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Pathology Station-Wlims Tumor
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Dr. Hamed Dadash Karimi
Urologist
Date:
4/28/2020
Views:
65
Catgory:
Describtion
Nephroblastoma -Wilms' Tumor
Constitute ~85% of childhood renal cancers.
Age: 2-4 years; extremely uncommon in children <6 months and >6 years.
Associations: WAGR syndrome, Beckwith-Wiedemann syndrome, and Denys-Drash syndrome.
Mostly presents as abdominal mass. (Detected by parents).
Bilateral in 5% of cases.
Radiology: calcification is uncommon (vs. neuroblastoma, which is often calcified).
Gross:
Usually large with solid soft gray tan cut surface and seemingly well-circumscribed borders
(image A)
.
Hemorrhage and necrosis are common
Microscopic: 3 distinct elements:
Blastema:
sheets of densely packed primitive small cells with scant cytoplasm and darkly staining nuclei ("small round blue cell tumor")
(image B)
&
(image C)
.
Epithelial:
small tubules or cysts lined by primitive or mature columnar or cuboidal cells.
Stroma:
spindle cells or may differentiate along the lines of any soft tissue such as fibroblasts, smooth muscles or skeletal muscles, cartilage
(image D)
; loose myxoid and fibroblastic spindle cell stromas are the most common.
Most tumors though have biphasic or monophasic growth.
Immunohistochemistry: WT1+ in blastemal and epithelial components.
Overall survival is good (>90%).
Unfavorable prognostic factors are high stage and presence of diffuse anaplasia.
DDX:
Other small round blue cell tumors including neuroblastoma (WT1-)
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