The document presents a case study of a 14-year-old boy diagnosed with pheochromocytoma after experiencing severe hypertension and fainting. Initial ultrasound and subsequent CT scans indicated the presence of solid masses in the adrenal glands and renal hilum, prompting further investigation for urinary catecholamines. Pheochromocytoma is a neuroendocrine tumor of the adrenal gland that can cause significant cardiovascular symptoms due to excessive catecholamine secretion.

1. Phaeochromocytoma.....
a case
Dr Rekha Khare
MD Radiology

2. Case history
• A young boy of about 14 year came in the department of
Radiology for ultrasound examination
• The patient gave history of while doing some domestic
work, suddenly he became dizzy and unconscious.
• His father took him to near by hospital
• On examination his blood pressure was recorded very
high 220/120 mm of Hg.
• There was no neurological deficit.
• He was given some antihypertensive medicine,
provisionally treated for hypertensive crises

3. Ultrasound exam..
• A well outlined solid hypoechoic mass lesion with
few tiny cystic degeneration in right suprarenal region
• Mass measuring about 3.5x 5.0 cm
• Two small about 1x1.25 cm hypoechoic lesion is noted at
renal hilum displacing right renal vein
• Ill defined hypoechoic mass like lesion left anterior
paranephric region
• No dilatation of pelvicalyceal system on any side

4. well outlined solid hypoechoic right suprarenal mass lesion

6. Two small hypoechoic mass lesion at right
renal hilum displacing RRV

10. • On the basis of ultrasound finding,
case is provisionally diagnosed as a case of
Phaeochromocytoma
• So asked for urinary catecholamine and
contrast CT scan

11. CECT scan as next imaging modality
• A well outlined about 4.5x4.5cm heterogeneously
enhanced mass at right suprarenal region
• Two small nodular lesion about 2.5x2.o cm ,0.5x0.5 cm
in right renal hilum minimally compressing and
displacing right renal vein
• Another hyperdense markedly enhanced about 4.5x2.5
cm mass lesion at left suprarenal region
• Wash out effect in delayed film
• Normal functioning both kidneys, ureter and bladder
•

16. hyperdense markedly enhanced about 4.5x2.5
cm mass lesion at left suprarenal region

22. Provisional diagnosis
• Bilateral suprarenal mass
• In correlation with strong clinical history of attack
of severe hypertension in young boy,
radiological findings are suggestive of
Phaeochromocytoma
• Patient was treated temporarily on conservative
treatment and then referred to higher center for urinary
catecholamine and further management

23. Phaeochromocytoma
• Pheochromocytoma—Greek term
phaios ..dark, chroma..color, kytos..cell,
oma..tumor
• It is the tumour of the adrenal gland with strong clinical
history and to lesser degree of imaging features
• 90% tumour arise from adrenals, others can be found
anywhere in the sympathetic system

24. Site & size … Phaeochromocytoma
• Adrenal… approximately 80% of pheochromocytomas are unilateral and
solitary, 10% are bilateral
• Extra-adrenal …. about 10%. Most are located within the abdomen in
association with the celiac, superior mesenteric, and inferior mesenteric
ganglia
• Approximately 10% are in the thorax, 1% are within the urinary bladder,
and less than 3% are in the neck, usually in association with the
sympathetic ganglia or the extracranial branches of the ninth cranial
nerves
• Pheochromocytomas usually weigh 20 to 40 g and are <5 cm in diameter
but may grow to large size up to 100gm
• Pheochromocytomas are highly vascular..

25. Phaeochromocytoma
• Pheochromocytoma is a neuroendocrine tumour arising
from the chromaffin cells of adrenal medulla
• or from extra-adrenal chromaffin tissue that failed to
involute after birth, that secretes high amounts of
catecholamine
• Extra-adrenal paraganglioma are closely related, though
less common, tumors that originate in the ganglion of the
sympathetic nervous system and are named based upon
the primary anatomical site of origin.

26. Phaeochromocytoma
• Phaeochromocytoma arising from adrenals can secrete
catecholamine
• Phaeochromocytoma arising from others sites
may also secrete other hormone which can give rise to
confusing endocrine symptoms

27. Phaeochromocytoma---10% rule
Meaning 10% could be
---Familial
---Bilateral
---Children
---Multiple/ extra adrenal
---Malignant
---May not be associated with hypertension
**some 5% cases could be associated with neurofibromatosis but reverse is less
common
Less commonly well recognized association with von Hippel-Lindau syndrome and with
medullary Ca thyroid and hyperparathyroidism

28. Classic clinical presentation
• Sign and symptoms are due to hyperactivity of sympathetic
nervous system
attacks of paroxysmal hypertension accompanied by
headache, sweating, palpitation, anxiety and tremor
• Uncontrolled secondary hypertension with hypertensive crises
• Patient may present with myocardial infarction or pulmonary
edema or severe headache or visual disturbance or
hemorrhagic stroke

29. How to investigate ?
• The first investigation in suspected cases of
pheochromocytoma is usually urinary catecholamine
• When those results are positive, then imaging is
performed to confirm the site texture and to localize
the tumour
• Imaging modalities:
…Ultrasound
... CT rather than MRI is recommended as the
first line imaging

30. Other imaging
• Rotinely CECT
• T2 weighted MRI of head,neck chest and abdomen to
localize the tumour
• Tumors can also be located using Isotope scan
MIBG using iodine 123- marked meta iodobenzyleguanidine
• Even finer localization can be obtained in certain PET- CT
centre with 18F fluorodopamine or FDOPA

31. PPGL:
Phaeochromocytoma and paraganglioma
• Suspected cases of both phaeochromocytoma or
paraganglioma should be investigated by urinary
fractionated and plasma free metanephrine
• Patients with a known germ-line mutation that predisposes
to PPGL should undergo periodic biochemical testing.
• Patients with PPGLs should participate in shared decision
making for genetic testing

32. Catecholamine

33. • Catecholamine produced by pheochromocytomas are
metabolized within chromaffin cells.
• Norepinephrine is metabolized to normetanephrine
• Epinephrine is metabolized to metanephrine.
• This process occurs within the tumor, independently of
catecholamine release
• So phaeochromocytomas are best diagnosed by
measurement of these metabolites rather than by
measurement of the parent catecholamine

34. Basal urinary catecholamine
Normal value ----urine collected for 12 hours during night
• Epinephrine---0 to 20 mg/day
• Norepinephrine ----0 to 100 mg/day
• Metanephrine ---0 to 300 mg/day
• Normetanephrine ---50 to 800 mg/day
• VMA--- 0 to 7 mg/day
• Patient with phaeochromocytoma have high urinary
catecholamine ( 3-4 times more )

35. Pathological features
• Phaeochromocytomas type of paraganglioma –
catecholamine secreting tumour
• Histologically they show nesting( Zellballen) pattern.
• Besides of well-defined clusters of tumour cells it may
contain eosinophilic cytoplasm, cystic or fat
degeneration, necrosis, calcification and fibrosis
separated by fibro vascular stroma
• These pathological features have strong correlation with
radiological picture

36. Phaeochromocytoma-- characteristic nature
• Varied and changeable appearance on radio-pathology
merits the “chameleon” epithet given to this tumor.
• A chameleon derives its name from the Greek word
khamailen, meaning “lion on the ground,” and is a reptile
characterized by the ability to change color.

37. Phaeochromocytoma
on ultrasound
• Phaeochromocytoma have a variable appearance ranging
from solid to cystic or mixed echo pattern
• As a rule Pheochromocytoma are tend to be large more than 3cm in
adrenal gland fossa
• Small extra adrenal tumour is a challenge
• About 98% are in the abdomen and 90% are confined to gland

38. Pheochromocytoma on CT scan
•CT is the first imaging modality to be used, with an overall sensitivity of
89%
•Usually large, heterogeneous masses with areas of necrosis and cystic
change
•Few may demonstrate areas of calcification or evidence of fat
•They typically enhance avidly ---characteristic enhancement & washout
• may wash out similar to an adrenal adenoma but
• tend to enhance more on the portal venous phase than the arterial
phase
• 110 HU of enhancement on the arterial phase is compatible with
pheochromocytoma
• hyper vascular metastases could be considered as differential
diagnosis in an appropriate setting

39. • Before imaging----clinical suspicion is almost always
present: secondary hypertension and positive urinary
catecholamine
• On imaging ----usually large and heterogeneous adrenal
masses, with cystic and necrotic components
• vivid enhancement of arterial/portal venous phase: > 110-
120 HU
• The diagnosis of pheochromocytoma is dependent on the imaging
identification of an appropriately located mass with accompanying
clinical and biochemical confirmation.

40. Practical points
• When an adrenal mass is found, it is important to determine
whether the mass represents a functioning tumor.
• Functioning adrenal mass---- produce endocrine disorders
(e.g., Conn syndrome, Cushing syndrome, virilizing adrenal
cortical carcinoma, pheochromocytoma)
• Nonfunctioning adrenal mass ---- adenomas

41. References
• Pheochromocytoma https://radiopaedia.org/articles/pheochromocytoma-2dr
Craig Hacking and A Prof Frank Gaillard
• Pheochromocytoma Medscape Michael A Blake,; GeorgeT Griffing, Updated: Oct
10, 2017
• Pheochromocytoma:The Range of Appearances on Ultrasound, CT, MRI, and
Functional Imaging AJR Katherine Leung1
, Michael Stamm1
, Asim Raja1
, and Gavin
Low February 2013,Volume 200, Number 2
• The adrenal glandsText book of radiology and imaging volume 1 page 825-847 by
David Sutton
• Adrenals Pheochromocytoma: An Imaging Chameleon October 2004 Michael A.
Blake, Mannudeep K. Kalra Michael M. Maher, DushyantV. Sahani, , AnnT.
Sweeney, , Peter R. Mueller, Peter F. Hahn, and Giles W. Boland,
RSNA RadiographicsVolume 24, Issue suppl_1Special Issue