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The tumour stage is the critical parameter for treatment, and its accurate assignment  is one of the most important and challenging duties of the pathologist.

Renal tumours of childhood are usually very large, and often it is very difficult to assess their relationship with normal anatomical structures such as the renal capsule and the renal hilum.

 The tumours are usually soft and friable resulting in difficulties during sampling and histological evaluation, such as displacement artefacts ("carry-over")  and capsular retraction.

The following procedure is recommended for handling the nephrectomy specimen:

1. Specimens should be received intact by the pathologist. The common surgical practice of bisecting in the operating theatre should be strongly discouraged.

2. The surface of the specimen should be inked (at least in areas of possible tumour infiltration).

3. After the ink is dry, an initial incision can be made to obtain samples  for cytogenetic, molecular biological and ultrastructural studies. This incision should display the relationship of the tumour to the kidney and renal hilum.

4. Further parallel incisions can be made and the specimen left to fix overnight. Never strip the capsule!

5. Tumour sampling should be extensive (at least 1 block per cm of tumour diameter), and the site of each block carefully documented (on  a drawing, or photograph). It is important to sample not only central areas of the tumour but also its periphery, the tumour-kidney interface, the renal hilum and its structures. Finally, the renal background parenchyma should be sampled.

                 

Staging should generally be done according to the criteria shown below.

Stage      Definitions

Stage 1     Tumour confined to the kidney and completely resected

                 (no penetration of the renal capsule ; no invasion of

                 renal hilum soft tissues or vessels)

Stage 2    Tumour extends beyond kidney but completely resected

                (renal capsule penetrated but resection margins clear;

                tumour present in the renal hilum fat or vessels, but the

                medial resection margin clear)

Stage 3    Gross residual tumour, involved resection margins; or

                 involved regional lymph nodes.

Stage 4     Hematogenous metastases, or lymph nodes beyond

                 local regional drainage area (e.g. mediastinal nodes)

Stage 5     Bilateral renal tumours  (But note: each tumour should

                 be substaged separately).  

Visit: Paediatric Pathology Online ; Paediatric Renal Tumours ; Wilms’ tumour (nephroblastoma)  ; Wilms' tumour related lesions ; Mesoblastic Nephroma ;  Nephrogenic rests ; Clear Cell Sarcoma of the Kidney ; Malignant Rhabdoid Tumour of Kidney  ; Immunohistochemistry and Molecular Biology of Wilms' Tumour and related lesions.


October 2007

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