Wilms' tumour in infancy

Abstract

During a 15-y period, 48 children were treated for Wilms' tumour (WT). Seven of them were < 1 y of age at diagnosis. One child presented with non-traumatic haematuria, but in all the other children WT was revealed as a palpable abdominal mass at routine examination or investigation due to another disease. The four children under 6 months of age at diagnosis were primarily operated upon; the others received preoperative chemotherapy. Two children had chromosomal aberrations in the WT tumour specimen. The follow-up revealed that postoperatively six children are healthy with no evidence of relapse from WT, but one child had a contralateral relapse successfully enucleated. The clinical behaviour and management of WT in infants differ compared with that in older children. The diagnosis may be uncertain and it can be difficult to distinguish between malignant and non-malignant lesions. It is essential to realize the possibility of WT, even in children < 1 y of age.

Characteristics and survival of 750 children diagnosed with a renal tumor in the first seven months of life: A collaborative study by the SIOP/GPOH/SFOP, NWTSG, and UKCCSG Wilms tumor study groups

“…Metastatic disease at diagnosis was rare in WT and CCSK, whereas most MRTK cases were stage III and IV. This predominance of low stage WT in this young age group confirms earlier studies 1, 4, 6, 8, 12. Moreover, it underlines the aggressive biological behavior of MRTK and indicates that if a patient younger than 213 days presents with a metastatic renal tumor, this is unlikely to be WT, and MRTK should be seriously considered.…”

“…However, the relative proportion of Wilms tumor varies at the extremes of childhood. In particular, benign tumors and the rare malignant rhabdoid tumor of kidney (MRTK) are relatively common in very young infants in the first 7 months of life [1][2][3][4][5][6][7][8][9]. This has led to the recommendation that all such children should be treated with primary nephrectomy to establish a histological diagnosis prior to initiating chemotherapy where necessary.…”

A Rare Occurrence of Neonatal Nephroblastoma in Sub-Saharan Africa: A Case Report and Management in a Resource-Constrained Region

“…Approximately twenty cases in pediatric population could be found with the use of available computer-assisted medical literature search programs. 3 , 5 , 6 , 10-12 Clinical and radiological features of our patient were similar of those described in the literature. 4 , 8 Histopathological analysis was compatible with a nephroblastoma blastemal type as described in the literature.…”

Reliability of Her2/neu, estrogen receptor, and progesterone receptor testing by immunohistochemistry on cell block of FNA and serous effusions from patients with primary and metastatic breast carcinoma

“…However, the relative proportion of Wilms tumor varies at the extremes of childhood. In particular, benign tumors and the rare malignant rhabdoid tumor of kidney (MRTK) are relatively common in very young infants in the first 7 months of life 1–9. This has led to the recommendation that all such children should be treated with primary nephrectomy to establish a histological diagnosis prior to initiating chemotherapy where necessary.…”