Episodic headache, sweating and palpitation- 5 yrs

Decreased visual acuity

Fundus : Retinal hemorrhages, macular degeneration BE.
Detected hypertensive
Evaluated by endocrinologist for secondary HT
Case 2, 23/M
Clinical Presentation
Evaluated by endocrinologist for secondary HT
Left suprarenal mass on USG abd, refd to SGPGI
Family History- not significant
BP :210/120 mm Hg supine, 180/120 standing
No marfanoid Habitus/ MEN2B phenotype
Single caif au lait spot antr abd wall 5 cm in size
24 hour URINARY
MN : 307 micg ( <350)
NMN : 298 micg ( <600)
Case 2, 23/M
Investigative Work-up
USG Abd: 6.6x3 cm mass
inseparable from lt kidney in
suprarenal area
Serum DHEAS: 1.4 nmol/L
(3.6-11.9 umol/L )
ONDST was suppressible
ENHANCING MASS ANTR ENHANCING MASS ANTR
TO UPPER POLE OF LT TO UPPER POLE OF LT
KIDNEY 5.1 X4 CM KIDNEY 5.1 X4 CM
HYPODENSE mass 20X 15 HYPODENSE mass 20X 15
MM IN LT RENAL VEIN MM IN LT RENAL VEIN
upto the IVC upto the IVC
Case 2, 23/M
Investigative Work-up Contd
upto the IVC upto the IVC
Colour Doppler:
dilated left adrenal &
renal vein with thrombus.
IVC thrombus free
Case 2, 23/M
Operative Management
Left Adrenalectomy with Thrombectomy
(post- alpha blockade)
Well encapsulated Lt Adrenal mass extending upto lt renal hilum
Adhered to postr wall of stomach, tail of pancreas
Tumour thrombus in lt adrenal vein, renal vein
Tumour removed in toto with thrombus
5.5x4.5x 3.5 cm
Wt- 40 gms
Max BP- 190/ 110 mm
Min BP- 80/50 mm
C/S- fleshy, yellow well
Case 2, 23/M
Surgical Pathology
C/S- fleshy, yellow well
encapsulated with few
areas of haemorrhage
Thrombus directly extending
from tumour
Histopathology for discussion
Gross specimen
Left adrenalectomy specimen measuring
6.5x4.5x3.5cms
Outer surface was bosselated Outer surface was bosselated
Cut surface showed an encapsulated, yellowish
brown tumor measuring 5x3x2.5cms
Thrombus from left renal vein measuring 1x1cm
Normal adrenal measured 2x1.5cms
Microscopic findings
Capsule
Alveolar pattern
Alveolar pattern/ Zallbellen pattern
Trabecular pattern
Round to polygonal cells with amphophilic cytoplasm
Necrosis
Tumor
Normal adrenal tissue
Normal adrenal tissue
Thrombus
Final Diagnosis
Left Adrenal mass :
PHAEOCHROMOCYTOMA WITH TUMOR THROMBUS
Malignant Pheochromocytoma
Pheochromocytoma of Adrenal Gland Scaled Score
1. Capsular invasion
2. Vascular invasion
3. Extension into periadrenal
adipose tissue
4. Expanded large and
confluent nests
5. Diffuse growth
9. Profound cell and nuclear
pleomorphism
10. Cellular monotomy with high
N/C ratio
11. Nuclear hyperchromasia
12. Macronucleoli
13. Increased mitotic figure 5. Diffuse growth
6. Necrosis
7. Increased cellularity
8. Tumor cell spindling
13. Increased mitotic figure
(more than 3/10 or 20HPF)
14. Atypical mitoses
15. Absence of hyaline globules
Score of 4 suggests potential malignant biological behavior
Uneventful.
Normotensive without drugs
Post op 24 hr Uri MN/NMN: normal
Case 2, 23/M
Post-op Course
Post op 24 hr Uri MN/NMN: normal
Wound healed well