A case of Wilms Tumor with thrombus extending into Right Atrium of heart

1. 2 YEARS FEMALE WITH
LEFT FLANK MASS
DR. MUHAMMAD KAMRAN
DR. FATIMA MAJEED
DR IMRAN HASHIM
PROF MUHAMMAD SALEEM

2. Bio Data
● Fatima
● 2.5years old female
● 12 kg
● Resident of Sialkot
● Admitted via OPD
● 16th March 2022

3. Presenting Complaints
● Abdominal pain –1 month
● Vomiting –2 days

4. HOPI
• Presented in some private hospital in Sialkot
• Vomiting for 2 days Half a cup of gastric contents,
3-4 episodes per day, associated with oral intake, not
relieved with medications
• Abdominal pain for 1month Left hemi abdomen,
dull, moderate, no migration/radiation, partially
relieved by medication, no aggravating factor

5. • Attendants did not notice any mass
• But attending physician noticed a mass in left flank
on examining the child
• At Sialkot initial management done and on workup
pt. diagnosed as a case of left renal mass and
referred to CHL.

6. Systemic Inquiry
No H/O constipation/ diarrhea, hematuria, dysuria,
facial flushing, cough ,fever or lower limb swelling

7. Birth & Family History
• Born of non consanguineous marriage
• S.V.D at term
• 3rd among 4 siblings.
• No other siblings have any significant medical or
surgical history.

8. Developmental History
• As normal child

9. Vaccination History
• Complete according to EPI Schedule

10. EXAMINATION

11. GPE
• Healthy looking playful child with average built sitting
comfortably in mothers lap.
• Weight 12 kg
• Normal looking except for loss of hairs on head
• No Pallor, jaundice, cyanosis, lymphadenopathy
• Vitals
• Pulse: 110/min
• Temp: 98 F
• BP: 95/70 mmHg
• RR: 30 breaths/min

12. Systemic Examination
● Abdominal Examination
Abdomen soft, distended on left side with 7x7 cm firm
to hard mass in left lumbar area, bimanually palpable
and ballotable. No visceromegaly or other mass.

13. • Respiratory System
• B/L Non vesicular breathing, no added sound
• CVS
• No murmur appreciated on auscultation, apex beat normal
• CNS and Motor/Sensory
• Grossly intact, No weakness in any limbs, intact sensation

14. Management at CHL
● Pt was received in Oncology department
● Baselines and Radiological investigations performed
● Biopsy of mass performed
● Chemotherapy commenced as 8 cycles
doxyrubicin, vincristine and dactinomycin

15. Investigations

16. Baselines
● Hb 9.4
● TLC 7.53
● Platelets 322
● RFTs
○ Urea 21
○ Creatinine 0.9
● LFTs
○ Bilirubin 0.65
○ ALT 31
○ ALP 120
● Serum Electrolytes
○ Na 139
○ K 3.4
● PT/APTT 16.9/33.7

17. Ultrasonography
● A solid hyperechoic lesion measuring
12.8x9.8x8.6cm is noted arising from lower pole
of left kidney
● Right kidney and rest of organs appeared normal
with no significant findings

18. Initial CT Scan

22. Post Chemo CT Scan

25. Echocardiography
14/03/2022
● Mass present in IVC hanging into RA and extending
to RV
● Mild obstruction in IVC

26. MDT
● Plan made to involve cardiac surgery and go for left
nephrectomy with extraction of thrombus.
● Cardiac surgery team to perform extraction of intra
cardiac and thoracic part of IVC thrombus
● Peadiatric Surgery to perform Nephrectomy and
thrombolectomy from intra abdominal part of IVC.

27. SURGICAL PLAN
Sternotomy for atrial thrombus removal under Cardiac Bypass
plus Trans-abdominal left sided Radical Nephrectomy, venous
thrombus removal and lymph node biopsy

28. Pre Op Preparation
● Shifted to Surgery ward
● Detail explanation of procedure to parents
● Informed consent obtained
● Optimization of patient for surgery
● ICU bed availability with ventilator
● Fresh labs & arrangement blood and components
● Detailed discussion with cardiac surgery for plan of action

29. Cardiothoracic Surgery
● Median sternotomy
● Cardio pulmonary bypass machine
● Evacuation of thrombus from RA thoracic part of
IVC

30. Cardiac Thrombus

31. ABDOMINAL SURGERY

32. Nephrectomy + Thrombectomy
● Left supra umbilical transverse incision
● A 15*7 cm mass with enlarged precaval, para-
aortic and mesenteric lymph nodes, along
with thrombosed renal vein and thrombus
extending to IVC
● Nephrectomy with lymph node dissection

34. ● Partial control of IVC was taken with Satinsky clamp
to prevent decreased pre load
● Thrombectomy performed
● IVC repaired
● Drains placed in pelvis and renal bed

36. Post vascular anastomosis IVC

37. Post Operative Management
● Pt shifted to cardiac ICU on elective mechanical
ventilation
● Extubated on 2nd post op day
● Started oral liquids on and shifted to SICU on 3rd pod
● Urinary catheter out on 4th pod
● Drains out on 6 th pod and shifted to ward
● Patient discharged after establishing oral diet on 9th pod

38. Post op Echo
● No residual mass in IVC
● No residual mass in RA & RV
● Good biventricular function

39. Histopathology Report
● 13x8.5x7cm mixed type triphasic Wilm’s Tumor.
● RA and IVC Thrombus involved by tumor
● Para-aortic lymphnodes involved
● Mesenteric lymphnode not involved

40. Histopathological Aspect
Dr. Moizza Khalid

41. GROSS EXAMINATION
Case No. 1320-26/2022
1462) Received a left radical nephrectomy measuring 15 x 7 x 6cm.
Ureter measuring 10 x 0.4cm. The specimen was bivalved. There was a
tan white firm tumour measuring 13 x 8.5 x 7cm (80% viable and 20%
necrotic), capsule is 0.1cm away from tumour. Normal kidney
measuring 2 x 0.5cm. Small cysts identified grossly. Renal sinus fat and
hilum was not identified grossly. Representative sections were taken as:
A = Ureter resection margin B = Hilum
C = Perinephric fat D = Hilar blood vessels
E = Tumour with capsule F = Tumour with normal kidney
G = Normal kidney H-U = Sections from tumour

42. 1321) Received two pieces of tissue, larger measuring 2.5 x 2 x 0.2cm
and smaller measuring 1.5 x 1 x 0.5cm, representative sections were
taken in two blocks.
1322) Received a single piece of tissue, measuring 2 x 1 x 0.2cm,
passed entirely in one block.
1323) Received a single piece of tissue, measuring 5 x 2 x 1.5cm,
hemorrhagic areas seen grosly, representative sections were taken in four
blocks.
1324) Received a single piece of tissue, measuring 1.5 x 1 x 0.4cm,
bisected and passed entirely in one block.
1325) Received a single piece of tissue, measuring 1.5 x 1.5 x 1cm,
bisected and passed entirely in one block.
1326) Received a single piece of tissue, measuring 2 x 1 x 0.4cm,
bisected and passed entirely in one block.

43. Triphasic Tumour
Epithelial, Mesenchymal and Blastemal component

44. High Power View
Epithelial component

45. High Power View
Mesenchymal component showing
Rhabdomyomatous Differentition

46. Capsule: Free of Tumor

47. Hilar Vessels: Free of Tumor

48. OPINION
1320) Post Chemotherapy
Residual Wilm’s Tumour
Mixed type (Intermediate Risk)

49. OPINION
• 1321) Thrombus from renal vein, biopsy. Involved by Tumour.
• 1322) Wall of renal vein, biopsy. Free of Tumour.
• 1323) Thrombus from IVC, biopsy. Involved by Tumour.
• 1324) Hilar lymph node, biopsy. Reactive Lymph
Node.
• 1325) Para aortic lymph node, biopsy. Involved by Tumour.
• 1326) Mesenteric lymph node, biopsy. Reactive Lymph
Node.

50. Follow up
● Follow up was done on 15th post op day
● No active issues
● Wounds were healthy, stitches were removed.
● Currently patient is on H/oncology followup

51. Literature Review
Dr Fatima Majeed

52. RENAL TUMORS
●6.3% of childhood cancers
●Include
○Wilms Tumour
○Renal Cell Carcinoma
○Clear Cell Sarcoma of Kidney
○Rhabdoid Tumour of the Kidney
○Congenital Mesoblastic Nephroma
○Renal Cystic Tumour
○Angiomyolipoma

53. WILMS TUMOUR
● Nephroblastoma or Renal Embryoma
● 91% of paediatric renal tumors
● 6% of all paediatric tumors
● Named after Carl Max Wilhelm Wilms, a German
pathologist and Surgeon

54. EPIDEMIOLOGY
●2nd most common malignant abdominal tumor after
neuroblastoma.
●Risk in general population is 1:10,000.
●Mean age at diagnosis is 36 months
●Most children present between 12 to 48 months of
age.
●Unilateral or bilateral

55. ASSOCIATED CONGENITAL
ANOMALIES
1. WAGR Syndrome
2. Beckwith-Wiedemann Syndrome
3. Denys-Drash Syndrome
4. Perlmann Syndrome
5. Hemihypertrophy
6. Urological Anomalies
Hypospadias, Cryptorchidism, Nephromegaly

56. MOLECULAR BIOLOGY &
GENETICS
• LOSS OF HETEROZYGOSITY & DNA PLOIDY
• Chromosomes 11p, 16q, 1p
• TPG3 GENE
• Chromosome 17
• Wild-type gene is involved

57. CLINICAL PRESENTATION
● Asymptomatic Abdominal Mass (most common)
● Abdominal Pain
● Hematuria (gross or microscopic)
● Coagulopathy (10%)
● Hypertension (20%-25%)

58. ● Fever, Anorexia, Weight loss (10%)
● Left varicocele ( due to extension of tumour into renal vein)
● Cardiac malfunction (extension into atrium)
● Acute Abdomen (rare, due to tumour rupture and
haemorrhage)
⮚ Generally, children with WT are healthy toddlers with
palpable abdominal mass

59. DIFFERNTIAL DIAGNOSIS
● Neuroblastoma
● Hepatoblastoma
● Rhabdomyosarcoma
● Lymphoma

60. DIAGNOSIS
● ULTASONOGRAPHY
○ Site of origin and extension
○ Sensitive for intravascular extension
● CT SCAN
○ Confirms renal origin of mass
○ Rules out bilateral Wilms Tumour
○ Confirms presence of metastasis if any

61. ● MRI
○ Avoids radiation exposure
○ Distinguish nephrogenic rests from WT
○ Follow up children with bilateral WT after resection
● Echocardiography

62. SCREENING
● Children at high risk for developing WT (syndromics )
● Scan every 3 to 4 months.
● Confirmation with CT or MRI needed

63. PATHOLOGY
● Embryonal tumours with all components seen in normal
developing kidneys, including:
○ Blastemal
○ Stromal
○ Epithelial tubules
● Class WT is triphasic, containing all
● Biphasic and Monophasic lesions also occur.

64. ● Monophasic can be very invasive and difficult to
distinguish
● Histologically 2 groups:
○ Favourable Histology almost 90%
○ Unfavourable Histology anaplastic, clear cell sarcoma of
kidney and rhabdoid tumours

65. Favorable Histology
• Triphasic pattern of
blastema, epithelial and
stromal tissue
• Small uniform nuclei
• Good response to treatment
Unfavorable Histology
• Higher degree of anaplasia
• Hyperchromatic, pleomorphic
nuclei larger than 3 times of
adjacent cells
• Abnormal mitotic figures
• Poor response to treatment

66. PRETREATED TUMOURS AND
PATHOLOGY
● Pre-treated tumours differ in histology from non
treated tumours.
● Chemotherapy may either produce necrosis of
tumour or differentiation of tumour.
● Without neoadjuvant chemotherapy:
○ Triphasic mixed histology (45%)
○ Blastemal (39%)
○ Epithelial dominant (15%)

67. ● With neoadjuvant chemotherapy:
○ Regressive (38%)
○ Mixed (29%)
○ Stromal (14%)
○ Blastemal (6%)
○ Epithelial predominant (3.1%)
○ Necrotic (6.6%)

68. STAGING
● Local Staging: refers to abdominal disease.
● Disease Stage: considers both local and distant
hematogenous metastatic disease.
● Described by:
○ COG (children oncology group)
○ SIOP (Societe Internationale D'oncologie Pediatrique)

71. TREATMENT
● Based on studies by COG and SIOP.
● According to COG protocols:
○ Nephrectomy
○ Chemotherapy
○ Radiotherapy

72. ● According to SIOP protocols:
○ Neo-adjuvant chemotherapy
○ Nephrectomy
○ Chemotherapy
○ Radiotherapy

76. SURGERY
● Mainstay of either treatment regimen.
● Fundamental Tasks include:
i. Safe resection of tumour
ii. Accurate staging of tumour
iii. Avoidance of complications that will upstage tumour
iv. Correct documentation of intraoperative findings
and details of procedure

77. ● Factors that negatively affect patient survival include:
i. Tumour spills
ii. Failure to biopsy lymph nodes
iii. Complete tumour removal
iv. Failure to assess for extra-renal tumour extension
v. Surgical complications

78. TECHNICAL CONCERNS IN
UNILATERAL TUMOURS
● Wide abdominal exposure using transverse
transabdominal or thoracoabdominal incision
● Complete removal of Gerota fat and fascia.
● Complete mobilization of tumour.
● Ligation of renal artery first when ligating renal
pedicle.
● Renal vein should be palpated first for any
intravascular extension and then ligated.

79. ● Adrenalectomy if mass arises in upper pole of kidney.
● Ligation and division of ureters as low as possible.
● Sampling of lymph nodes: hilar, pericaval and para-aortic
nodes.
● In case of invasion into surrounding structures, en bloc
resection should be avoided
● Liver metastasis should be treated with adjuvant
chemotherapy and resection should be avoided.

80. UPSTAGING OF TUMOUR
● SPILL: refers to break in tumour capsule;
transaction of ureter or renal vein while these
structures are containing tumour.
● RUPTURE: traumatic or spontaneous rupture of
tumour pre-operatively; incisional biopsy; tumour
invading capsule with open neoplastic tissue surface
being in contact with peritoneal cavity.
⮚All these events makes child stage 3; so such
events must be documented carefully.

81. MANAGEMNT OF
EXTENSION IN RENAL
VEIN, IVC & ATRIUM
● Detected pre-operatively
by USG/CT/MRI
● Neo-adjuvant
chemotherapy if tumour
thrombus extends into
IVC at the level of liver
or higher.

82. ● Palpation of renal vein before tumour mobilization
of kidney.
● If tumour extends into renal vein or IVC below level
of liver it should be removed en bloc with kidney by
taking control of renal vessels above and below the
kidney.
• If tumour thrombus still persists above level of liver
it should be removed by putting patient on
cardiopulmonary bypass.

83. Daum’s Classification

84. Drawbacks of IVC & Cardiac
Extension
• Extended course has no additional benefits
• Sugical morbidity
• Prlonged surgery time
• Increased blood loss
• Prolonged hospital stay
• Complications

85. CHEMOTHERAPY
● Dactinomycin remains the mainstay of treatment
regimen today.
● FAVORABLE HISTOLOGY TUMOURS:
○ WITHOUT LOH:
○ Stage I&II: 18 weeks of vincristine &
dactinomycin; OS 98.4% and 98.7%
○ Stage III&IV: 24 weeks of vincristine,
dactinomycin and doxorubicin

86. ● Bone marrow transplant has been performed with
EFS rates of 36% to 60% in these small series.

87. Take Home Message
● The surgical resection of Wilms tumor with
intravascular extension remains a formidable
challenge.
● This complicated task is achieved by
multidisciplinary approach

88. THANK
YOU