Fever on & off -1 yr, Lt upper abdominal

heaviness :3 mo
Occasional palpitations & sweating
HTN- on drugs, DM-recently detected
Case 3, 63/M
Clinical Presentation
HTN- on drugs, DM-recently detected
BP:130/80 mmHG
Palpable mass left hypochondrium, Firm
Bimanually palpable
Left renal angle fullness, Lt Varicocele
USG abdo, CE-CT- Left supra-renal mass,
Refd to SGPGI
24 hr Urinary
MN :123 micg (0-300)
NMN :307 micg (0-600)
ONDST- suppressable
DHEAS-7.6 pmol
Case 3, 63/M
Investigative Work-up
DHEAS-7.6 pmol
Chest xray- NAD
CECT- 12 x 11.8 x 15.7 cm
non enhancing mass lt
adrenal area
Tumour in-seperable
from left kidney.
Multiple upto 3cm LNs
Case 3, 63/M
Operative Management
En-bloc radical excision with left nephrectomy
(Adreno-Nephrectomy) with RPLND
Multiple upto 3cm LNs
in para-aortic and
aorto-caval region
RPLND done
Case 3, 63/M
Surgical Pathology
Wt (with kidney) 2kg
Tumour size 14x10x10 cm
Cut section- fleshy
Heterogenous, areas of
necrosis, Calcification
Histopathology for discussion
Gross Gross
Retroperitoneal mass with left nephrectomy Retroperitoneal mass with left nephrectomy
(20x14x13cm) (20x14x13cm)
Tumor at superior pole of kidney Tumor at superior pole of kidney
Separated from the kidney by a capsule Separated from the kidney by a capsule
RPLND specimen (~24 LNs)
-- tumour was encapsulated tumour was encapsulated
-- measured15x10x10cm measured15x10x10cm
-- Cut surface Cut surface variegated variegated
TUMOUR WITH CAPSULE
CORDS OF TUMOUR CELLS
NESTED PATTERN
TUMOUR WITH NECROSIS
NECROSIS AND HEMORRHAGE
Microscopy Microscopy ::
Encapsulated Encapsulated tumor, tumor, well well demarcated demarcated from fromthe the kidney kidney
Arranged Arranged in in sheets, sheets, cords, cords, trabecular trabecular pattern pattern and and nests nests
interspersed interspersed by by thin thin fibrovascular fibrovascular septae septae
Large Large round round to to polygonal polygonal cells cells with with mild mild pleomorphic pleomorphic
nuclei,fine nuclei,fine to to coarse coarse chromatin, chromatin, occasional occasional prominent prominent
nucleoli nucleoli..
Mitosis Mitosis >>55//50 50 HPF HPF were were evident evident
Clear Clear cytoplam cytoplam<< 25 25%% tumor tumor cells cells Clear Clear cytoplam cytoplam<< 25 25%% tumor tumor cells cells
Large Large areas areas of of hemorrhage hemorrhage and and necrosis necrosis
No No evidence evidence of of capsular capsular or or vascular vascular invasion invasion..
No No evidence evidence of of LN LN or or metastasis metastasis..
Vimentin Vimentin +
Synaptophysin Synaptophysin +
NSE NSE +
CK CK -
Chromogranin Chromogranin -
VIMENTIN
SYNAPTOPHYSIN
Modified Weiss system for separating benign from
malignant adrenocortical neoplasms (Aubert et al Am J
Surg Pathol 2002;26:1612-9)
Criteria Score Present Case
1. Mitosis >5/50 HPF 2 2
2. Clear cells < 25% 2 2 2. Clear cells < 25% 2 2
3. Necrosis 1 1
4. Capsular invasion 1 -
5. Atypical Mitosis 1 -
A total score of 3/7 correlates with subsequent malignant behaviour
In present case score was 5/7
FINAL DIAGNOSIS :
ADRENOCORTICAL CARCINOMA
Other Scoring systems
Hough et al - 1979
Weiss criteria 1984
Van Slooten et al 1985
Modified Weiss - 2002 Modified Weiss - 2002
AFIP criteria for pediatric tumors 2003
Uneventful recovery
Negative metastatic work-up
Normotensive off drugs, euglycemic without
drugs
Case 3, 63/M
Post-op Course
drugs
Wound healed well
Free of recurrence, mets in limited follow up