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19–22 APRIL, 2017, BARCELONA, SPAIN
2 2m F
20x20mm solid hyper-
vascular tumor in the
posteromedial of left
kidney with malignant
features
Left
nephrectomy
Metanephric
stromal tumor
WT1:Positive
CD34:Positive
Vimentin:Positive
CD56:Negative
CD57:Negative
3 7m F
42x30mm solid
hypoecoic mass on
the lower pole of right
kidney
Right
nephrectomy
Metanephric
stromal tumor
WT1:Positive
CD34:Positive
4 8y
F
24x23mm solid well
circumscribed tumor on
the upper pole of left
kidney
Left
robotic partial
nephrectomy
Metanephric
stromal tumor
WT1:Focal positive
CD34:Focal positive
CD56:Negative
CD57:Negative
CONCLUSIONS
Wilms’ tumor constitutes the majority of renal tumors in children. As other renal tumors are very rare,
clinicians may opt to radical surgery for not taking them into consideration. Benign kidney tumors
should be kept in mind for nephron sparing surgery options especially in the presence of atypical
findings.
16:11–16:13
S3-11 (CP)
★
PRUNE PERINEUM SYNDROME: RARITY
AND COMPLEXITY. REPORT OF TWO CASES
Min Jeong BAG
1
, Marcos MELLO
2
, Bruno N CEZARINO
1
, Roberto I LOPES
1
,
Marcos G MACHADO
1
, Amilcar M GIRON
1
and Francisco T DÉNES
1
1) Hospital das Clinicas - FMUSP, Urology, São Paulo, BRAZIL - 2) Hospital das Clinicas - FMUSP, U, São Paulo,
BRAZIL
INTRODUCTION
Prune Perineum syndrome is characterized by a perineal mass with wrinkled skin - as in Prune Belly
syndrome -, imperforated anus, abnormal genitalia and pelvic abnormalities. We report 2 cases of
this rare syndrome.
CASES
Case 1: male patient born elsewhere was sent to our department aged 46 months with a colos-
tomy performed neonatally. He presented flaccid perineum, hypoplasic penis, impalpable testes,
imperforated anus and the buttocks were substituted by a mass of wrinkled skin with an orifice
draining urine. Image studies revealed single right kidney, intestinal malrotation, pubic diastasis and
distorted lumbo-sacro-coccygeal spine. Endoscopy of the posterior cavity did not visualize ureteral
meatus and urethra was identified with a guidewire.
An ileal neobladder with appendical conduit and orchiopexy were successfully performed at
52 months of age. Reconstruction of the spine and buttocks was planned for a second stage. The
patient had excellent recovery and was discharged after 2 weeks. Five months later he died from
complications of a misdiagnosed intestinal obstruction.
Case 2: male patient born elsewhere with flaccid inferior abdomen and perineum, anal imperfo-
ration, impalpable testes and hypoplasic penis, was sent to our institution aged 8 months, with
colostomy and vesicostomy performed neonatally. Image evaluation showed poor abdominal and
pelvic musculature, pubic diastasis, two normal kidneys with slight left ureterohydronephrosis and
colovesical fistula. Both ostomies are functional. A continent urinary diversion with reconstruction of
the pelvic floor with a muscular flap is planned.