Bilateral nephroblastomatosis Case presentation: Dr Babak Abdolkarimi 18/5/97

دختربچه 3 ساله باسابقه عفونت ادراری مکرردرمان شده وتحت پیگیری سرویس نفرولوژی که درارزیابی دوره ای به طوراتفاقی درسونوگرافی متوجه 3توده درهردوکلیه میشوند

Incidentally finding in KUB-US:

اولین CT بعدازشروع کموتراپی

Biopsy after resection & nephrectomy

General points: Nephroblastomatosis is defined by the presence of diffuse or multifocal nephrogenic rests (NR), The treatment of nephroblastomatosis remains controversial . Histologic examination within a lesion has limited usefulness, as hyperplastic NR can be similar to favorable Wilms tumor on microscopy ..

However, an open biopsy that includes a portion of the lesion + adjacent normal renal cortex may be helpful to eliminate the possibility of a more tumor that would have required more chemotherapy. Given the reliability of imaging studies, biopsies do not now appear warranted when the appearance is typical for NB, especially in infants. Beyond infancy, the differential diagnosis : WT leukemic and lymphomatous infiltrates. If the imaging appearance or clinical presentation are atypical, biopsy proof is indicated.

treatment The treatment of NB is controversial. It is a benign process, yet has a malignant potential---. prophylactic chemotherapy

The arguments against initial chemotherapy are: 1) the risks of chemotherapy to treat a benign disease that may regress spontaneously; 2) the uncertainty that chemotherapy will actually prevent the development of WT; 3) the fear that chemotherapy will select more responsive cells, hence leaving anaplastic WT clones that will proliferate.

The arguments in favor of chemotherapy for NB are: 1) to treat an undiagnosed WT within the NB lesions; 2) to decrease the number of cells at risk for malignant transformation; 3) to prevent damage to the normal renal cortex by compression from massive NB [6].

1. If the lesions are typical of NB in a young child, he/she is either observed or treated with VCR + AMD if renal function appears at risk. Imaging studies are repeated every 3–4 weeks initially. Stable or regressing lesions are observed, while patients with progression are either started on chemotherapy or 2.surgically indications for exploration: a.if progression occurs during chemotherapy. b.It is the changing appearance of a lesion on imaging studies, occurred .

3.Nephrectomy should be avoided as the initial treatment of NB, even when the disease is unilateral, because of the risk of WT on the contralateral side. 4.summarizes the need for a “nephron sparing” approach: “If you leave it in today, you can take it out tomorrow; but if you take it out today, you can’t put it back in tomorrow.” 5.Regrowth of NB lesions after an initial course of chemotherapy is a challenging problem. a. If a single nodule regrows and is amenable to wedge resection or partial nephrectomy, this should be done; b.on the other hand, diffuse lesions that retain the typical imaging appearance of NB should be treated again with VCR and AMD. There are no guidelines as to the ideal duration of a course and the interval between each course.

مرحله 1 درمان

Consult 1:

Recommended pathologist:

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