wilms tumor has 3 types of tissues unlike amny tumors which have 2 types

Name General Origin N.E. Microscopic Clinical

most common primary renal tumor of childhood. Most children with Wilms tumors present
age : between 2 and 5 years and 95% of tumors Wilms tumors are characterized by with a large abdominal mass that may be
occur before 10 years. recognizable attempts to recapitulate unilateral or, when very large, extend
Pathogenesis and Genetics: different stages of nephrogenesis. The across the midline and down into the
• Three groups of congenital malformations are classic triphasic combination of pelvis • Hematuria, pain in the abdomen
associated with an increased risk for Wilms tumor (in blastemal, stromal, and epithelial cell after a traumatic incident, intestinal
10%): 5% to 10% of Wilms types is observed . obstruction, and hypertension are other
• 1-WAGR syndrome (i.e., Wilms tumor, aniridia, tumors involve both • blastemal component Sheets of small patterns of presentation • increased risk
genital abnormalities, and mental retardation); it is kidneys, or multicentric blue cells with few distinctive features . of developing second primary tumors,
due to WT1 gene deletions. present as a large, • Epithelial differentiation: is usually in including bone and soft tissue sarcomas,
• 2- Denys-Drash syndrome, which is characterized solitary, well the form of abortive tubules or glomeruli leukemia and lymphomas, and breast
Wilms Tumor by gonadal dysgenesis (male circumscribed mass. • Stromal cells :are usually fibroblastic or cancers
Nephrogenic
(nephroblast pseudohermaphroditism) and early onset On cut section, the myxoid in nature, although skeletal
rests.
oma) nephropathy leading to renal failure, due to bi-allelic tumor is soft, muscle differentiation is not uncommon.
inactivation of WT1 homogeneous, and tan Rarely, other heterologous elements are
• 3- Beckwith-Wiedemann syndrome (BWS), to gray with occasional identified, including squamous or
characterized by enlargement of body organs foci of hemorrhage, mucinous epithelium, smooth muscle,
(organomegaly), macroglossia, hemihypertrophy, cyst formation, and adipose tissue, cartilage, and osteoid
omphalocele, and abnormal large cells in the adrenal necrosi and neurogenic tissue.
cortex (adrenal cytomegaly), due to defect in the • 5% of tumors have anaplasia, defined
chromosomal region localized to band 11p15.5. as the presence of cells with large,
• in sporadic (i.e., nonsyndromic) tumors, which hyperchromatic, pleomorphic nuclei and
account for 90% of cases, mutations occur in genes abnormal mitoses
encoding
proteins involved in micro-RNA (miRNA)
5% to 10% of primary renal tumors originate from the usually become clinically apparent within
Urethial urothelium of the renal pelvis. These tumors range a relatively short time, because they lie
carcinoma of from apparently benign papillomas to invasive On histologic examination, as urinary
within the pelvis and, by fragmentation,
urothelial (transitional cell) carcinomas. produce noticeable hematuria. These
the renal bladder
tumors may block urinary outflow and
pelvis lead to palpable hydronephrosis and
flank pain.