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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