RESULTS: All the authentic human pathological samples, except one anaplastic WT, as well the WT xenografts, expressed COX-2 in all cellular components of WT. Expression was also observed in WT lung metastasis and in tumors which over-grew chemotherapy. Expression of COX-2 in normal kidneys was confined to the tubular epithelium, both in the cortex and medulla, and to the sinusoidal cells in normal liver. No expression of COX-2 was detected in normal lung.

CONCLUSIONS: Expression of COX-2 is characteristic of all WT tumors stages and is in correlation with the pan-expression of the erbB2 receptor in these tumors. Since we have already demonstrated a therapeutic effect of the anti ebrB2 antibodies in WT xenografts, and since COX-2 is downstream of the erbB2 receptor, the potential therapeutic role of COX2 inhibitors should be evaluated in WT.

69

MYOGENIN AND DESMIN IMMUNOHISTOCHEMISTRY IN THE ASSESSMENT OF POSTCHEMOTHERAPY GENITOURINARY EMBRYONAL RHABDOMYOSARCOMA: PROGNOSTIC AND MANAGEMENT IMPLICATIONS

Prasad GODBOLE, Alice OUTRAM^*, Duncan WILCOX^†, Patrick DUFFY^† and Neil SEBIRE^‡
Great Ormond Street Hospi, Paediatric Urology, London, UNITED KINGDOM - * Great Ormond Street Hospital, Urology, London, UNITED KINGDOM - † Great Ormond Street Hospital, Paediatric Urology, London, UNITED KINGDOM - ‡ Great Ormond Street Hospital, Histopathology, London, UNITED KINGDOM

PURPOSE: The presence of definite areas of residual undifferentiated embryonal rhabdomyosarcoma (eRMS) post genitourinary (GU) eRMS treatment indicates residual / recurrent disease. The clinical significance of scanty positive desmin and myogenin staining cells in resected specimens and surveillance biopsies which appear ‘undifferentiated’ on light microscopy is unknown. We reviewed our retrospective experience of GU eRMS to examine the relationship between presence of immunostained ‘undifferentiated’ cells and clinical course.

MATERIAL AND METHODS: The histopathological findings of l2 children with biopsy proven localised GU eRMS on the initial resection / post treatment biopsy were reviewed and all specimens were immunostained with desmin and myogenin to detect residual ‘undifferentiated’ rhabdomyoblasts. The relation between histopathological findings and outcome was determined.

RESULTS: Eleven showed of well-differentiated post treatment rhabdomyoblasts on H&E staining, with margins apparently free of tumour, and one showed no morphological evidence of residual RMS. All demonstrated at least scanty desmin and myogenin positive cells. Four had no further treatment nor recurrence at 10y(8-13) post treatment. Eight had further treatment followed by further resection in three. One patient died from her disease but seven remain alive and well at 7.2y(8m-13y)

CONCLUSIONS: In some cases the presence of ‘undifferentiated’ myogenin / desmin positive cells in post treatment GU eRMS is associated with clinical disease recurrence, whilst others , have no clinical sequelae even in the absence of further treatment. In GU eRMS close and regular clinical surveillance is essential, and the use of desmin / myogenin immunohistochemistry to detect scattered ‘undifferentiated’ cells does not appear to provide useful prognostic information in an individual case.

70

DOES THE LESS AGGRESSIVE MULTIMODAL APPROACH OF BLADDER-PROSTATE RHABDOMYOSARCOMA PRESERVE BLADDER FUNCTION?

Roberto SOLER, Antonio MACEDO, Homero BRUSCHINI, Fabiola PUTY^*, Eliana CARAN^*, Antonio PETRILLI^†, Gilmar GARRONE, Valdemar ORTIZ and Miguel SROUGI
Pediatric Oncology Institute - Paulista School of Medicine, Urology, São Paulo, BRAZIL - * Pediatric Oncology Institute - Paulista School of Medicine, Pediatric Oncology, São Paulo, BRAZIL - † Pediatric Oncology Institute - Paulista School of Medicine, Paediatric Oncology, São Paulo, BRAZIL

PURPOSE: The treatment of bladder-prostate rhabdomyosarcoma has evolved into multimodal therapy. Despite this less invasive approach, some authors described bladder dysfunction in all patients receiving radiotherapy. The aim of this study is to evaluate urinary dysfunction in children submitted to this modality of therapy in our institution.

MATERIAL AND METHODS: We evaluated 10 children treated in our institution from 1999 to 2003 according to IRS-IV criteria. All patients were submitted to anamnesis and multichannel urodynamic study at least 6 months after treatment.

RESULTS: Two patients were excluded from the study, one boy because of local recurrence and need for salvage surgery; and another one due to cranium encephalic trauma during the course of the treatment. In the series of 8 patients only one nedded surgery due to disabling dysuria. The results are shown in Table 1.

71

LAPAROSCOPIC NEPHRECTOMY FOR WILMS’ TUMOR AFTER CHEMOTHERAPY: INITIAL EXPERIENCE

Francisco DéNES, Ricardo DUARTE^*, Vicente ODONE FILHO^† and Lilian CRISTOFANI^†
Saõ Paulo University, Urology, São Paulo, BRAZIL - * São Paulo State University, Urology, São Paulo, BRAZIL - † São Paulo State University, Pediatrics, S, BRAZIL

PURPOSE: Treatment of unilateral Wilms’ tumor includes chemotherapy followed by radical nephrectomy. Herein we describe the results of laparoscopic radical nephrectomy .

MATERIAL AND METHODS: Seven children, mean age 3.7 ys., underwent transperitoneal nephrectomy with four ports. After evaluation of the cavity, the colon is mobilized, exposing the renal vessels and ureter, that are isolated and sectioned. The kidney, perirenal fat and adrenal are dissected en-bloc. When liberated, the specimen is mobilized to allow lymphadenectomy. The specimens are inserted in a bag and removed through Pffanenstiehl incision. The cavity is reviewed, the trocars removed and incisions closed, without drains. The patients were evaluated regarding complications, the pathological results reviewed.

RESULTS: There were no complications, transfusion or tumor rupture. In one patient, the tumor had adhesions to the liver, being successfully separated with harmonic scalpel. The postoperative period was uneventful in all cases, except for abdominal distension in one. The patients were discharged in the second or third postoperative day, without analgesics. Histopathology revealed complete tumor necrosis in two patients, with contamination of perirenal fat and surgical margin in one. All others had viable tumor cells, but the tumor was totally resected. No patient presented lymphatic metastasis. All patients received further chemotherapy and remain free of disease for 6+ to 13+ months.

PURPOSE: Intravascular extension of Wilms tumor has been reported at 4.1%. A series of case reports have described the management of Wilms tumor with intra atrial extension with only one multi institutional study investigating outcomes within NWTS-4. We review our experience with cardiopulmonary bypass in the management of Wilms tumor with extension of tumor thrombus into the right atrium.

MATERIAL AND METHODS: A retrospective review of all operative cases from 1993 to 2003 identified all patients that underwent Wilms nephrectomy with the assistance of cardiopulmonary bypass. These charts were reviewed and analyzed for patient demographics, disease classification, renal function, surgical operative technique, incidence of complications, and outcomes. Details of the surgical technique are described.

RESULTS: Four patients were identified with Wilms tumor that after preoperative chemotherapy had evidence of tumor extension into the right atrium by CT scan. Median age of diagnosis was 39 months (range 30-53). One case was bilateral, two 2 right-sided tumors, and 1 left sided tumor. All children underwent nephrectomy and thrombectomy under cardiopulmonary bypass support. Median time on bypass was 90 minutes (range 47-127 minutes). Blood loss could not be estimated due to use of the bypass pump. The inferior vena cava was opened in all cases and subsequently repaired with a bovine pericardial patch. There was no evidence of postoperative renal insufficiency as monitored by plasma creatinine levels. Complications included pleural effusion (n=2), pericardial effusion (n=1), gross hematuria (n=1), and ileus (n=2). Three children have no evidence of disease on follow up while one has local recurrence after post operative chemotherapy and radiation.

Please log-in or register in order to submit comments.

Powered by AkoComment!