A clinical case--- Adrenocortical carcinoma (Pre operatively confused as renal cell carcinoma)

1. Dr. Ravi Roshan Khadka
Phase B resident
Department of Urology
BSMMU

2. Patient Profile
 Name: Md .Sakhawat
 Age : 28 years
 Gender: Male
 Occupation: day labor in Malaysia
 Marital status : unmarried
 Fathers name : Md Mujim sheikh
 Permanent add: Dhaka
 Date of admission : 02/09/15

3. Chief complaints
1) Pain at left upper abdomen------1 year
2) Loss of appetite and nausea ---- 1 year
3) Occasional fever --------- 1 year

4. History of present illness
 According to patient’s statement he was apparently
well 1 year back and then he gradually developed pain
at left upper abdomen which was dull aching in nature
& mild to moderate in intensity. Pain radiates to back
which used to aggravated by exertion and food intake
and relieved by medication. Pain was episodic in
nature and associated with nausea and loss of appetite
and weight loss of 21 kg in 1 year. He also complaints
of occasional low grade fever which was relived by
antipyretic.

5. History of present illness
 He gave no history of coughing out of blood, blood
mixed urine, passage of stone in urine, cloudy urine or
bone pain.
 With these complaints he visited general physician
and treated symptomatically and 1½ months back after
returning to bangladesh he underwent thorough
investigations and later referred to department of
urology for further evaluation and management.

6. Past medical and surgical history
 Nothing contributory
Family history
• Nothing contributory

7. Personal history
 Education level : SSC
 Occupation: day labor in Malaysia
 Smoking/ Tobacco: non smoker
 Alcohol : non alcoholic
 Socioeconomic status : lower middle class

8. Drug history
 Analgesic, antipyretic and antiemetic and antibiotics
for symptomatic relief.

9. General physical examinations
 Appearance: anxious and illooking
 Built: average
 Weight: 54 kg height: 1.65 m BMI=19.8
 Anaemia: moderately pallor
 Jaundice: Absent
 Oedema: Absent
 Lymph nodes: Accessible lymph nodes are not palpable
 Clubbing : Absent
 Koilonychia : Absent
 Cyanosis : absent

10. Abdomen Examination
 Inspection:
 Scaphoid shape with slight bulge at left hypochondriac
region
 Umbilicus centrally placed and inverted
 Flanks are not full
 No visible peristalsis and engorged vein
 No visible cough impulse

11. Abdomen Examination
 Palpation
 Temperature ; not raised
 Mass at left hypochondriac region
 There is a Hard, mild tender, irregular mass at left
hypochondriac , epigastric and lumbar region measuring
about 8 cm from left costal margin along MCL.
 Margins are irregular and upper limit of margin couldn’t be
traced.
 Insinuation is not possible and notch is absent.
 Mass moves with respiration.
 Ballotable
 Head rising test; Mass didn’t bulge
 Knee elbow test ; Mass doesn’t fall forward
 No other organomegaly

12. Abdomen Examination
 Percussion
 Dull with band of resonance
 Tympanytic all over abdomen
 Auscultation
 No bruit
 Bowel sound ; present
 D/R/E ; Normal
 Genital exam : normal

13. Systemic examination
 CVS
 Respiratory system
 Nervous system
NAD

14. Salient feature
 Md. Sakhawat 28 years old , non diabetic,
normotensive, nonsmoker, male , hailing from dhaka
presented with gradual onset of episodic dull aching
left hypochondriac pain for 1 year. Pain was mild to
moderate in intensity that radiates to back and
aggravates by exertion and food intake and relieved by
medication. He also complaints of nausea , vomiting
and significant weight loss (21 kg in 1 year) for same
duration. He is also experiencing occasional low grade
fever.

15. Salient feature
 He gave no h/o hemoptysis, hemturia, graveluria,
pyuria or bone pain. With these complaints he visited
general physician and treated symptomatically and 1 ½
months back after returning to Bangladesh he
underwent thorough investigations and later referred
to department of urology for further evaluation and
management.

16. Salient feature
 On physical examination, vitals are stable, moderately
pallor, All accessible lymph nods are not palpable.
Jaundice and koilonychia are absent.
 Abdominal examination revealed; Hard, mild tender,
irregular mass at left hypochondriac ,epigastric and
lumbar region measuring about 8 cm from left costal
margin along MCL. Margins of mass are irregular
whose upper limit couldn’t be traced and notch is not
felt. Insinuation is not possible. Mass moves with
respiration and its ballotable .
 It is dull on percussion with band of resonance and
bruit is absent.

17. Salient feature
 No other organomegaly, ascites is absent and hernial
orifices are intact.
 Genitalia, CVS, Respiratory and CNS reveled no
abnormalities

18. Provisional diagnosis
1) Left Renal mass
2) Left Retroperitoneal extra renal mass
3) Splenomegaly

20. CBC

21. Urine R/M/E

22. +
USG
W/A
Lt
Rt

24. HORMONE ANALYSIS

26. CT SCAN

27. C
E
C
T

32. After one pint of blood transfusion

33. OTHER INVESTIGATIONS

34. INVESTIGATIONS

35. Hb Electrophorosis
INVESTIGATIONS
•Serum ferritin = 459 ng/ml (20-300 ng/ml)
• Montoux test = negative

36. CLINICAL DIAGNOSIS
1) Left adrenal mass
2) Left Renal mass

37. Left sided Radical Nephrectomy
Date:15/09/15

38.  Position
 Under G/A patient kept
in supine position with
bridge beneath left
flank and painting and
draping done.
 Incision
 modified chevron /
hockey stick (extended
left sub costal).
 Intervening layers are
incised along the line of
incision

39.  Abdomen cavity
reached and findings
noted
 Large ,hard irregular
mass seen subcostally
which was pushing
pancreas and spleen
upward.
 Mass was adherent to
transverse mesocolon
and diaphragm
 Multiple dilated vessels
were seen over mass.
 Operability assessed.

40. • Dissection was
started laterally by
dissecting
posterior
peritoneum along
left colon and
carried out
proximally.
• Adherent transverse
mesocolon was
clamped, cut and
ligated to free it
from mass and
hence colon was
reflected medially.

41. • Dilated vessels over mass
were clamped, cut and
ligated which made the
mass slightly free.
• Blunt and sharp dissection
carried out along the lower
border of mass as there was
still confusion regarding
origin.
• As the upper pole of kidney
was reached ,a plane was
found between mass and
kidney hence dissection
was continued but after a
while plane obliterated
hence Nephrectomy was
planned, thinking of mass
arising from upper pole of
kidney

42.  Dissection was carried
out laterally and
posteriorly

43.  Superiorly a plane was
found between panaceas
and the mass and hence
dissection carried out
along the plane .

44.  Medial dissection
 Renal hilum was approached and dissection was carried out
to identify renal vessels separately.
 First renal artery and then renal vein were clamped, cut and
ligated separately by 1/0 silk.
 Ureter was identified , clamped, cut and ligated.

45.  While dissecting
medially ,another
large vessel found
reaching kidney
which was clamped,
cut and ligated (main
renal artery).
 Finally blunt
dissection carried
laterally and
posteriorly to remove
specimen

46.  After removing specimen,hot mop was placed and then
observed for any bleeding.
 After ensuring haemostatis 16 fr drain kept in situ
 Abdomen was closed in layer by 1/0 vicryl and skin closed
by stapler

50. Follow up ----1st POD
 Subjective
 Pain abdomen and one episode of fever at evening (101 F)
 Objective
 BP: 100/70 mmHg , pulse: 84/min
 T: 98.4deg F , R/R = 16/min
 Lung; B/L expansion normal, Vesicular breath sound and no
added sound,
 Heart : NAD
 P/A : Mild tenderness, soft, Bowel sound absent and dressing
dry.
 Drain : 270 ml ,reddish
 Urine Output : 2800ml, clear
 Plan: I/V fluids and antibiotics, analgesic, Chest physio and
limb exercise.

51. Follow up ----2nd POD
 Subjective
 Pain abdomen and one episode of fever at evening (100.4 F)
 Objective
 BP: 110/70 mmHg , pulse: 80/min
 T: 98.6deg F , R/R = 16/min
 Lung; B/L expansion normal, Vesicular breath sound and no
added sound,
 Heart : NAD
 P/A : Mild tenderness, soft, Bowel sound- absent and
dressing dry.
 Drain : 40-50 ml ,blackish
 Urine Output : 1900ml, clear
 Plan: I/V fluids and antibiotics, analgesic ensured, Chest
physio and limb exercise.

52. Follow up ----3rd POD
 Subjective
 Pain abdomen an one episode of fever at evening (100.6 F)
 Objective
 BP: 100/60 mmHg , pulse: 86/min
 T: 98.2deg F , R/R = 14/min
 Lung; B/L expansion normal, Vesicular breath sound and no
added sound,
 Heart : NAD
 P/A : Mild tenderness, soft, Bowel sound- present and
dressing dry.
 Drain : 20 ml ,blackish
 Urine Output : 1900ml, clear
 Plan: sips of water, I/V fluids and antibiotics, analgesic
ensured, Chest physio and limb exercise,

53. Follow up
 4th POD
 Slight pain
 BP: 100/60 mmHg, Pulse = 82/min,
 Swelling and mild tenderness at cannula site
 Heart= NAD
 Lung =NAD
 P/A = Soft, mild tender, BS + , Dressing dry
 Drain : Nil
 Plan:
 Oral, catheter off, i/v cannula changed
 CBC, S. Creatinine and CXR-P/A sent

57. Follow up
 5th POD
 Slight pain
 BP: 110/70 mmHg, Pulse
= 86/min,
 Heart= NAD
 Lung =NAD
 P/A = Soft, mild tender,
BS + , wound healthy
 Drain : Nil
 Plan: oral , drain off,
check dressing .
6th POD
 No fresh complaint
 BP: 100/60 mmHg,
Pulse = 80/min,
 Heart= NAD
 Lung =NAD
 P/A = Soft, mild tender,
BS + , dressing dry
 Plan:
 Anxiolytics

58. Follow up
 7th POD
 No fresh complaint
 BP: 100/70 mmHg,
Pulse = 82/min,
 Heart= NAD
 Lung =NAD
 P/A = Soft, mild
tender, BS + , dressing
dry
8th POD
 No fresh complaint
 BP: 100/60 mmHg,
Pulse = 80/min,
 Heart= NAD
 Lung =NAD
 P/A = Soft, mild
tender, BS + , dressing
dry
 Plan : Alternate stitch
off

60. Final diagnosis
 Adrenocortical carcinoma
 Stage III (T3N0M0)

61. Short review

62. Introduction and Epidemiology
 Rare malignancy with an incidence of 0.5 to 2 per
million.
 Bimodal age distribution that peaks in children in the
first decade of life and adults in the fourth to fifth
decdes of life.
 Slight female predominance of 1.5 to 2.1
 Majority of ACCs are sporadic and unilateral
 loss of TP53 function and increased IGF expression
represent late events in the tumorigenesis of sporadic
ACC

63. Clinical Characteristics
 Incidental detection.
 Tumor-related symptoms
 Local symptoms: abdominal fullness, back pain, nausea
and vomiting.
 Systemic / metastatic futures: Anorexia, anaemia,
asthenia and weight loss and features of metastasis.
 Hormone secreting ACC (50% to 79% of adult and 90%
of pediatric ACCs)

64. Clinical Characteristics
most common hormone secreted by adrenal cortical carcinoma
is cortisol

65. Investigations

66. Imaging- CT scan/ MRI
 Size
 Relative indicator of malignancy, with
 4% to 5% of tumors less than 4 cm, 10% of tumors larger than
4 cm, and 25% of tumors greater than 6cm.
 Common radiographic characteristic of ACC on CT
imaging include
 the presence of irregular borders,
 irregular enhancement,
 calcifications
 Necrotic areas with cystic degeneration.
 Evaluation of spread/mets
 IVC, Adjacent organs, Liver ,lungs, Bones

67. Role of biopsy
 For diagnosing adrenal cortical carcinomas,
percutaneous needle biopsy is generally not performed
prior to surgical excision
 due to a clinically unacceptable risk of needle-tract
seeding (Fassnacht et al, 2004; Schteingart et al, 2005).
 The primary indication for needle biopsy is
 in cases of unresectable, locally advanced, or metastatic
disease, to confirm the diagnosis prior to systemic
medical therapy.

68. Pathologic Evaluation.

69. •Stage I and II tumors are confined to the adrenal gland and are
distinguished by a size cutoff of 5 cm.
•Stage III disease includes tumor extension into adjacent adipose tissue or
having regional lymph node involvement.
•Stage IV disease includes tumors invading adjacent organs and the
presence of distant metastatic disease.

70. Management
 Unfortunately, the majority of patients with
adrenal cortical carcinomas present with advanced
disease those who do have localized disease are at a
high risk of local recurrence and metastatic
progression.
 Treatment of ACC often includes multimodal therapy
directed by a team of surgeons, medical oncologists,
endocrinologists, and radiation oncologists.
 Despite aggressive surgical resection, adrenal
carcinoma is associated with a high rate (60% to
80%) of recurrent disease

71. Surgery
 Complete surgical excision is essential in the management of
adrenal cortical carcinomas
 En bloc resection of surrounding organs involved with locally
advanced disease should be performed whenever possible.
 Cases of venous tumor thrombus involving the IVC may require
vascular bypass techniques, IVC replacement, and/or IVC interruption.
 In cases of metastatic adrenal carcinoma,
 Cytoreductive removal of the primary tumor and debulking
metastatectomy should be considered if greater than 90% of the disease
burden can be removed.
 Although debulking surgery may not improve survival, it may alleviate
tumor-related side effects and facilitate additional therapies .
 Local or distant disease recurrences, following initial resection,
should be considered for surgical excision and have been associated
with improved survival in retrospective series.

72. RADIATION
 Currently there is a limited role for radiation for
therapy in the treatment of primary adrenal cortical
carcinomas;
 Radiation therapy remains the treatment of choice in
 Management of bone and CNS metastasis
 To decrease local recurrence rates following complete
tumor resection, (with reported local recurrence rates
of 14% and 79%, with and without adjuvant radiation
therapy, respectively )
 Unfortunately, a significant improvement in disease-
free or overall survival was not observed.

73. MEDICAL THERAPY
 Mitotane is most commonly used chemotherapeutic
agent in the treatment of adrenal cortical carcinoma.
 Benefit in the adjuvant setting following surgical
resection and in patients with metastatic disease
 A significant increase in recurrence-free survival and
overall survival was noted in patients receiving
mitotane compared with controls

74. Follow up
 Follow-up should include CT examination of the chest,
abdomen, and pelvis every 3 months for the first 2
years.
 In patients with evidence of functional tumors,
measurement of the initially elevated hormones
postoperatively may help to reveal early disease
recurrence despite negative radiographic studies.
 After a disease-free interval of 2 years, surveillance
should continue, but the frequency of imaging may
decrease.

75. Prognosis
 Overall 5-year survival in adrenal cortical
carcinomas is poor, ranging from 20% to 47%.