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

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

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Metanephric adenoma and metanephric<br />

adenofibroma<br />

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

D.J. Grignon<br />

H. Moch<br />

Definition<br />

Metanephric adenoma is a highly cellular<br />

epithelial tumour composed of small, uniform,<br />

embryonic-appearing cells.<br />

ICD-O codes<br />

Metanephric adenoma 8325/0<br />

Metanephric adenofibroma 9013/0<br />

Metanephric adenosarcoma 8933/3<br />

Epidemiology<br />

Metanephric adenoma occurs in children<br />

and adults, most commonly in the fifth<br />

and sixth decades. There is a 2:1 female<br />

preponderance {561}. Patients with<br />

metanephric adenofibroma have ranged<br />

from 5 months to 36 years (median = 30<br />

months) {120}. There is a 2:1 ratio of males<br />

to females. A single case of high grade sarcoma<br />

arising in association with<br />

metanephric adenoma (metanephric<br />

adenosarcoma) has been reported {2072}.<br />

Clinical features<br />

Approximately 50% of metanephric adenoma<br />

are incidental findings with others<br />

presenting with polycythemia, abdominal<br />

or flank pain, mass, or hematuria.<br />

Presenting symptoms of metanephric<br />

adenofibroma have included polycythemia<br />

or hematuria; some have been<br />

incidental findings. Arroyo et al. {120}<br />

described several cases in which either<br />

Wilms tumour or carcinoma occurred in<br />

association with metanephric adenofibroma.<br />

Other than one patient with<br />

regional metastases from the carcinoma,<br />

these patients have had no progression.<br />

Macroscopy<br />

Metanephric adenomas range widely in<br />

size; most have been 30 to 60 mm in<br />

diameter {561}. Multifocality is uncommon.<br />

The tumours are typically well circumscribed<br />

but not encapsulated. The<br />

cut surfaces vary from grey to tan to yellow<br />

and may be soft or firm.<br />

Foci of haemorrhage and necrosis are<br />

common; calcification is present in<br />

approximately 20%,and small cysts in<br />

10% {561,1237}.<br />

Metanephric adenofibromas are typically<br />

solitary tan partially cystic masses with<br />

indistinct borders {120}.<br />

Histopathology<br />

Metanephric adenoma is a highly cellular<br />

tumour composed of tightly packed<br />

small, uniform, round acini with an<br />

embryonal appearance. Since the acini<br />

and their lumens are small, at low magnification<br />

this pattern may be mistaken for<br />

a solid sheet of cells. Long branching<br />

and angulated tubular structures also are<br />

common. The stroma ranges from inconspicuous<br />

to a loose oedematous stroma.<br />

Fig. 1.57 Metanephric adenoma.<br />

Hyalinized scar and focal osseous metaplasia<br />

of the stroma are present in 10-<br />

20% of tumours {561}. Approximately<br />

50% of tumours contain papillary structures,<br />

usually consisting of tiny cysts into<br />

which protrude blunt papillae reminiscent<br />

of immature glomeruli. Psammoma<br />

bodies are common and sometimes<br />

numerous. The junction with the kidney is<br />

usually sharp and without a pseudocapsule.<br />

The cells of metanephric adenoma<br />

are monotonous, with small, uniform<br />

nuclei and absent or inconspicuous<br />

nucleoli. The nuclei are only a little larger<br />

than those of lymphocytes and are round<br />

or oval with delicate chromatin. The cytoplasm<br />

is scant and pale or light pink.<br />

Mitotic figures are absent or rare.<br />

Metanephric adenofibroma is a compos-<br />

A<br />

B<br />

Fig. 1.58 Metanephric adenoma. A Well circumscribed tumour without encapsulation. B Complicated ductal architecture with psammoma bodies.<br />

44 Tumours of the kidney

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