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8/8/2019 Wilms Tumor 12.13.2010
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The painless abdominal massMorning report
December 2010
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Differential diagnosis Renal tumors:
(differentiated by
histology)
Wilms is the most common Clear cell sarcoma of the kidney
Rhabdoid tumor of the kidney
Congenital mesoblastic nephroma
Renal cell carcinoma
Renal medulary carcinoma
Neuroblastoma
Uncommon causes:
Hepatomegaly
Splenomegally
Bladder distenstion
Constipation
Intussuception
Lymphoma
Other tumors
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Pathogenesis
Has been associated with the loss of several tumor different suppressor genes(WT1, p53, FWT1, FWT2)
This leads to abnormal proliferation of persistent metanephric cells callednephrogenic rests
These cells are still present in about 1% of newborn kidneys and then regress In Wilms, they are present in about 35% of unilateral disease and 100% of
bilateral disease
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Incidence Wilms is the most common renal malignancy
in children
4th most common childhood cancer(7% of childhood malignancies)
Annual incidence: 8 per million
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Presentation Mean age of diagnosis for an isolated
Wilms tumor is about 3 yrs (earlier if there
are other associated features)
Classically, it is a solitary growth in any
part of either kidney
There is often compression of the normal
kidney
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Presentation Most commonly, isolated Wilms tumors
present with a painless abdominal mass or
abdominal swelling. Many are found byroutine abdominal exam
Abdominal pain 30%
Hematuria 12-25% Hypertension 25% (due to renal ischemia
from impingement on the renal artery)
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Associated syndromes WAGR syndrome:
Wilms tumor
Aniridia
GU anomalies
Renal impairment
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Associated syndromes
Denys-Drash syndrome:
Wilms tumor Progressive renal disease
Male pseudohermaphrodism
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Associated syndromes Beckwith-wiedeman syndrome:
5-10% will develop a Wilms tumor
Macrosomia
Macroglossia
Hemihypertrophy
Omphalocele
Prominent eyes
Ear creases
Large kidneys
Pancreatic hypertrophy
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Other associations Perlman syndrome
Sotos syndrome
Simpson-Golabi-Behmel syndrome
Isolated hemihypertrophy
Isolated GU anomalies
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Work up-Labs CBC looking for abnormal cell lines and
evidence of anemia
CMP looking at renal function and screeningfor liver involvement (mets)
Serum Ca can be elevated in other forms ofrenal masses
Coags: acquired VonWillebrands can occurin 8%
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Imaging US initially
CT abdomen to look for extent of disease
CT chest and pelvis for metastatic disease
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Prognosis at 2 years from diagnosis Favorable histology: >90% survival
regardless of stage
Tumor with anaplastic histology beyond stage1:
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Take home point Abdominal exams are important