Sapienza Università di Roma
U.O.C. Chirurgia Pediatrica - Policlinico Umberto I
Tumore di Wilms rotto:
chirurgia o chemioterapia?
Denis A. Cozzi
Caso clinico personale
Maschio, 10 anni
Riscontro di massa addominale palpabile
Asintomatico
Strategia terapeutica
Quali opzioni?
Indicazioni alla chemioterapia
neoadiuvante: protocollo americano
T. di Wilms bilaterale
T. di Wilms ad insorgenza su rene
unico
T. di Wilms con coinvolgimento di
organi adiacenti e/o estensione
tumorale nella cavale sopraepatica
NWTS-5 Staging system
III. Residual nonhematogenous tumor is present, confined to the
abdomen. Any one of the following may occur:
1. Lymph nodes within the abdomen or pelvis are found to be
involved by the tumor (renal hilar, pariaortic, or beyond).
2. The tumor has penetrated through the peritoneal surface.
3. Tumor implants are found on the peritoneal surface.
4. Gross or microscopic tumor remains postoperatively (e.g.,
tumor cells are found at the margin of surgical resection on
microscopic examination).
5. The tumor is not completely resectable because of local
infiltration into vital structures.
6. Tumor spill, not confined to the flank, occurred either before
or during surgery.
Preoperative chemotherapy in Wilms’ tumor
with contained retroperitoneal rupture
All 3 patients received post-op flank radiation
Rutigliano DN, et al. J Ped Surg 2007
Preoperative chemotherapy in Wilms’ tumor
with contained retroperitoneal rupture
Neoadjuvant chemotherapy may decrease
the chance of abdominal rupture at the time
of surgery and avoid total abdominal
radiation in favor of a more localized flank
radiation.
Rutigliano DN, et al. J Ped Surg 2007
SIOP-2001 Staging system
SIOP Postoperative Treatment Strategies for
Patients With Localized Wilms Tumors
According to Histologic Risk Group and Local
Stage
Brisse HJ, et al. Cancer 2008
Abdominal pain
(n=28)
Drop of hemoglubin
(n=12)
5
11
8
3
4
8
History of
abdominal trauma
(n=20)
Wilms Tumor Presenting With Abdominal
Pain: A Special Subgroup of Patients
Abdominal pain
(n=31)
No abdominal pain
(n=216)
Stage I
6%
47%
Stage II
23%
15%
Stage III
52%
23%
Stage IV
20%
15%
Davidoff AM, et al. Ann Surg Onc 1998
Retroperitoneal rupture
Brisse HJ, et al. Cancer 2008
Intraperitoneal rupture
Brisse HJ, et al. Cancer 2008
Intraperitoneal rupture
Brisse HJ, et al. Cancer 2008
Intraperitoneal rupture
Post-Chemotherapy
Brisse HJ, et al. Cancer 2008
Peritoneal recurrence (4 months post-op)
Brisse HJ, et al. Cancer 2008
Survival rate
5-year OS & EFS rates:
94.4% & 85.5%.
Local control rate significantly higher for
retroperitoneal-only vs intraperitoneal ruptures
Brisse HJ, et al. Cancer 2008
Conclusione (i)
Pazienti con TW stadio I/II e presenza
alla diagnosi di una rottura
retroperitoneale localizzata possono
esser trattati meno aggressivamente
dei pazienti di stadio III.
Does the pre-treatment tumour
rupture influence the outcome in
patients with nephroblastoma
submitted to preoperative
chemotherapy?
Godzinski J, Heij H, Cecchetto G, et al.
Pediatr Blood Cancer. 2005; 45:387.
Conclusione (ii)
Rotture intraperitoneali, descritte
all’imaging o all’intervento, devono
esser classificate come stadio III e
trattate più aggressivamente con
chemio e irradiazione di tutto l’addome.
Caso clinico personale
Quale strategia terapeutica?
Nefrectomia upfront
Caso clinico personale
AdenoCa renale tipo papillare,
moderatamente differenziato (G2)
Non infiltrata la capsula d’organo; vasi,
uretere, grasso perirenale e 12 linfonodi
esenti da infiltrazione neoplastica.
pT1a, pN0, pMx, stadio I
Follow-up: disease-free a 7 anni post-op.
Conclusione (iii)
Non vi è attualmente evidenza che il
rischio di recidiva addominale sia
aumentato in pazienti con imaging alla
diagnosi caratterizzato da una rottura
localizzata retroperitoneale.