Adrenal lymphoma

KG, 32/M, 2012090710

Referred as a case of
bilateral adrenal
masses with c/o-
Right upper abdominal
pain- 2 months, vague,
No history of-
Headache
Sweating
Palpitations
Visual blackouts
Postural giddiness
2
pain- 2 months, vague,
no shifting or migration
High grade fever- 2 days
Maximum BP recorded-
140/100 mm Hg
Postural giddiness
Tremors
Weakness
Graveluria
Neck swelling
Family History
70 yrs
Cause?
Young age
Cause ?
3
Accidental
death
70 yrs
Heart attack
Examination
No neurocutaneous markers
P/R- 80/min, regular in rhythm
BP-
Supine- 140/90 mm Hg
4
Supine- 140/90 mm Hg
Standing- 130/84 mm Hg
Neck- Single mobile LN in left level 2
Abdomen- NAD
USG abdomen
Bilateral large adrenal
masses-
Left-
11.4 x 10.0 x 9.3 cm
Predominantly
5
Predominantly
hypoechoic
Right-
11.6 x 8.6 x 7.4 cm
Predominantly
hypoechoic
Kidneys normal
Biochemical evaluation elsewhere
Hormone Value Normal range
24-hr urinary metanephrine 6.93 g/day 53-341
24-hr urinary metanephrine 4.01 g/gm creatinine 27-155
24-hr urinary normetanephrine 1530.84 g/day 88-444
24-hr urinary normetanephrine 885.13 g/gm creatinine 46-256
24-hr urinary Creatinine 140.61 mg/dl
6
24-hr urinary Creatinine 140.61 mg/dl
24-hr urinary VMA 140.61 ng/dl 1.8-6.7
24-hr urinary VMA 30.98 mg/gm creatinine 1.6-4.2
S. Cortisol 83.0 ng/ml 30-150
Potassium 4.2 mmol/L 3.5-5.4
S. ACTH 64.4 pg/ml 7.9-66.1
24-hr urinary Cortisol 30.4 g/day 28.5-213.7
Chest X-ray
7
CECT abdomen
8
CECT abdomen
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CECT abdomen
10
CT-Angio
11
MRI abdomen
12
Treatment elsewhere
Diagnosis made as bilateral adrenal
Pheochromocytoma
Patient was given -blockers that were
increased to 30 mg/day along with -
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increased to 30 mg/day along with -
blockers
Was planned for surgery elsewhere but
Surgeons refused; said it was inoperable
mass
Differential Diagnosis
Bilateral adrenal masses-
?Pheochromocytoma
?? Lymphoma
??? Adrenocortical Carcinoma
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??? Adrenocortical Carcinoma
Course @ SGPGI
Due to confusion in diagnosis, plan was to work
up again
Fever persisted despite antimalarials, antibiotics
Decreased oral intake with increasing pain
abdomen
15
abdomen
Plan-
FNAC from neck LN
Repeat 24-hour urinary fractionated metanephrines
Hematology, Biochemistry, cultures, serology,
malarial parasite (PBS)
Investigations @ SGPGI
FNAC neck LN- Predominance of large
atypical lymphoid cells, possibility of
lymphoproliferative disorder
Cultures- Blood, Urine, Sputum- Negative
16
Cultures- Blood, Urine, Sputum- Negative
for bacterial growth
Peripheral smear- negative for malarial
parasite
HIV, Widal- non reactive
Investigations @ SGPGI
Hormone Value Normal range
24-hr urinary metanephrine 26.43 g/day <350
24-hr urinary
normetanephrine
512 g/day <600
24-hr urinary Creatinine 0.937 mg/day
17
24-hr urinary Creatinine 0.937 mg/day
(vol 1600 ml)
LDH 2,152 U/L 85-450
TLC <4,000/L (persistently) 4,000-10,000
DLC, Blood film Normal
Tc
99m
MDP Bone scan Negative for skeletal
metastasis
Repeat CECT abdomen
Right- 11x10x8 cm
Left- 11x10.5x6 cm
Few necrotic areas
No calcification/ hge
Encasement of celiac,
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Encasement of celiac,
splenic and renal arteries
and veins and a focal
segment of aorta
Bilateral pleural effusion
Histopathology from Adrenal gland
Lymphoblastic lymphoma
Bone marrow infiltration absent
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+ve- -ve-
LCA, CD3 - CD20, TdT
Ki-67- 70-80%
Immunohistochemistry
Further course
Given chemotherapy first cycle- CHOP
Partial improvement in symptoms
Fever subsided
Intake improved
20
Intake improved
Patient discharged in stable condition after
one week of chemotherapy
Course @ home
Patient developed fever, vomiting and
dizziness after 10 days
Admitted at local hospital for supportive
management and discharged
21
management and discharged
Further course
Patient developed fever and cough with
anemia
Admitted in shock
SGOT- 260, SGPT- 158 U/L, progressively
22
SGOT- 260, SGPT- 158 U/L, progressively
rising (502, 166), viral markers negative
LDH increased to 4,602 U/L
Patients condition did not improved and
succumbed 13 days after admission
THANK YOU
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