A CASE OF

RENAL CELL CARCINOMA

IN A PATIENT WITH
TUBEROUS SCLEROSIS

PRESENTER – DR. PIYUSH THOMBARE

MODERATOR – DR. JAGADISH B
PROF AND HOD
DEPT OF GENERAL SURGERY
 2
 Patientname – mr X
 Age – 72 years.
 Sex – Male.
 Occupation – FARMER.
 Residence – PERIYAPATNA.
 Date of admission – 4th sept17
Chief complaints:- 3

 Pain
abdomen in rt lower
quadrant since 1 month
History of presenting illness:
4
• Patient complained of pain
abdomen in the right lower quadrant
since 1 month.
• It was insidious in onset, dull aching
type ,continuously present .
• It radiated to the back , no
aggravating or relieving factors
 5
• No h/o haematuria.
• No h/o burning micturition.
• No h/o nausea or vomiting .
• no h/o altered bowel habits like diarrhoea or
constipation.
• h/o loss of weight present
• h/o loss of appetite present.
• No h/o yellowish discoloration of urine/
cough/bony pain
• No h/o fever
 6
• Pt also complained of multiple swellings
over face since childhood
• No h/o seizures
 7
PAST HISTORY:-
• No history of similar complaints in the past.
• No h/o previous surgeries.
• No history of diabetes mellitus.
• No history of hypertension.
Family history:- 8

 h/o similar skin lesions in brother and

daughter.
 h/o epilepsy present in the daughter.
 Personal history:-. 9

• Diet – mixed.
• Appetite – reduced.
• Sleep –normal
• Bladder and bowel habits – normal &
regular.

• Pt was chronic alcoholic and chronic

smoker .
 Physical examination—
10
General survey-
• 72 year old male patient moderately built
and nourished.
• Patient was afebrile
• No Pallor
• No Icterus .
• No generalised lymphadenopathy.
• No Pedal edema .
• Pulse – 8 6 beats/min normal rhythm,
character, volume, no vessel wall
thickening.
• B.P – 120/70 mm hg .
Skin lesions

Adenoma sebaceum
Periungual fibromas
Systemic examination 14

 Per abdomen –
Inspection
fullness seen in rt lumbar region extending into rt
hypochondriac region
No visible peristalsis
No dilated veins
No scars
Hernial orifices normal
Rt renal angle was full
Palpation
 A Mass was felt in rt lumbar region
 Of size – 12*8 cm
 Shape - ovoid
 surface - smooth
 Borders – medial lateral and inferior border well defined.
superior not well defined
 Consistency – firm to hard
 Tenderness – mild tenderness present
 Bimanually palpable
 Ballotable
 Movement with respiration present
 No Hepatomegaly no splenomegaly
 on knee elbow position – mass doesn’t fall forward
 No other mass felt

 Percussion – mass was dull to percuss

 Renal angle – dull to percuss.
 No free fluid in the abdomen

 Auscultation – normal bowel sounds were heard.

 C . N . S - clinically normal
 C . V . S - clinically normal
 R . S – clinically normal
 p/r – gr1 prostratomegaly
Diagnosis 18

RT SIDE HYDRONEPHROSIS
RT RENAL CELL CARCINOMA
ANGIOMYOLIPOMA
WITH TUBEROUS SCLEROSIS COMPLEX.
Investigations. 19
• Hb -10.1gm%
• TLC – 2,400 cells/dl
• DLC - Polymorphs – 68%
Lymphocytes – 22%
Monocytes –10%
PLATELETS – 1.52 LAKHS/ cumm
• ESR – 115mm/hr.
• RBS- 86 Mg/dl
• Blood group – O positive.
RUE 20
• Pus cells – 6-8 cells / hpf
• Rbc – nil cells/hpf
• Epithelial cells – 1-2 cells/hpf
• Casts – nil

• RFT
• Blood urea- 106 mg/ dl
• Serum creatinine – 2.9 mg/dl
LFT 21

• Total bilirubin – 0.3 mg/dl

• Direct bilirubin – 0.2 mg/dl
• Total protein – 6.3 g/dl
• Albumin- 3.3 g/dl
• Sgot- 46 u/l
• Sgpt – 41u/l
• Alp – 675 u/l
Sr electrolytes

 Sodium – 136 meq/l

 Potassium – 4.7 meq/l
 Chloride – 102 meq/l
ULTRASOUND REPORT (12/9/17) 23

 Large well defined heteroechoic lesion in rt

lumbar region arising from the right kidney
 ? Renal Angiomyolipoma
 ? Renal cell carcinoma.
M.R.I (11/8/17) (Done outside)

 Large heterogeneous lesion in retroperitoneum

from posterior mid and lower pole cortex of rt
kidney compressing and displacing the same –
possibly haemorrhage from underlying
Angiomyolipoma
Ct scan report

 Well defined thick walled heterogeneous density mass

lesion with subtle central hyperdensity arising from mid
and inferior poles of right kidney
 In view of tuberous sclerosis complex possibility of lipid
poor Angiomyolipoma with haemorrhage to be
considered
Heterogeneous
mass
 27

 Patient was posted for surgery (rt

nephrectomy) on 20th september 2017.
OPERATIVE NOTES
 In supine position after painting and draping,
 A rt transverse oriented incision was made
 Peritoneum opened
 Small bowel packed on left side
 Peritoneum on the posterior wall was incised along the
lateral wall of ascending colon hepatic flexure and
second part of the duodenum
 The colon was mobilized and displaced medially to
expose the anterior surface of the kidney and its
pedicle.
Renal artery
 Dissection around the kidney done for full
mobilization of the organ.
 Renal Artery, renal vein and ureter (proximal one
third) were ligated separately in continuity and
divided. And nephrectomy was done
 No lymphadenopathy , no perinephric fat
infiltration was found.
 Drain was placed and skin closed in layers.
Renal pelvis
cut section
Postoperative period
 Postoperatively patient developed hypertension on
day 1 and was treated with amlong 5 mg od and
atenolol 50 mg od
 On postop day 1 pt developed hyperkalemia – k-6.5
meq/l . And was treated with inj 10iu plain insulin in 25d
iv over 30 mins.
 K was 5.3 meq/l on pod2 and 4.3 on pod7
 S creatinine was 2.4 on pod9
 Post operative urinary secretion was adequate.

 Foleys catheter and drain were removed on pod-7

 Sutures were removed on pod-9

Histopathology report

 Histopathologyreport was Renal Cell

Carcinoma ( Chromophobe Type) Right
Kidney.
Polygonal cells with slightly enlarged nucleus with
pleomorphism and hyperchromasia, abundant pale
eosinophilic cytoplasm with perinuclear cytoplasmic clearing
at places
 Patient was discharged on pod 15 and was
referred to higher center for further
management.
 Pt didn’t come for follow up but on phone
conversation pt was doing well .
 39

Discussion
TUBEROUS SCLEROSIS (BOURNEVILLE’S
DISEASE)

 Tuberous sclerosis (TS) is an autosomal dominant disorder

affecting 1 out of 6000 people. It results from inactivating
mutations in either the TSC1 gene encoding tuberin or the
TSC2 gene encoding hamartin
 Tuberous sclerosis is characterized by cutaneous lesions,
seizures, and mental retardation.
 The cutaneous lesions include adenoma sebaceum (facial
angiofibromas), ash leaf–shaped hypopigmented macules
, shagreen patches , and depigmented nevi.
 presence of subependymal nodules, is characteristic.

 the kidneys are affected in 80% of patients.

 Renal TS occurs in three forms:
 renal cysts, renal angiomyolipomas, and renal cell
carcinoma.
 Angiomyolipomas are the most common renal abnormality,
occur bilaterally, and are usually asymptomatic.
 Twenty to 30 percent of angiomyolipomas are in patients
with TSC, and approximately 50% of patients with TSC
develop angiomyolipomas
 Multicentric renal cell carcinomas have been reported
with increased frequency in TS.
 Spontaneous bleeding can produce flank pain, nausea
and vomiting, hematuria, and even life-threatening
retroperitoneal hemorrhage. Large lesions, >4 cm, are
most likely to cause pain and bleeding, and may require
surgical excision.
 RCC, which accounts for 2% to 3% of all adult malignant
neoplasms, is the most lethal of the common urologic
cancers. with a male-to- female predominance of 3 : 2

 This is primarily a disease of the elderly patient, with typical

presentation in the sixth and seventh decades of life

 The majority of cases of RCC are believed to be sporadic;

only 2% to 3% are familial

 The most commonly cited risk factors include smoking,

obesity, and hypertension.
 The major histologic variants include

 clear cell,
 papillary,
 chromophobe , and
 collecting duct tumors,
Chromophobe Renal Cell
Carcinoma
 Chromophobe RCC, first described by Theones and
colleagues in 1985.
 It appears to be derived from the cortical portion of the
collecting duct .
 It represents 3% to 5% of all RCCs
 The tumor cells typically exhibit a relatively transparent
cytoplasm with a fine reticular pattern that has been
described as a “plant cell” appearance
Clinical features of RCC
 Local Tumor Growth
 Hematuria
 Flank pain
 Abdominal mass
 Perirenal hematoma
 Metastases
 Persistent cough
 Bone pain
 Cervical lymphadenopathy
 Weight loss/fever/malaise
 Surgery remains the mainstay for curative treatment of
this disease.

The objective of surgical therapy is to excise all tumor with

an adequate surgical margin.
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