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Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

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Cystic partially differentiated<br />

nephroblastoma<br />

J.N. <strong>Eble</strong><br />

Definition<br />

Cystic partially differentiated nephroblastoma<br />

is a multilocular cystic neoplasm of<br />

very young children, composed of epithelial<br />

and stromal elements, along with<br />

nephoblastomatous tissue.<br />

ICD-O code 8959/1<br />

Fig. 1.76 Cystic partially differentiated nephroblastoma<br />

forms a well-circumscribed mass composed<br />

entirely of small and large cysts.<br />

Rarely, Wilms tumour may be composed<br />

entirely of cysts with delicate septa.<br />

Within the septa are small foci of<br />

blastema, immature-appearing stromal<br />

cells, and primitive or immature epithelium.<br />

Such tumours are called "cystic partially<br />

differentiated nephroblastoma"<br />

{329,1249}. When no nephroblastomatous<br />

elements are found, the term "cystic<br />

nephroma" has been applied<br />

although it is recognized that these<br />

lesions are not the same as the morphologically<br />

similar ones which occur in<br />

adults {646,650}.<br />

Cystic partially differentiated nephroblastoma<br />

occurs with greater frequency<br />

in boys than in girls; almost all patients<br />

are less than 24 months old, and surgery<br />

is almost always curative {592,<br />

1250,1251}. Joshi and Beckwith reported<br />

one recurrence, possibly a complication of<br />

incomplete resection {1250}.<br />

The tumours often are large, particularly<br />

considering the patient's age, ranging<br />

up to 180 mm in diameter. Cystic partially<br />

differentiated nephroblastoma is<br />

well circumscribed from the remaining<br />

kidney by a fibrous pseudocapsule and<br />

consists entirely of cysts of variable<br />

size. The septa are thin and there are no<br />

expansile nodules to alter the rounded<br />

contour of the cysts.<br />

The cysts in cystic partially differentiated<br />

nephroblastoma and are lined with<br />

flattened, cuboidal, or hobnail epithelium,<br />

or lack lining epithelium {1249}. The<br />

septa are variably cellular and contain<br />

undifferentiated and differentiated mesenchyme,<br />

blastema, and nephroblastomatous<br />

epithelial elements {1249}.<br />

Skeletal muscle and myxoid mesenchyme<br />

are present in the septa of<br />

most tumours. Cartilage and fat are<br />

present occasionally {1250,1251}.<br />

Focally, the septal elements may protrude<br />

into the cysts in microscopic papillary<br />

folds, or gross polyps in the papillonodular<br />

variant of cystic partially differentiated<br />

nephroblastoma. The epithelial<br />

components consist mainly of<br />

mature and immature microscopic cysts<br />

resembling cross sections of tubules<br />

and stubby papillae resembling immature<br />

glomeruli.<br />

A<br />

Fig. 1.77 Cystic partially differentiated nephroblastoma. A The septa of cystic partially differentiated nephroblastoma often contain aggregates of blastema. B<br />

Pericystic part of the tumour contains immature epithelial elements forming short papillae reminiscent of fetal glomeruli.<br />

B<br />

Cystic partially differentiated nephroblastoma<br />

55

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