ESPU Congress 2018 - Abstract Book

28 29 th CONGRESS OF THE ESPU 14:22–14:24 S2-7 (CP) MALIGN RHABDOID TUMORS OF KIDNEY AND BLADDER Hasan DELIAGA, Halil TOSUN, Bilge KARABULUT and Tugrul TIRYAKI University of Health Sciences, Ankara Child Health and Diseases Hematology Oncology Training and Res, Pediatric Urology, Ankara, TURKEY PURPOSE After defining the malignant rhabdoid tumor (MRT) as different entity by immunohistochemical studies, there has been little progress of rarity and aggressive behavior of the tumor. We aim to summarize three cases of malignant rhabdoid tumor with mean age of 13 months and discuss their clinical course. MATERIAL AND METHODS Nephrectomy was performed in two female patients with MRT of kidney and biopsy in one male MRT of bladder patient. All patients had adjuvant chemotherapy according to the NWTSG and IRSG protocols. Two patients had metastasis to lungs, brain and mesentery before diagnosis. Metastasis occurred in one MRT of kidney patient in liver and lungs 4 months after diagnosis while taking chemotherapy. Both patients with MRT of kidney died of disease six months after diagnosis and the MRT of bladder patient is alive with the disease 2 months after the diagnosis. RESULTS The main problem for these patients is making the diagnosis. Because of its resemblance to Wilms and rhabdomyosarcoma clinically, suitable chemotherapy and radiotherapy are late onset until pathologic diagnosis is made. Aggressive behavior of the tumor gives rise to metastases before diagnosis and even during chemotherapy. Surgery is both reported to improve and worsen the prognosis. We observed that surgery seemed to have no effect on survey. There is no recom- mended chemotherapy protocol but multidrug high-dose chemotherapy seems to be promising. CONCLUSIONS Although extensive studies are made by NWTSG, SIOP and IRSG, there is not a proper treatment algorithm for treatment. The survey is still poor and survivors are still sporadic. 14:24–14:26 S2-8 (CP) PRIMARY RENAL NEUROBLASTOMA WITH EXTENDED TUMOR THROMBUS IN VENA CAVA INFERIOR PRESENTING LIKE WILMS TUMOR IN AN 2 YEARS-OLD BOY Mazen ZEINO  1 , Milan MILOSEVIC  1 , Roland AMMANN  2 , Axel KAROW  2 and Jürg SCHMIDLI  3 1) University of Bern, Inselspital Bern, Department of paediatric urology, Bern, SWITZERLAND - 2) University of Bern, Inselspital Bern, Pediatric Hemato- Oncology, Bern, SWAZILAND - 3) University of Bern, Inselspital Bern, Vascular Surgery, Department of Cardiovascular Surgery, Bern, SWITZERLAND PURPOSE Neuroblastoma is a rare cancer of the sympathetic nervous system that affects children, mostly under the age of 5 years old. Most frequently it starts from one of the adrenal glands, but can also develop in the neck, chest, abdomen, or spine. Primary renal neuroblastoma is extremely rare, with very few case reports in the literature.