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Fulltext Extrarenal Wilms' tumour

Looi LM, Jagadeesan K

Med J Malaysia, 1983 Jun;38(2):134-6.
PMID: 6312279

A rare case of extrarenal Wilms' tumour in a Kadazan child is presented with a description of its clinical and pathological features. The possible origin of this tumour is discussed

Matched MeSH terms: Wilms Tumor/pathology*
Extrarenal Wilms Tumor With Skeletal and Glial Differentiation

Tan GC, Prasad V

Int. J. Surg. Pathol., 2018 Feb;26(1):34.
PMID: 28508688 DOI: 10.1177/1066896917709946
Matched MeSH terms: Wilms Tumor/pathology*
Wilms' tumour in Malaysian children: a histopathological study of cases encountered at the University Hospital, Kuala Lumpur over a 22-year period

Cheah PL, Looi LM, Lin HP

Malays J Pathol, 1992 Dec;14(2):111-5.
PMID: 1338998

Formerly thought to have a constant incidence rate throughout the world, Wilms' tumour (nephroblastoma) has been shown to be less common among Asian children. A retrospective demographic and morphological study of Wilms' tumour histologically diagnosed over a 22-year period at the Department of Pathology, University Hospital, Kuala Lumpur was conducted to assess for inherent demographic and morphological differences between tumours in Malaysian children and those of Western populations. Thirty-seven cases of histologically proven Wilms' tumour qualified for inclusion in this study. 19 patients were Chinese, 13 Malay, 4 Indian and 1 Anglo-asian. 21 were male and 16 were female (M:F ratio = 1.3:1). Their ages ranged from 1 month to 4 years. 70.3% of the patients were below 2 years of age. 36 cases had unilateral and 1 bilateral tumours. Of unilateral tumours, 19 involved the left kidney and 17 the right. Histological assessment, based on criteria of the National Wilms' Tumor Study Group, revealed 20 (52.6%) tumours with a mixed pattern while 8 (21.1%) showed epithelial, 7 (18.4%) blastemal and 3 (7.8%) stromal-predominant patterns. Anaplasia was observed in only 2 tumours (5.3%). There was no obvious difference in age range and sex distribution, laterality of tumours and incidence of anaplasia between this and Western studies. No ethnic predilection was observed. A notably larger percentage of cases were below 2 years of age. Also, a larger proportion of epithelial-predominant and a lower proportion of blastemal-predominant tumours was observed compared with patterns reported from Western populations.

Matched MeSH terms: Wilms Tumor/pathology*
Clear cell sarcoma of kidney: a clinicopathological study of eight cases from Malaysia

Cheah PL, Looi LM, Lin HP

Histopathology, 1992 Oct;21(4):365-9.
PMID: 1328018

Eight cases of clear cell sarcoma of kidney were seen in the Department of Pathology, University Hospital, Kuala Lumpur, Malaysia over the 16-year period from 1973 to 1989. Five of the patients were males. Six patients were Malay, one Chinese and one Indian. The patients' ages ranged from 8 months to 3 years. Clear cell sarcoma was the original diagnosis in two patients while six were diagnosed as blastemal-predominant Wilms' tumours at presentation. Metastases developed in five patients. Metastatic sites included the thoracic vertebra, skull, orbit, humerus, radius, ulna, shoulder, lung and liver. The prolonged survival, of 9 years and 9 months, seen in one patient despite omission of Adriamycin (doxorubicin) from the chemotherapeutic protocol is highlighted. We also emphasise the histological factors which are of help in differentiating clear cell sarcoma from Wilms' tumour.

Matched MeSH terms: Wilms Tumor/pathology*
p53, stem cell biology and childhood blastomas

Oh L, Hafsi H, Hainaut P, Ariffin H

Curr Opin Oncol, 2019 03;31(2):84-91.
PMID: 30585860 DOI: 10.1097/CCO.0000000000000504

PURPOSE OF REVIEW: Childhood blastomas, unlike adult cancers, originate from developing organs in which molecular and cellular features exhibit differentiation arrest and embryonic characteristics. Conventional cancer therapies, which rely on the generalized cytotoxic effect on rapidly dividing cells, may damage delicate organs in young children, leading to multiple late effects. Deep understanding of the biology of embryonal cancers is crucial in reshaping the cancer treatment paradigm for children.
RECENT FINDINGS: p53 plays a major physiological role in embryonic development, by controlling cell proliferation, differentiation and responses to cellular stress. Tumor suppressor function of p53 is commonly lost in adult cancers through genetic alterations. However, both somatic and germline p53 mutations are rare in childhood blastomas, suggesting that in these cancers, p53 may be inactivated through other mechanisms than mutation. In this review, we summarize current knowledge about p53 pathway inactivation in childhood blastomas (specifically neuroblastoma, retinoblastoma and Wilms' tumor) through various upstream mechanisms. Laboratory evidence and clinical trials of targeted therapies specific to exploiting p53 upstream regulators are discussed.
SUMMARY: Despite the low rate of inherent TP53 mutations, p53 pathway inactivation is a common denominator in childhood blastomas. Exploiting p53 and its regulators is likely to translate into more effective targeted therapies with minimal late effects for children. (see Video Abstract, Supplemental Digital Content 1, http://links.lww.com/COON/A23).

Matched MeSH terms: Wilms Tumor/pathology
An immunohistochemical study comparing clear cell sarcoma of the kidney and Wilms' tumor

Looi LM, Cheah PL

Pathology, 1993 Apr;25(2):106-9.
PMID: 8396229

This study explores immunohistochemical characteristics that may be of diagnostic value in differentiating clear cell sarcoma of the kidney (CCSK) from Wilms' tumor (WT) and may provide some insight into the histogenesis of CCSK. Formalin-fixed, paraffin-embedded sections of 8 CCSK and 9 WT were stained, using the standard avidin-biotin peroxidase complex method, for vimentin (VIM), Factor-8 related antigen (F8A), epithelial membrane antigen (EMA), desmin (DES), S-100 protein and Mac 387. CCSK cells consistently exhibited moderate to strong diffuse cytoplasmic positivity for VIM and were negative for F8A, EMA, DES, S-100 and Mac 387. In contrast, only patchy groups of stromal cells and primitive glomeruloid structures in WT exhibited VIM-positivity. Blastemal cells were VIM-negative. Stromal cells with rhabdomyomatous differentiation exhibited cytoplasmic positivity for DES. Epithelial cells of maturing tubular structures showed EMA-positivity whereas immature tubular structures were EMA-negative. Neither blastemal, stromal nor epithelial elements in WT were positive for F8A, S-100 or Mac 387. Podocytes and mesangial cells of glomeruli in 3 mid-trimester human abortuses (controls) exhibited moderate to strong VIM-positivity. The importance of differentiating CCSK from WT has been repeatedly emphasized because of its poorer prognosis and the necessity of adding Adriamycin to the chemotherapeutic regime. The consistent VIM-positivity of CCSK cells can be a useful feature in differentiating it from "blastemal-predominant" WT, with which it is often confused. Although vimentin expression by CCSK cells is consistent with a mesenchymal character, the possibility of a histogenetic link with glomerular podocytes or mesangial cells should also be considered.

Matched MeSH terms: Wilms Tumor/pathology*
Immunohistochemical expression of p53 proteins in Wilms' tumour: a possible association with the histological prognostic parameter of anaplasia

Cheah PL, Looi LM, Chan LL

Histopathology, 1996 Jan;28(1):49-54.
PMID: 8838120

Wilms' tumour (nephroblastoma) has been associated with chromosomal abnormalities at the 11p13, 11p15 and 16q regions. A study into the possibility of mutations occurring within p53, the ubiquitous adult tumour suppressor gene, in Wilms' tumour was carried out. Thirty-eight cases were studied. Of these 36 were categorised into the favourable histology group and two into the unfavourable histology group based on the National Wilms' Tumour Study criteria. Archival formalin-fixed, paraffin-embedded tissue sections from each case were stained with a polyclonal (AB565:Chemicon) and a monoclonal (DO7:Dako) antibody raised against p53 protein using a peroxidase-labelled streptavidin biotin kit (Dako). 'Cure' (disease-free survival of 60 months or longer) was documented in 39% of cases with favourable histology tumours. Eleven percent in this group succumbed to the disease. Both cases with unfavourable histology died. Four out of 36 (11%) tumours with favourable histology demonstrated weak to moderate staining with both AB565 and DO7 in more than 75% of tumour cells. In contrast, p53 protein expression in unfavourable histology tumours was significantly increased compared with the favourable histology group (P = 0.021) with both cases demonstrating immunopositivity in > 75% of tumour cells when stained with AB565 and DO7. The intensity of staining ranged from moderate to strong in both cases. It appears from this preliminary study that the immunohistochemical expression of p53 protein in Wilms' tumour, presumably a result of mutation in the p53 tumour suppressor gene, correlates with histological classification, histological categorisation being one of the useful features in the prognostic assessment of Wilms' tumours.

Matched MeSH terms: Wilms Tumor/pathology*
Implications of p53 protein expression in clear cell sarcoma of the kidney

Cheah PL, Looi LM

Pathology, 1996 Aug;28(3):229-31.
PMID: 8912350

Eight histologically-confirmed cases of clear cell sarcoma of the kidney (CCSK) were studied for possible mutations in the p53 tumor suppressor gene by the immunohistochemical demonstration of mutant p53 proteins using a monoclonal (DO7: Dako) and a polyclonal (AB565: Chemicon) antibody to p53 protein. All cases exhibited p53 protein nuclear immunopositivity, although in varying numbers of tumor cells and with different staining intensities. p53 protein (DO7 or AB565) was expressed in < 25% of the tumor cells in four (50%) of the cases, including the one case with a known long term survival of 13 years from the time of diagnosis. The other tumors showed p53 protein immunopositivity in > 25% of the tumor cells when stained with either DO7 or AB565 or both. The intensity of staining, graded on visual impression into weak, moderate or strong, did not correlate well with the ratio of positive staining tumor cells. While this study is unable to clarify the relative prevalence and importance of p53 mutational events in the pathogenesis of this aggressive renal tumor of childhood, it is reasonably suggestive that alterations in the p53 tumor suppressor gene do occur in CCSK.

Matched MeSH terms: Wilms Tumor/pathology