27. Pheo • Post-op - hypotension (most common) sec to secondary to hypovolemia • Surgical outcomes - excision does NOT always lead to long- term cure - recurrence 5% benign 10% malignant

  28. Part II • Conn’s syndrome • Cushing’s syndrome • Addison’s disease • Adrenal cortical carcinoma • Metastatic disease to adrenal gland • Principles of adrenalectomy

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