Nephroblastoma
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Case
A 3 yr old female presents with
Abdominal distension and discomfort mass urine
Abdominal red
Examination reveal a palpable mass in the Rt flank extending to umbilical region and elevated blood pressure
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Introduction
Wilms'
tumor (WT) or nephroblastoma,
is an embryonal tumor of renal origin.
Wilms
tumor, is the most common
childhood abdominal malignancy.
Approximately
500 cases of WT are
diagnosed in the USA each yr.
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Epidemiology
It
arises in approximately 10 children per
million under age 15 years and is usually diagnosed between ages 2 and 5 years.
Bodkyn
et al in 2010 found and incidence
of Wilms tumour : 2.7 per 100,000 pyrs for females in Trinidad and Tobago which was also less in males.
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Pathophysiology
Wilms'
tumor (WT) is attributed to an altered residual WT1 gene that is required for the maturation of blastemal and epithelial cells but not for the maturation of stromal and heterologous elements
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Pathophysiology
Associated with 3 groups of congenital malformations
WAGR syndrome characterized by aniridia, genital anomalies and mental retardation
WT1 suppressor gene and 11 p13 deletion
Denys-Drash
WT1
syndrome- characterised by male puedohermaphroditism , progressive nephropathy
suppressor gene and 11 p13 mutation
Beckwith-Wiedemann
syndrome -an overgrowth 5/28/12 syndrome characterized by visceromegaly,
Morphology
Grosslarge,
solitary, wellcircumscribed mass On cut section, the tumor is soft, homogeneous, and tan to gray with occasional foci of hemorrhage, cyst formation, and necrosis
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Histology
Favorable
-The classic triphasic combination of :
Blastemal cell types-small blue cells, with little distinctive features cells type- fibrocytic or myxoid in nature, skeletal muscle differentiation is not uncommon cell types-abortive tubules or glomeruli.
Stromal
Epithelial
Unfavoarable - Anaplasia ( 5%) - cells with large, hyperchromatic, pleomorphic
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Presentation
Most
common presentation 60 % :
abdominal mass distension without pain
Palpable With/
Abdominal
Hematuria
( 15%) ( 25%)
Hypertension Weight Fever
loss
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Presentation
In
association with other syndromes
WT with anirdia , genitourinary
WAGR
anomalies mental retardation
Beckwith-
Wiedemann syndrome WT present
later into adult hood
Denys
Drashy WT with intersex disorder,
and progressive nephropathy
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Presentation
Examination
A
palpable abdominal mass
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Investigations
The
following lab investigations are used
CBC
count
Chemistry Urinalysis
profile- Renal function Test and electrolytes and calcium studies
Coagulation Cytogenetic
studies for WT1 gene and concomitant chromosomal abnormalities 11p 15, 11p 13 5/28/12
Imaging Studies
Renal
If
ultrasonography detects :
mass originated from kidney is cystic or solid
Presence
of potential tumor thrombus within the renal vein and inferior vena cava
Ultrasound demonstrates a large heterogenous mass ( Wilms' tumour) arising from the left kidney containing multiple areas of haemorrhage or necrosis.
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Imaging Studies
CT
scanning of abdomen
origin
of the tumor, involvement of the lymph nodes, bilateral kidney involvement, invasion into major vessels (eg, inferior vena cava), and liver metastases.
CT scan of a Wilms' tumor involving the right kidney. Arrows show
CT showing bilateral Wilms' tumors
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Imaging Studies
MRI
scanning of abdomen
Caval
patency the IVC is directly invaded by tumor and tumor thrombus in IVC . to liver or lung
Metastasis
Both
CT and MRI are important in differentiating Wilms tumor from neuroblastoma radiography Images may depict lung 5/28/12 metastases.
Chest
Differentials
Neuroblastoma Polycystic
Kidney Disease
Rhabdomysarcoma
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Management
Treatment is determined via Stage of tumour is done via
imaging studies
This
Surgical exploration and
Preoperative Histology
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Staging- National Wilms Tumour Study
Stage I Encapsulated tumor, completely excised Stage 2 Extends beyond the kidney, completely excised Stage 3 Residual tumor confined to the abdomen or nodes Stage 4 Haematogenous metastases (lung metastases on CXR or on CT Stage 5 Bilateral tumours at diagnosis
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Treatment
In Stage 1 tumors - nephrectomy and adjuvant chemotherapy for 3 months. In Stage 2 tumors, nephrectomy and twoagent chemotherapy for 1215 months Stage 3 tumors, nephrectomy with a three-agent chemotherapy for 1215 months, with radiotherapy as an occasional adjunct.
If the tumor is large, chemotherapy may be used to shrink the tumor before surgical removal.
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Treatment
In
Stage 4 tumors, nephrectomy with
chemotherapy is the mainstay of treatment.
Stage
5 not well established but
preservation of renal parenchyma following initial preoperative chemotherapy.
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Surgery
Exploratory
laparotomy
asses
to confirm diagnosis the opposite kidney resect the primary tumour, ureter and adjacent lymph nodes
Completely
Partial
nephrectomy is perform if tumour is bilateral for sparing
Intra operative picture of large Wilms' tumor within the left kidney outlined by arrows
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Prognosis (U.S.A.)
Stage Overall survival Favourable (4 yr ) histology Unfavourable histology 98 % 95% 96% 70% 95% 56% 90% 14%
I II III IV
Patient
with stage V disease have 70-80% survival rate if synchronous and 45-50% if metachronous .
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References
www.emedicine.com Sabiston
Paediatric Wilms tumor
Textbook of Surgery, 18th ed. Chapter 71 Pathology Museum Case studies Wilms tumor et al 2010 Common childhood caners in Trinidad and Tobago.WIMJ. Vol 65
Anatomical Bodykyn
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Thank you
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