Case based discussion of a pheochromocytoma

1. PHEOCHROMOCYTOMA
Allison Becker (Medical Student)
Jayanth H.Keshavamurthy.M.D.

2. HPI
• 67 year old female presents with RUQ pain following large meals. She
states that the pain is crampy and colicky and moderate in severity.
• She also complains of diarrhea for the past 2 weeks.
• Past Medical History: Type II diabetes mellitus, hyperlipidemia,
hypertension, atrial fibrillation, diabetic neuropathy
• Past Surgical History: No previous surgery
• Allergies: PCN
• Medications: amlodipine, benazepril, aspirin, atorvastatin, carvedilol,
levemir, gabapentin, ranitidine
• Family History: Not significant
• Social History: Denies alcohol, tobacco, and illicit drugs.

3. PHYSICAL EXAM
• Vitals: Temp: 36.9ºC, HR: 67 bpm, BP: 172/85 mmHg
• General: Alert and oriented x4
• HEENT: Normocephalic, atraumatic. Extraocular movements intact,,
normal conjunctiva. No pharyngeal erythema.
• Respiratory: Lungs are clear to auscultation bilaterally. Breaths are
unlabored.
• Cardiovascular: RRR, symmetric pulses in all extremities.
• Gastrointestinal: RUQ pain on palpation. No masses, soft, non-
distended. Normal bowel sounds.
• Neurologic: CN II-XII intact

4. ULTRASOUND

5. CT ABDOMEN WITHOUT CONTRAST

6. CT ABDOMEN WITH CONTRAST (60 SECONDS)

7. CT ABDOMEN WITH CONTRAST (15 MINUTE DELAY)

8. ADRENAL WASHOUT
• Absolute Washout Right Mass: 78.4%
• Relative Washout Right Mass: 52.4%
• Absolute Washout Left Mass: 68.6%
• Relative Washout Left Mass: 49.3%

9. SIGNIFICANT LABS
• Urine Normetanephrines: 6785 mcg/24 hours(135-521 mcg/24 hours)
• Total Metanephrines: 7115 mcg/24 hours (171-616 mcg/24 hours)
• VMA: 32. 7 mg/24 hours (<6 mg/24 hours)

10. MIBG

11. PHEOCHROMOCYTOMA
• Catecholamine-secreting tumors that arise from chromaffin cells of the
adrenal medulla
• Catecholamine-secreting tumors originate outside the adrenal medulla are
paragangliomas (sympathetic or parasympathetic nervous system)
• Occur in approximately 0.8/100,000 (underestimation as many are only
found at autopsy)
• Most occur in the fourth or fifth decade of life
• Patients present in 1 of 4 ways: classic, ”pheo crisis,” as an incidental finding,
or during screening for genetic or familial disease
• 40% are found as an incidental finding

12. CLASSIC
• Classic Triad: headache, palpitations,
sweating (40%)
• Hypertension (continuous or episodic)
(some patients present with normal
blood pressure)
• Difficulty breathing
• Weakness
• Panic attack-type symptoms
• Less common symptoms:
• Pallor, low blood pressure, blurred vision,
weight loss, increased thirst and urination,
constipation, abdominal pain, elevated
blood sugar, psychiatric disturbances,
cardiomyopathy
• 10% of patients
• Life-threatening release of
catecholamines
• Stroke
• Heart attack
• Organ failure
• Coma
• Death
“PHEO CRISIS”
PRESENTATION

13. RULE OF 10
• 10% Familial
• 10% Bilateral
• 10% in Children
• 10% Malignant
• 10% Extraadrenal
• 10% Not Associated with Hypertension
• 10% Contain Calcification

14. GENETIC/FAMILIAL DISEASE
• Family history of pheochromocytoma
• Multiple endocrine neoplasia, type II
• Von Hippel-Lindau
• Familial paraganglioma
• Neurofibromatosis 1

15. RADIOLOGIC FINDINGS
• Ultrasound
• Variable appearance from solid, solid to cystic, or mixed cystic
• CT
• Usually the initial modality
• Appear as heterogeneous masses with areas of necrosis and cystic change
• Avidly enhancing on arterial phase (more so than an adenoma)
• 110 HU enhancement or more on arterial phase indicates pheochromocytoma
• MRI
• T1: hypointense
• T2: markedly hyperintense (lightbulb sign)
• MIBG
• Neuroendocrine tumors have uptake on MIBG scan

16. TREATMENT
• Alpha-adrenergic blockers are given for 10-14 days (longer for certain
indications such as cardiomyopathy, recent MI, or resistant
hypertension) prior to surgical resection
• Phenoxybenzamine is the preferred agent
• Beta-adrenergic blockers should be given 2-3 days preoperatively
• Surgical resection with close monitoring of BP and heart rhythm
• Laproscopic adrenalectomy is preferred if there are no malignant features on
imaging
• Glucocorticoids should be given preoperatively if bilateral
adrenalectomy is planned
• Long term monitoring is necessary due to recurrence and metastatic
disease (which can be found many years after surgery)

17. REFERENCES
• Young WF Jr. Adrenal causes of hypertension: pheochromocytoma and
primary aldosteronism. Rev Endocr Metab Disord 2007; 8:309.
• Lenders JW, Duh QY, Eisenhofer G, et al. Pheochromocytoma and
paraganglioma: an endocrine society clinical practice guideline. J Clin
Endocrinol Metab 2014; 99:1915.
• Tauzin-Fin P, Sesay M, Gosse P, Ballanger P. Effects of perioperative alpha1
block on haemodynamic control during laparoscopic surgery for
phaeochromocytoma. Br J Anaesth 2004; 92:512.
• Guerrero MA, Schreinemakers JM, Vriens MR, et al. Clinical spectrum of
pheochromocytoma. J Am Coll Surg 2009; 209:727.
• Manger WM, Gifford RW. Pheochromocytoma. J Clin Hypertens (Greenwich)
2002; 4:62.
• Ros PR, Mortele KJ. CT and MRI of the abdomen and pelvis, a teaching file.
Lippincott Williams & Wilkins. (2006) ISBN:0781772370.
• Pacak K, Eisenhofer G, Lenders JW. Pheochromocytoma, Diagnosis,
Localization, and Treatment. Wiley-Blackwell. (2007) ISBN:1405149507.