1) A 15-year-old female presented with headaches, palpitations, and increased sweating. Imaging found a 5.7 cm mass in her right adrenal gland. 2) Biopsy of the removed mass confirmed pheochromocytoma. Pheochromocytomas are rare tumors that produce catecholamines, causing high blood pressure. 3) She was given alpha-blockers preoperatively to lower her blood pressure. A right adrenalectomy was performed to remove the tumor, and she required blood transfusions and medications post-surgery to manage her blood pressure before being discharged.

1. PHEOCHROMOCYTOMA
(Case Discussion)
Dr Shuah Ullah
Resident

2. Pt. Uzma , 15 yrs. old, un-married, known case of hep C, resident of
Tando Allah Yar presented with complain of
Increased sweating
Headache (One Year)
Palpitations

3. • She is a diagnosed case of hep C and undergoing treatment.
• Recently diagnosed hypertensive, not taking any medicine.

4. Ultra Sound :
• Heterogeneous mass in right
supra-renal area measuring 5.7 x
5 cm, showing vascularity on
CDI.
• Gall bladder stone measuring 0.7
cm.

5. CT Abdomen & Pelvis Dec. 2018

12. Labs Investigations:
HB 11.4
PLT 517
TLC 11.4
INR 0.98
S.CRE 0.49
NA/K/CL 138/4.3/105

13. Special Investigations :
24 Hour Urinary VMA 32.66 (<13.6 mg/24 hrs)
S.Cortisol 10.9 ( Morning 7-28 microgm/dl)
(Afternoon 2-18 microgm/dl)

14. • Pt. was managed with alpha and beta blockers (Tab Cardura and
Merol) and fluid therapy pre-operatively.

15. Right Adrenalectomy 03-04-2019
• Right Adrenal mass, capsulated approx. 6 x 7 cm.
• Single adrenal Artery and 2 x adrenal veins identified.
• Normal Right Kidney
• No any enlarge lymph nodes.
• Clear tissue planes defined between liver, duodenum and surrounding
structures.
• Iatrogenic IVC rent during adrenal vein ligation, repaired.

17. Post Operative Course :
• Pt. become hypotensive intra-operatively, so Nor-Adrenaline support was started
and continued till 5th Post Operative day.
• 2 x PCV txed on 1st Post op day as HB dropped to 6.6 mg/dl.
• Inj Solucortif 100 mg IV x TDS started from 2nd post operative day.
(Glucocorticoid)
• Tab Florinef 0.1 mg OD started from 4th post operative day. (Mineralocorticoid)
• She remained in SICU till 6th post-operative day.

18. • Discharge on 13th post-op day.
• Currently taking Tab Deltacortil 5mg 1+1+1.
• NO any active complain.

19. PHEOCHROMOCYTOMA
• Tumor of catecholamine producing cells of Adrenal medulla.
• Responsible for 0.5% cases of hypertension.
• 5 % of incidentally found adrenal masses.
• Peak in 4th to 6th decade while hereditary cases affect younger
population, equally affecting both genders.
• 10-25 % are extra adrenal, called as paraganglionomas.

20. Pathophysiology
• They have an enzyme PNMT (Phenylethanolamine N-methyltransferase)
which converts nor-epinephrine to epinephrine.
• Ability vary in different types which results in different clinical outcomes.
• 1/3rd cases are familial, VHL is most relevant as tendency to develop RCC
and MEN 2 is most common i.e. 50 %

22. • Malignant Pheochromocytoma is life threatening neoplasm. But it can
only be defined by clinical metastasis as pathologic tools like PASS
score are yet not validated.

23. Clinical Presentation.

24. Classic Triad
• Uncommon
1- Headache
2- Episodic sudden perspiration
3- Tachycardia

25. Diagnosis :
• Important to understand that secretion of catecholamines is episodic but
their metabolism is constantly going on inside pheochromocytomas.
• Best screening test for pheochromocytoma is assessment of metabolites of
epinephrine and nor-epinephrine which are metanephrine and nor-
metanephrine respectively.
• It has high (97-100%) sensitivity, but low specificity (82-85%) resulting in
high false positive cases.

26. • Secretion of catecholamines from pheochromocytoma is episodic;
thus single estimation of urinary epinephrine and norepinephrine is
likely to miss the diagnosis of pheochromocytoma in many cases,
more so in familial cases where up to 29% cases may have false
negative results.
• A plasma-free metanephrine and normetanephrine test can miss
exclusively dopamine secreting tumors, or small pheochromocytomas
(<1 cm size).

27. • Twenty-four-hour urinary-fractionated metanephrines and
normetanephrines can be used with slightly less sensitivity (97%) but
better specificity (98%).
• One advantage of urinary assays is that they are more standardized;
however, 24-hour urine samples are difficult to collect in children and
are quite cumbersome to the patient and many times urinary
sampling is inaccurate so it is advised to measure urinary creatinine
along with it to confirm adequacy of specimen.

28. False Positive: 24h Urine:
Drugs: TCAs, MAO- i , levodopa , methyldopa, labetalol , propanolol ,
clonidine (withdrawal).
Ilicit drugs ( opiods , amphetamines, cocaine), ethanol,
sympathomimetics (cold remedies)
Hold these medications for 2 weeks
Major physical stress (hypoglycemia, stroke, raised ICP, etc .)

29. • Plasma Catecholamine drawn with patient fasting, supine, with an
indwelling catheter in place > 30 min. Avoid drugs, diuretics and
smoking. Plasma total catechols > 11.8 nM (2000 pg/mL) SEN 85%
SPEC 80%
• Plasma Metanephrines: Not postural dependent: can draw normally
Secreted continuously by pheochromocytoma SEN 99% SPEC 89%
Free metanephrine > 400pg/ml, False Positive: acetaminophen.

30. • Clonidine Suppression Testing.
an α2 agonist, suppresses catecholamine (specifically norepinephrine) production by the
sympathetic nervous system but not by pheochromocytoma.
Comparison of normetanephrine levels before and after clonidine administration has been
shown to yield results with favorable test characteristic. Clonidine administration can result
in significant hypotension in certain patients
• Chromogranin A Testing.
Belongs to a group of compounds known as granins, which exist in the secretory vesicles of
the neuroendocrine and the nervous systems.
Elevation of serum chromogranin A levels has been documented in patients with
pheochromocytoma. Sensitivity of the test for detecting pheochromocytoma is suboptimal
Chromogranin A is renally cleared, and the specificity of the test decreases significantly in
patients with glomerular filtration rates less than 80 mL/min

31. • CT abdomen
Adrenal pheo SEN 93-100%
Extra-adrenal pheo SEN 90%
• MRI > SEN than CT for extra-adrenal pheo
• MIBG Scan SEN 77-90% SPEC 95-100%
MIBG catecholamine precurosr taken up by the tumor
Inject MIBG, scan @ 24h, 48h, 72h
False negative scan: Drugs: Labetalol , reserpine , TCAs, phenothiazines Must hold
these medications for 4-6 wk prior to scan

32. Diagnosis

33. Management
• An important aspect of BP control in pheochromocytoma is initial
treatment with α-blockers and after achieving adequate α-blockade,
the patient can be treated with β-blockers to achieve heart rate
control. Phenoxybenzamine is a preferred α-blocker; however,
prazosin, terazosin, doxazosin can also be used.

34. • Phenoxybenzamine is a nonselective blocker of α-receptors.
• It is started with initial dose of 10 mg twice daily and increased by 10--20 mg every third
day.
• Factors favoring phenoxybenzamine use are its long duration of action leading to twice
daily dosing and that it causes noncompetitive blockade of α-receptors; thus it prevents
episodic surges of catecholamine releases during pre- and post-operative period.
• Disadvantages of phenoxybenzamine include tachycardia, persistent postoperative
hypotension in view of covalent, noncompetitive binding to the α-receptor, somnolence,
stuffiness of nose, headache, and postural hypotension requiring intravenous fluid
replacement

35. • Prazosin is another option and is usually well tolerated with the
exception of occasional first-dose hypotension and poor
intraoperative BP control because of short duration of action.
Prazosin can be used in doses of 1 mg thrice daily initially and
increased to maximal doses of up to total 12 mg daily dose.
• Doxazosin and terazosin are selective α1receptor blockers which can
also be used. Doxazosin has a long half-life allowing once daily dosing
while prazosin is short acting.

36. • Adequate α-blockade is indicated clinically by postural hypotension and
then the patient is advised liberal salt and fluid intake for reexpansion of
plasma volume.
• Diuretics should not be used.
• Target BP is less than 120/80 mm of Hg in sitting position and systolic BP
not less than 90 mm of Hg on standing.
• Once adequate α-blockade is achieved β-blockers are started to control
tachycardia to achieve pulse rate of 60--80 per minute.
• β-blockers are to be used only after adequate α-blockade as otherwise
initial use of β-blockers will lead to unopposed α-stimulant action of
catecholamines leading to hypertensive crisis.

39. Surgical Management
• Laparoscopic Adrenalectomy
• Robot assisted Adrenalectomy
• Open Adrenalectomy.

40. INTRAOPERATIVE MANAGEMENT
• Drugs to be avoided during anaesthesia Morphine, Atracurium -
Atropine, Pancuronium , Succinylcholine - Halothane – Droperidol ,
Chlorpromazine, Metoclopramide , Ephedrine-
• Drugs can be used safely Induction Agent- Thiopentone , Etomidate
Analgesics- fentanyl , sufentanyl , alfentanyl
• Inhalational Agent- Enflurane , isoflurane , Nitrous oxide
• Muscle Relaxant – Vecuronium , Rocuronium

41. • Intraoperatively, hypertensive episodes should be anticipated and can be controlled with
intravenous drugs with rapid onset and short half-life such as nitroprusside,
phentolamine, nitroglycerin, and nicardipine.
• Temporary cessation of surgical manipulation of the pheochromocytoma may be
necessary.
• Short-acting β-blockers such as labetalol and esmolol are also good choices.
• Aggressive fluid management with volume repletion is necessary after removal of
pheochromocytoma because hypotension can occur as a result of sudden loss of tonic
vasoconstriction.

46. Post Operative :
• Postoperatively, fluid administration and use of vasopressors such as
phenylephrine, guided by invasive monitoring, are useful to manage
hypotension.
• Electrolyte abnormalities and hypoglycemia should be corrected.
• It is not uncommon for patients to remain hypertensive
postoperatively, and antihypertensive management should be
continued.

47. Follow Up
• Long-term vigilant postoperative follow-up of patients with
pheochromocytoma is essential (Lenders et al, 2005; Pacak et al, 2007).
• Lifelong screening for recurrence is recommended by some experts,
because 10-year recurrence rates are as high as 16% in some series of fully
resected lesions (Amar et al, 2005b; Plouin and Gimenez-Roqueplo, 2006a).
• Recurrent disease has been noted in patients more than 15 years following
resection of the original tumor.
• Biochemical testing at 6 months after surgery, followed by annual testing,
has been suggested (Pacak et al, 2001b, 2007).

48. Keypoints

49. THANK YOU