40 / 330
40
28
TH
CONGRESS OF THE ESPU
RESULTS
Among 44 patients with 9q22.3 syndrome, 7 developed a WT (16%) at a mean age of 45 months
(range: 5-84).
One patient had a synchronous bilateral WT. This patient was treated by bilateral nephron spar-
ring surgery (NSS). Another patient with a unilateral WT had a NSS (upper pole nephrectomy) via
laparoscopy.
The 5 other patients had unilateral radical nephrectomy (1 laparoscopy) with simultaneous resec-
tion of a pelvic rhabdomyosarcoma in 1 case.
All the patients had a standard histology.
Five tumors were stage !, 1 stage II and 1 stage III.
All the patients are in complete remission.
CONCLUSIONS
9q22.3 syndrome has to be considered as a new predisposing syndrome to WT.
The patients should be followed as the patients with Beckwith- Wiedemann syndrome.
As in other predisposing syndromes, nephron sparring surgery should be done whenever possible.
16:09–16:11
S3-10 (CP)
★
CAN METANEPHRIC TUMORS OF KIDNEY BE PREDICTED
PREOPERATIVELY?
Gozde SAKUL
1
, Sibel TIRYAKI
2
, Ali TEKIN
3
, Ismail YAGMUR
4
, Banu SARSIK
5
,
Nevra ELMAS
6
, Hudaver ALPER
6
, Ibrahim ULMAN
7
and Ali AVANOGLU
1
1) Ege University, Pediatric Surgery, Izmir, TURKEY - 2) Ege University-Faculty of Medicine, Department of Pediatric
Surgery, Division of Pediatric Urology,, Izmir, TURKEY - 3) Cigli State Hospital, Pediatric Urology, Izmir, TURKEY
- 4) Harran University, Urology, Division of Pediatric Urology, Izmir, TURKEY - 5) Ege University, Pathology, Izmir,
TURKEY - 6) Ege University, Radiology, Izmir, TURKEY - 7) Ege University, Pediatric Surgery Division of Pediatric
Urology, Izmir, TURKEY
PURPOSE
Metanephric tumors of kidney are rare benign kidney tumors of childhood. Some authors accept
them as a well differentiated variant of Wilms’ tumor; however, tumor behaviour is totally different.
The aim of this study is to evaluate the diagnostic features and treatment options of these rare
tumors.
PATIENTS
We report four children with metanephric kidney tumors diagnosed incidentally. Three of them
were considered as malignant preoperatively and underwent total nephrectomy. The fourth patient
underwent partial nephrectomy for a strong suspicion of a benign tumor. When the former patients
were evaluated retrospectively, their magnetic resonance images were considered to have some
differing features from Wilms’ tumor. All tumors were well circumscribed, have a solid component,
less heterogenous and had lower contrast enhancement when compared to Wilms’ tumor.
RESULTS
Age Gender
Imaging-MR
Surgery
Pathology
Immuno-
histochemical
Staining
1 5y
F
34x30mm solid mass
on the lower pole of left
kidney
Left
nephrectomy
Metanephric
adenoma
WT1:Positive
CD56:Focal positive
CD57:Focal positive