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Congenital Mesoblastic Nephroma: Possible Prognostic and Management Value of Assessing J Clin Pathol: First Published As 10.1136/Jcp.44.4.317 on 1 April 1991

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Congenital Mesoblastic Nephroma: Possible Prognostic and Management Value of Assessing J Clin Pathol: First Published As 10.1136/Jcp.44.4.317 on 1 April 1991 J Clin Pathol 1991;44:317-320 317 Congenital mesoblastic nephroma: Possible prognostic and management value of assessing J Clin Pathol: first published as 10.1136/jcp.44.4.317 on 1 April 1991. Downloaded from DNA content J C Barrantes, C Toyn, K R Muir, S E Parkes, F Raafat, A H Cameron, H B Marsden, J R Mann Abstract appears benign with a leiomyomatous pattern The case records and pathology of all composed of interlacing bundles of spindle children with kidney tumours treated in cells and few mitotic figures; congenital the West Midlands Health Authority mesoblastic nephroma is densely cellular, with Region (WMHAR) from 1957 to 1986 many mitotic figures. Both patterns can be were reviewed. The histology was re- found in the same tumour, referred to as a viewed by a panel of three paediatric mixed tumour.9 pathologists. Thirteen (6%) out of 211 In trying to explain the histogenesis of these cases were considered to have congenital tumours, Snyder et al"' proposed a theory mesoblastic nephroma (CMN). Nine using the "two hit" model, described by were of the conventional type, three of Knudson." In this model congenital meso- the atypical cellular type, and one blastic nephroma would occur after a neoplas- mixed. DNA ploidy was investigated and tic mutation in the early stages of embryogen- showed two of the tumours to be aneu- esis, whereas atypical cellular mesoblastic ploid and nine diploid (tissue was not nephroma would develop in the later stages, available in the two other cases). The two but before the blastema has undergone meta- aneuploid tumours were of atypical nephric differentiation. In both cases a second cellular and mixed histology, respec- mutation would be necessary to produce the tively; the diploid tumours were of the malignant transformation. Its origin from pre- conventional type in eight cases and metanephric blastema is supported by an atypical cellular in one. immunohistochemical study by Kumar et al.'2 http://jcp.bmj.com/ The atypical cellular type has been Furthermore, Haas et al"' suggested that, on reported to behave more aggressively, the basis of the highly cellular histological but the benefit of additional treatment appearances of atypical cellular mesoblastic after surgery to prevent recurrence nephroma, clear cell sarcoma of the kidney remains unclear. Measurement of DNA (CCSK) may be the malignant counterpart of content by flow cytometry, together with congenital mesoblastic nephroma and atypical histological subclassification, may be cellular mesoblastic nephroma. More recent on September 30, 2021 by guest. Protected copyright. useful in selecting patients who will studies, however, indicate that this is un- Department of benefit from further treatment after likely.9 Oncology, The surgery. With a view to investigating the biological Children's Hospital, characteristics and the possible factors that Ladywood, Birmingham B16 SET could influence the decision as to which J C Barrantes Congenital mesoblastic nephroma is one of patients would need further treatment in J R Mann the commonest kidney tumours in infants. It addition to surgical resection, we undertook Department of has been associated with a very good prog- review of patients diagnosed as having kidney Pathology, The nosis and, in most cases, surgery alone may tumours in the West Midlands Health Auth- Children's Hospital, Birmingham effect a cure.' ority Region between 1957-1986. As DNA C Toyn It has also been recognised, however, that ploidy has shown a correlation with prognosis F Raafat not all of the cases follow the same course.24 A in Wilms' tumour,'4 15 which explains its The West Midlands more aggressive subtype, atypical cellular aggressive behaviour in some patients, we also Regional Children's mesoblastic with evidence of local Tumour Research nephroma performed flow cytometry wherever possible. Group, The Children's recurrence and even distant pulmonary metas- The objectives of the present study were: Hospital, Birmingham tases, has been reported in some patients,56 (1) To review the incidence, clinical presen- K R Muir but the reasons for the more aggressive be- and outcome of with con- S E Parkes tation, patients A H Cameron haviour are not known precisely. The exis- genital mesoblastic nephroma in the West The Royal Manchester tence of a spectrum of tumours covering the Midlands over a 30 year period. Children's Hospital, whole span of biological behaviour has been (2) To determine if there is any difference in Manchester proposed,78 identifying congenital mesoblastic DNA ploidy between typical congenital H B Marsden nephroma as a low grade tumour and placing mesoblastic nephroma and atypical Correspondence to: atypical cellular mesoblastic nephroma in the cellular mesoblastic nephroma and any Dr J R Mann on histo- Accepted for publication intermediate category. This is based possible implications for the biological 21 November 1990 logical appearances: mesoblastic nephroma nature and behaviour of the subgroups. 318 Barrantes, Toyn, Muir, Parkes, Raafat, Cameron, Marsden, Mann Methods Results We reviewed the case records of children aged From a total of 211 kidney tumours diagnosed 0-14 years, resident in the West Midlands in the study period, 13 (6-2%) cases of con- Health Authority Region (average childhood genital mesoblastic nephroma were found. J Clin Pathol: first published as 10.1136/jcp.44.4.317 on 1 April 1991. Downloaded from population 1 17 million) who were diagnosed Table 1 shows the principal characteristics of as having kidney tumours between 1957 and the whole group. Although most children 1986. (eight of 13) presented during infancy, two The cases were identified from the West were over 1 year of age. There were eight males Midlands Regional Children's Tumour and five females, ratio 1 -6: 1. Research Group. Data are held on all cases of The most common presenting features were childhood cancer (0-14 years) in the abdominal distension and mass. Only one WMHAR from 1957, based on the records of patient had a congenital abnormality, present- the Birmingham and West Midlands Regional ing with a congenital mesoblastic nephroma in Cancer Registry, the Children's Cancer the left side of a horse-shoe kidney. Research Group, Oxford, and the files of the All patients had surgery as the principal Oncology and Histopathology Departments of treatment. Eleven had a nephrectomy, one had The Children's Hospital, Birmingham. the tumour excised from a horse-shoe kidney, Clinical and histological staging of these and one with a well localised tumour had a tumours were carried out following the same partial nephrectomy. In seven cases surgery staging system as for Wilms' tumour, pro- was the only treatment (including the child posed after the National Wilms' Tumour with the horse-shoe kidney). Three of the Study (NWTS-2).'6 The available pathology patients received chemotherapy after surgery, material was reviewed by a panel of three two had radiotherapy to the tumour bed, and paediatric histopathologists. one had both. Sections 30 mm thick from paraffin wax The tumours were stage I in four patients embedded pathological material were and stage II in eight, because of local spread obtained and prepared for flow cytometry, through the capsule into the perirenal tissue. according to the method described by One patient (case 6) had the primary tumour Hedley,'7 with minor modifications. confined to the kidney, but malignant cells were The nuclear DNA content of 20 000 cells suspected in the bone marrow, so he was was measured using a Becton Dickinson treated with chemotherapy and radiotherapy, FACS 440 flow cytometer with an excitation following the Medical Research Council wavelength of 448 nm, emission filter of Wilms' tumour protocol. Histological review 590 nm LP, and laser power of 0-2 watts. showed that nine had congenital mesoblastic Normal kidney tissue was used as an external nephroma of the conventional type, three had control, and stromal cells from each sample atypical cellular mesoblastic nephroma, and were taken as the normal internal standard. one had a mixed tumour. http://jcp.bmj.com/ The DNA content was considered to be Analysis of DNA content was possible in 11 diploid when only one gO/gl peak was present cases, nine ofwhich were diploid (fig 1) and two in the histogram and aneuploid when two or aneuploid (fig 2). The two aneuploid tumours more gO/gl peaks were obtained. S phase were of cellular and mixed types, respectively. (synthetic phase) was identified as the per- The results of the DNA studies are shown in centage of cells lying between the gO/gl and table 2. the g2M peaks. The DNA index (DI) repre- The PI was very high in one patient (case 9) s-ented the ratio of the modal channel number (table 2), mildly increased in another (case 1) on September 30, 2021 by guest. Protected copyright. of the aneuploid gO/gl peak to the modal (table 2), and the remainder of the patients had channel number of the diploid gO/gl peak. a PI of < 10%. The DI of the aneuploid The proliferation index (PI) was obtained tumours was 1-4 and 13, respectively. The by the sum of the cells in S phase and g2M. median CV of the whole group was 8-3 with a The coefficient of variation (CV) was obtained range of 4 7-10. The histograms of some of our using the resident Becton Dickinson software. patients exhibited considerable variation in the Evaluable information for DNA ploidy was position of the gO/gl and g2M peaks in other- obtained in 11 of the 13 patients. wise normal histograms. This effect has been Table 1 Congenital mesoblastic nephroma: general characteristics Case Age Histological Follow up No (m) Sex Side Stage type Treatment* Outcome (years) 1 NB M L 2 CMN RT D 0 75 3 NB M L 1 CMN None A 14 5 4 NB F R 2 CMN None D 5 days 5 NB M L 2 CMN None A 11.6 8 NB M R 2 CMN None A 5 8 9 13 F L 2 CMN CT A 5 10 NB M L 2 CMN None A 0 4 11 NB F L 2 CMN None A 1-4 13 NB F L 1 CMN None A 2-8 6 19 M R ?4t ACMN CT; RT A 8-3 7 1 M L 1 ACMN CT A 5-8 12 7 F L 1 ACMN CT A 3-3 2 3 M R 2 Mixed RT A 25 *In addition to nephrectomy.
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