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Morphological and Immunohistochemical Characteristics of Surgically Removed Paediatric Renal Tumours in Latvia (1997–2010)

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Morphological and Immunohistochemical Characteristics of Surgically Removed Paediatric Renal Tumours in Latvia (1997–2010) DOI: 10.2478/v10163-012-0008-6 ACTA CHIRURGICA LATVIENSIS • 2011 (11) ORIGINAL ARTICLE Morphological and Immunohistochemical Characteristics of Surgically Removed Paediatric Renal Tumours in Latvia (1997–2010) Ivanda Franckeviča*,**, Regīna Kleina*, Ivars Melderis** *Riga Stradins University, Riga, Latvia **Children’s Clinical University Hospital, Riga, Latvia Summary Introduction. Paediatric renal tumours represent 7% of all childhood malignancies. The variable appearances of the tumours and their rarity make them especially challenging group of lesions for the paediatric pathologist. In Latvia diagnostics and treatment of childhood malignancies is concentrated in Children’s Clinical University Hospital. Microscopic evaluation of them is realised in Pathology office of this hospital. Aim of the study is to analyze morphologic spectrum of children kidney tumours in Latvia and to characterise them from modern positions with wide range of immunohistochemical markers using morphological material of Pathology bureau of Children’s Clinical University Hospital. Materials and methods. We have analyzed surgically removed primary renal tumours in Children Clinical University Hospital from the year 1997 till 2010. Samples were fixed in 10% formalin fluid, imbedded in paraffin and haematoxylin-eosin stained slides were re-examined. Immunohistochemical re-investigation was made in 65.91% of cases. For differential diagnostic purposes were used antibodies for the detection of bcl-2, CD34, EMA, actin, desmin, vimentin, CKAE1/AE3, CK7, Ki67, LCA, WT1, CD99, NSE, chromogranin, synaptophyzin, S100, myoglobin, miogenin, MyoD1 (DakoCytomation) and INI1 protein (Santa Cruz Biotechnology). Results. During the revised period there were diagnosed 44 renal tumours. Accordingly of morphological examination data neoplasms were divided: 1) nephroblastoma – 75%, 2) clear cell sarcoma – 2.27%, 3) rhabdoid tumour – 4.55%, 4) angiomyolipoma – 4.55%, 5) embrional rhabdomyosarcoma – 2.27%, 6) mesoblastic nephroma – 4.55%, 7) multicystic nephroma – 4.55%, 8) angiosarcoma – 2.27%. Immunohistochemical investigation specify the diagnosis in 4.55% of patients. In case of embryonal rhabdomyosarcoma we found that immunophenotype is more typical for cellular mesoblastic nephroma (infantile renal fibrosarcoma) but one neuroblastoma was misdiagnosed as nephroblastoma. Conclusions. 1. The most common childhood renal tumours in Latvia like in the world is nephroblastoma. 2. In Latvia there are diagnosed such rare pediatric tumours as clear cell sarcoma and rhabdoid tumour; amount of first one is like in the world, but rhabdoid tumour was more common in Latvia then in the world. 3. No cases of renal cell carcinoma was diagnosed under age of 18 what differs from the data of the world. 4. The results of our investigations proved that immunohistochemical analytic schemes for blastemal type nephroblastoma and mesenchymal kidney tumours in our hospital should be extend but for the precise diagnosis of rhabdoid tumour of kidney would be recommended cooperation between laboratories of several countries for the detection of INI1 protein expression. Keywords: Paediatric renal tumours in Latvia, immunohistochemistry Abbreviations: WT – Wilms’ tumour, CCSK - clear cell sarcoma of the kidney, RTK - rhabdoid tumour of kidney, MN - mesoblastic nephroma, IFS - infantile fibrosarcoma, CMN - cellular mesoblastic nephroma WT1 – Wilms’ tumour gene marker, CKAE1/AE3 - common cytokeratin EMA - epithelial membrane antigen, LCA - leukocyte common antigen, NSE - neuron-specific enolase, H&E - haematoxylin- eosin, PNET – primitive neuroectodermal tumour, MA - metanephric adenoma. INTRODUCTION the development of accurate diagnostic criteria, stage Paediatric renal tumours represent 7% of all tumours in and histology-based therapeutic stratifications, and first 15 years of life. Wilms’ tumour or nephroblastoma appropriate surgical techniques. In relation to 5 year is the most common (85% of cases), followed by renal survival of patients there is a dramatic improvement cell carcinomas (3-5%), mesoblastic nephroma (3%), in prognosis in WT case – from 8% at the beginning clear cell sarcoma of the kidney, rhabdoid tumour of the of 20th century, to approximately 50% in 1960 kidney (2%) and miscellaneous rare tumours (2%) [6]. and greater than 90% in 2000 [13]. Equally 5 year Accurate histological diagnosis and staging of these survival of patients with CCSK has increased from tumours are critical because their treatment and only 20% up to 70% due to the addition doxorubicin prognosis are very different. During the past 40 years to chemotherapeutic protocols [3]. At the same time cooperative groups targeting paediatric renal tumours outcome of RTK is typical dismal, as over 80% of have been remarkably successful. They have enabled patients will die of tumour within 2 years of diagnosis 44 ACTA CHIRURGICA LATVIENSIS • 2011 (11) [18]. Mesoblastic nephroma in general is benign RESULTS tumour that classical variant histological is identical to Due to the clinically diagnosed primary renal tumours the fibromatosis with a survival rate 100%. The cellular 45 nephrectomies and 3 partial kidney resection were variant is associated with the worse prognosis as a done in years 1997-2010. The tumour diagnosis was survival rate of 5 years is 85% [1]. Histological cellular confirmed in 91.67% of cases (n=44), but in 8.33% (n=4) variant is identical to the infantile fibrosarcoma and pathologist findings did not meet the clinical diagnosis of shows translocation t(12; 15)(p13;q25) which leads to tumour. 6.25% (n=3) of cases the clinical diagnosis was the ETV6-NTRK3 gene fusion. Identical translocation primary renal tumour (lymphoma, renal cell cancer) has also been reported in fibrosarcoma suggesting that but histologically was found xanthogranulomatous the cellular subtype MN would represent an intrarenal pyelonephritis. In 2.08% (n=1) pathological process IFS [16]. 5% of the patients with mesoblastic cellular morphologically was not found. The amount of analyzed nephroma have recurrence usually within the first paediatric renal tumours during overviewed period year after the nephrectomy and only few cases had 1997-2010 is showed in Fig.1. metastases [1]. It is believed that completely excised MN 10 is associated with an excellent prognosis. Effectiveness 1997 9 of chemotherapy in CMN case is discussible as not all of them are chemosensitive. Candidates to adjuvant 8 chemotherapy are patients older than 3 month with the 7 cellular variant of tumour, neoplastic tissues in surgical 6 2008 margins, rupture of tumour during resection or vascular 5 2002 2009 microinvasions [1]. 4 1998 2000 2004 2005 2006 2007 3 AIM TO THE STUDY 2010 2 Aim of the study is to analyze morphologic spectrum of 1999 2003 1 children kidney tumours in Latvia and to characterise 2001 them from modern positions with wide range of 0 immunohistochemical markers using morphological material of Pathology bureau of Children’s Clinical Fig. 1. Number of Primary Paediatric Renal University Hospital. Tumours in Children’s Clinical University Hospital (1997_2010), Riga, Latvia. MATERIALS AND METHODS Our morphological examinations had proved such We have analyzed all surgically removed primary different subtypes of renal tumours: renal tumours cases in Children Clinical University 1) nephroblastoma – 75% (n=33), 2) clear cell sarcoma – Hospital from 1997 till 2010. In this study we have 2.27% (n=1), 3) rhabdoid tumour - 4, 55% (n=2), not included surgical material and biopsies from 4) angiomyolipoma – 4, 55% (n=2), 5) embrional kidneys due to others reasons: e.g., secondary tumours rhabdomyosarcoma – 2,27% (n=1), 6) mesoblastic of kidney or due to retroperitoneal neoplasm like nephroma – 4,55% (n=2), 7) multicystic nephroma – neuroblastoma with invasion into kidney. Primary 4,55% (n=2), 8) angiosarcoma- 2, 27% (n=1). morphological examination data and tissue material of After modern immunohistochemical analysis one case diagnosed tumours were retrieved from the archive of of nephroblastoma was interpreted as neuroblastoma. the Pathology bureau of Children Clinical University Histologically the tumour cells were small and Hospital. All tumour tissues were fixed in 10% formalin regular, with round, deeply staining nuclei and fluid, imbedded in paraffin, stained with haematoxylin– little cytoplasm. Few Homer Wright rosettes were eosin and re-tested. Complete immunohistochemical present. Immunohistochemistry showed complete investigation was provided in 65.91 % of cases. In lack of WT1expression, the tumour had no EMA and all analysed cases were detected WT1, vimentin, CKAE1/AE3 positive epithelial structures but reaction CKAE1/AE3, EMA, Ki67 antigen (all antibodies with neuronal markers as NSE, synatophysin and DakoCytomation, Denmark). In addition we have used chromogranin showed strong immunoexpression. antibodies for the detection of bcl-2, actin, desmin, CK7, Reactions for other childhood small round cell tumours LCA, CD99, NSE, chromogranin, synaptophyzin, S100, markers as LCA, desmin, vimentin, aktin, myoglobin, myoglobin, miogenin, MyoD1, CD34 (DakoCytomation, myogenin and CD99 were negative. Ki67 labelling Denmark) and INI1 protein (Santa Cruz Biotechnology, index was 40%. In one other nephroblastoma case re- USA). Proliferation activity of tumours was assessed investigation was encountered differential diagnostic counting Ki67 positive cells by magnification x400 and difficulty. Histological diagnosis in the morphological expressed as percentage.
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