This document discusses pheochromocytoma and neuroblastoma. It provides details on: 1) Pheochromocytoma - an uncommon adrenal gland tumor that follows a 10% rule and can be associated with conditions like MEN2 or VHL disease. It typically presents with uncontrolled hypertension and may be found using tests like urinary metanephrines. 2) Neuroblastoma - the most common extracranial childhood malignancy that can occur anywhere along the sympathetic chain but most often the adrenal gland. It may be associated with conditions like Beckwith-Wiedemann syndrome. Symptoms vary based on location and size of tumor. 3) A case example is provided of

1. PHEOCHROMOCYTOMA
&
NEUROPBLASTOMA
DR. ROHIT
PG-2

2. PHEOCHROMOCYTOMA
Uncommon tumor of the adrenal gland, with characteristic clinical, and to a
lesser degree, imaging features. The tumors follow a 10% rule:
• ~10% are extra-adrenal
• ~10% are bilateral
• ~10% are malignant
• ~10% are found in children
• ~10% are not associated with hypertension
• ~10% contain calcification

3. ASSOCIATION
• The majority of cases are sporadic. In 25% of cases, a pheochromocytoma is a manifestation of an underlying
condition, often familial, including :
• multiple endocrine neoplasia type II (MEN2): both MEN IIa and MEN IIb
• account for 3% of all pheochromocytomas
• almost never extra-adrenal
• almost always bilateral
• von Hippel-Lindau disease
• neurofibromatosis type 1
• Sturge-Weber syndrome
• Carney triad: for extra-adrenal pheochromocytoma
• tuberous sclerosis
• familial pheochromocytoma

4. Clinical presentation:
• Rare but classical cause of uncontrolled secondary hypertension
• may also present with:
- Cardiac dysfunction (myocardial infarction, pulmonary edema)
- Neurological events : severe headache
visual disturbance,
Hemmorrhagic stroke
Ist investigation : 24-hour urinary or plasma metanephrine,
metabolites of norepinephrine and epinephrine

5. LOCATION :
• Mostly arise from the chromaffin cells of the adrenal medulla.
• 10% Extra-adrenal tumors are more likely to be malignant and
• found along the sympathetic chain as well as in the urinary
Zuckerkandl.
• Thoracic paragangliomas :1-2%

6. USG

7. CT :
CT=First imaging modality to be used with 89% sensitivity

11. MRI
most sensitive modality (98%) .
• T1
• slightly hypointense to the remainder of the adrenal
• if necrotic and/or hemorrhagic then the signal will be more
• T2
• some are markedly hyperintense (lightbulb sign)
• ~1/3 do not have marked T2 hyperintensity 18
• areas of necrosis/hemorrhage/calcification will alter the signal
• in/out-of-phase: no signal loss on out-of-phase imaging
• T1 C+ (Gd)
• heterogeneous enhancement
• enhancement is prolonged, persisting for as long as 50 minutes 4

12. IN PHASE-T1 OUT OF PHASE- T1

13. AXIAL T2
CORONAL T2
AXIAL T2 FAT SAT

14. ‘LIGHT BULB’ sign in PHEOCHROMOCYTOMA

15. -123 MIBG (metaiodobenzylguanidine)
• I-123 MIBG uptake in an adrenal nodule is strong supporting
pheochromocytoma. Overall sensitivity is ~80%
• However, as many neuroendocrine tumors demonstrate
not as specific for pheochromocytoma outside of the

16. NEUROBLASTOMA
• Neuroblastic in origin
• They may occur anywhere along the sympathetic chain, the vast majority
arise from the adrenal gland.
• most common extracranial solid childhood malignancy
• third commonest childhood tumor after leukemia and brain malignancies

17. Associations
• The vast majority of neuroblastomas are
instances, they may be associated with :
• Beckwith-Wiedemann syndrome
• central failure of ventilation
• DiGeorge syndrome
• Hirschsprung disease
• neurofibromatosis type 1

18. Clinical presentation
• Pain
Palpable Mass
Abdominal Distension
other may be due to local mass effect.
• Other accompanying syndromes include:
• Hutchinson syndrome: bone metastases may present with pain or limping and irritability or
proptosis with periorbital and cranial bumps
• Pepper syndrome: hepatomegaly due to extensive liver metastasis
• blueberry muffin syndrome: multiple cutaneous lesions
• opsomyoclonus : rapid, involuntary conjugate fast eye movements
• proptosis and periorbital ecchymoses ("raccoon eyes"): orbital metastases

19. Location
• adrenal glands: most common site of origin, 35%
• retroperitoneum: 30-35%
• organ of Zuckerkandl
• celiac axis
• paravertebral sympathetic chain
• posterior mediastinum: 20%
• neck: 1-5%
• pelvis: 2-3%

20. • Non-specific,
• Intrathoracic soft-tissue mass or an intra-abdominal mass displacing adjacent organ
• Pressure on adjacent bones may cause remodeling of ribs, vertebral bodies or pedicle thinning. Up to 30% may
have evidence of calcification on the plain film.
• Bone metastases are usually ill-defined and lucent (i.e. osteolytic), with periosteal reaction or metaphyseal
lucency. Sclerotic bone metastases are uncommon 2
PLAIN RADIOGRAPH

21. USG

22. CT

24. MRI
• T1: heterogeneous and iso to hypointense
• T2
• heterogeneous and hyperintense
• cystic/necrotic areas very high intensity
• C+ (Gd): variable and heterogeneous enhancement

25. CASE
A one-year-old girl was admitted to hospital with abdominal distention of two days and
abdominal discomfort of 10 days' duration.. Routine laboratory findings were within
normal limits, except for elevated urine vanillyl mandelic acid (VMA) levels (157 mg/L)
O/E – Palpable mass in right hypochondrium
USG – b/l adrenal anechoic cystic lesions with thick irregular walls.

26. MRI

28. CASE

32. THANK YOU