Wilms Tumor

Wilms tumor (WT), also known as nephroblastoma, is the most common primary malignant
renal tumor of childhood; other renal tumors are very rare. It is the second most common
malignant abdominal tumor in childhood. The most common sites of metastases are the lungs,
regional lymph nodes, and liver. Histologically, the classic WT is made up of varying proportions
of blastemal, stromal, and epithelial cells, recapitulating stages of normal renal development. The
treatment includes surgery and chemotherapy with or without radiotherapy. The use of
multimodality treatment and multiinstitutional cooperative group trials has dramatically
improved the cure rate of WT from <30% to approximately 90%.

Epidemiology

WT accounts for 6% of pediatric malignancies and more than 95% of kidney tumors in children.
In the United States, the incidence of WT is approximately 8 cases per 1 million children
younger than 15 yr of age per year, and about 650 new cases are diagnosed each year.
Approximately 75% of the cases occur in children younger than 5 year with a peak incidence at
2-3 year of age. It can arise in 1 or both kidneys; the incidence of bilateral WTs is 7%. Most
cases are sporadic, but approximately 2% of patients have a family history. In 8-10% of patients,
WT is observed in the context of hemihypertrophy, aniridia, genitourinary anomalies, and a
variety of rare syndromes, including Beckwith-Wiedemann syndrome and Denys-Drash
syndrome (Table 499-2). An earlier age of diagnosis and an increased incidence of bilateral
disease are generally observed in syndromic and familial cases.

Tumor wilms terhitung sekitar 6% dari seluruh penyakit keganasan pada anak dan lebih dari 95%
tumor ginjal pada anak. Di Amerika Serikat, kejadian WT kira-kira 8 kasus per 1 juta anak-anak
yang lebih muda dari 15 tahun usia per tahun, dan sekitar 650 kasus baru didiagnosis setiap
tahun. Sekitar 75% dari kasus terjadi pada anak-anak yang lebih muda dari 5 tahun dengan
insidensi puncak pada usia 2-3 tahun. Dapat muncul di 1 atau kedua ginjal; itu insidensi WT
bilateral adalah 7%. Sebagian besar kasus bersifat sporadis, tetapi kurang-lebih 2% pasien
memiliki riwayat keluarga. Pada 8-10% pasien, WT diamati dalam konteks hemihipertrofi,
aniridia, genitourinari anomali, dan berbagai sindrom langka, termasuk Beckwith-Wiedemann
syndrome dan sindrom Denys-Drash (Tabel 499-2). Usia diagnosis yang lebih dini dan
peningkatan insiden bilateral Penyakit umumnya diamati pada kasus sindromik dan keluarga.