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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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Mixed epithelial and stromal tumour<br />

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

Definition<br />

Mixed epithelial and stromal tumour is a<br />

complex renal neoplasm composed of a<br />

mixture of stromal and epithelial elements.<br />

Synonyms<br />

Some authors have applied other names<br />

(cystic hamartoma of renal pelvis or<br />

adult mesoblastic nephroma) but the<br />

name "mixed epithelial and stromal<br />

tumour" best captures its nature {2035}.<br />

Clinical features<br />

There is a 6:1 predominance of women<br />

over men {35}. All have been adults and<br />

the mean age is perimenopausal (46<br />

years). Presenting symptoms include<br />

flank pain, haematuria or symptoms of<br />

urinary tract infection; 25% are incidental<br />

findings. Histories of estrogen therapy<br />

are common. Surgery has been curative<br />

in all cases.<br />

to complex branching channels which<br />

may be dilated. These varied elements<br />

often are present intermingled in the<br />

same area of the tumour. The stroma<br />

consists of a variably cellular population<br />

of spindle cells with plump nuclei and<br />

abundant cytoplasm. Areas of myxoid<br />

stroma and fascicles of smooth muscle<br />

cells may be prominent. Densely collagenous<br />

stroma is common and fat is<br />

occasionally present. Mitotic figures and<br />

atypical nuclei have not been reported.<br />

Fig. 1.115 Mixed epithelial and stromal tumour.<br />

Large tumour attached to the renal pelvis.<br />

Macroscopy<br />

The tumours often arise centrally in the<br />

kidney and grow as expansile masses,<br />

frequently herniating into the renal<br />

pelvic cavity. The tumours are typically<br />

composed of multiple cysts and solid<br />

areas.<br />

Histopathology<br />

These are complex tumours composed<br />

of large cysts, microcysts, and tubules.<br />

The largest cysts are lined by columnar<br />

and cuboidal epithelium, which sometimes<br />

forms small papillary tufts.<br />

Urothelium, which may be hyperplastic,<br />

may also line some portion of the cysts.<br />

The microcysts and tubules are lined by<br />

flattened, cuboidal, or columnar cells.<br />

Their cytoplasm ranges from clear to<br />

pale, eosinophilic, or vacuolated.<br />

Epithelium with müllerian characteristics<br />

has also been described {205}. The<br />

architecture of the microcysts is varied<br />

and ranges from simple microcysts with<br />

abundant stroma between them, to<br />

densely packed clusters of microcysts,<br />

A<br />

Fig. 1.116 Mixed epithelial and stromal tumour. A Predominantly solid mass with scattered cysts. B Note<br />

glancing inner surface of the cystic tumour.<br />

Fig. 1.117 Mixed epithelial and stromal tumour forming spatulate papillae. Note fat cells in stroma.<br />

B<br />

Cystic nephroma / Mixedepithelial and stromal tumour<br />

77

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