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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.