Abstract

GARCIA PENALVER, C.; LOPEZ VAZQUEZ, F.; GOMEZ FRAILE, A.  and  ARANSAY BRAMTOT, A.. What is the appropriate therapeutic approach to nephroblastomatosis?: A report of 2 cases. Actas Urol Esp [online]. 2003, vol.27, n.10, pp.809-813. ISSN 0210-4806.

Nephroblastomatosis is a complex of pathological conditions defined by the persistence of nephrogenic elements after the conclusion of nephrogenesis (beyond week 36 of gestation) that conserve the capacity to evolve into nephroblastoma. Luckily, this malignant transformation is exceptional (<1%¹), which is why the disease is considered to have a good prognosis. The nephrogenic remains are frequently associated with Beckwith-Wiedemann syndrome, hemihypertrophy and aniridia, in which case there is a greater risk of the development of Wilms tumor. Consequently, these children must be followed-up closely. The difficulty lay in knowing when to act, which children should be treated, and what treatment is appropriate because what little has been published in the literature is debatable. We report the cases of two patients, one with unilateral nephroblastomatosis and the other bilateral, who required surgical treatment after not responding to chemotherapy; and we expose our therapeutic algorithm and follow-up strategy.

Keywords : Nephrogenic rests; Nephroblastomatosis; Wilm's tumor.

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