8/9/2019 Wilms Tumor 5.5.2010

1/9

Wilms TumorWilms Tumor

Megan Linn

Morning ReportCinco de Mayo, 2010

Megan Linn

Morning ReportCinco de Mayo, 2010

8/9/2019 Wilms Tumor 5.5.2010

2/9

EpidemiologyEpidemiology

EFourth most common childhoodcancer

E7% of all childhood malignancies

E2/3 < 5 yo

E95% < 10 yo

E8 cases per million < 15yo

EBlacks > Whites> Asians

EFourth most common childhoodcancer

E7% of all childhood malignancies

E2/3 < 5 yo

E95% < 10 yo

E8 cases per million < 15yo

EBlacks > Whites> Asians

8/9/2019 Wilms Tumor 5.5.2010

3/9

PresentationPresentation

E**Abdominal mass only**

EAbdominal pain (30%)

EHematuria (12-25%)

EHypertension (25%)

E**Abdominal mass only**

EAbdominal pain (30%)

EHematuria (12-25%)

E

Hypertension (25%)

8/9/2019 Wilms Tumor 5.5.2010

4/9

PathologyPathology

EUsually a solitary lesion

E7% bilateral

E12% multi-focal within a kidney

EUsually a solitary lesion

E7% bilateral

E12% multi-focal within a kidney

8/9/2019 Wilms Tumor 5.5.2010

5/9

Associated SyndromesAssociated Syndromes

EWAGR syndrome

EDenys-Drash syndrome

EBeckwith-Wiedemann syndrome

EPerlman syndrome

ESotos syndrome

ESimpson-Golabi-Behmel syndrome

EWAGR syndrome

EDenys-Drash syndrome

EBeckwith-Wiedemann syndrome

EPerlman syndrome

E

Sotos syndromeESimpson-Golabi-Behmel syndrome

8/9/2019 Wilms Tumor 5.5.2010

6/9

WAGR SyndromeWAGR Syndrome

EWilms tumor

EAniridia

EGU anomaliesEIntellectual disability

EWT1 deletion (11p13)EInvolved in renal and gonadal

development

EWilms tumor

EAniridia

EGU anomaliesEIntellectual disability

EWT1 deletion (11p13)EInvolved in renal and gonadal

development

8/9/2019 Wilms Tumor 5.5.2010

7/9

Denys-Drash SyndromeDenys-Drash Syndrome

EProgressive renal disease

EDiffuse mesangial sclerosis

EMale pseudohermaphrodism

EWilms tumor

EPoint mutation on WT1

EProgressive renal disease

EDiffuse mesangial sclerosis

EMale pseudohermaphrodism

EWilms tumor

EPoint mutation on WT1

8/9/2019 Wilms Tumor 5.5.2010

8/9

Beckwith-WiedemannBeckwith-Wiedemann

EMacrosomia

EMacroglossia

EOmphalocele

EProminent eyes

E

Large kidneysEPancreatic hyperplasia

EHemihypertrophy

EMacrosomia

EMacroglossia

EOmphalocele

EProminent eyes

E

Large kidneysEPancreatic hyperplasia

EHemihypertrophy

8/9/2019 Wilms Tumor 5.5.2010

9/9

Cinco de MayoCinco de Mayo

EMay 5, 1862: Mexican army victory againstFrench at the Battle of PueblaE France invaded b/c Mexico stopped paying interest on

debts to other countries

EMexico army greatly out-numbered by and less-equipped than the French

E Celebrated primarily in state of Puebla and the

U.S.

EMexicos Independence Day is actually Sept 16

EMay 5, 1862: Mexican army victory againstFrench at the Battle of PueblaE France invaded b/c Mexico stopped paying interest on

debts to other countries

EMexico army greatly out-numbered by and less-equipped than the French

E Celebrated primarily in state of Puebla and the

U.S.

EMexicos Independence Day is actually Sept 16