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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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Osteosarcoma<br />

L. Guillou<br />

Definition<br />

A malignant mesenchymal tumour showing<br />

osteoid production.<br />

ICD-O code 9180/3<br />

Epidemiology<br />

Most osteosarcomas of the urinary bladder<br />

occurred in male patients (male to<br />

female ratio: 4:1), with an average age of<br />

60-65 years {215,863,2900}.<br />

Etiology<br />

One case of bladder osteosarcoma<br />

occurred 27 years after radiation therapy<br />

for urothelial carcinoma {754}. A few<br />

patients had concurrent urinary schistosomiasis<br />

{2900}.<br />

Localization<br />

Most osteosarcomas occurred in the urinary<br />

bladder, especially in the trigone<br />

region {2900}. Anecdotal cases have<br />

been reported in the renal pelvis {655}.<br />

Clinical features<br />

Haematuria, dysuria, urinary frequency,<br />

and recurrent urinary tract infections are<br />

the most common presenting symptoms.<br />

Pelvic pain and/or palpable abdominal<br />

mass are less frequent.<br />

Macroscopy<br />

Osteosarcoma of the urinary bladder typically<br />

presents as a solitary, large, polypoid,<br />

gritty, often deeply invasive, variably<br />

haemorrhagic mass. Tumour size<br />

varies between 2 and 15 cm (median: 6.5<br />

cm) {215,863,2900}.<br />

Fig. 2.78 Osteosarcoma of the urinary bladder. Abundant trabeculae of neoplastic bone surrounded by a<br />

malignant spindle cell component.<br />

Histopathology<br />

Histologically, the tumour is a high grade,<br />

bone-producing sarcoma. Foci of chondrosarcomatous<br />

differentiation and/or<br />

spindle cell areas may also be observed<br />

{215,2900}. Variably calcified, woven<br />

bone lamellae are rimmed by malignant<br />

cells showing obvious cytologic atypia<br />

(as opposed to stromal osseous metaplasia<br />

occurring in some urothelial carcinomas<br />

{655}). A recognizable malignant<br />

epithelial component should be absent,<br />

allowing discrimination from sarcomatoid<br />

carcinoma {2057}, which is the most<br />

important differential diagnosis.<br />

Prognosis<br />

Osteosarcoma of the urinary tract is an<br />

aggressive tumour with poor prognosis.<br />

A majority of patients have advanced<br />

stage (pT2 or higher) disease at presentation<br />

and die of tumour within 6 months,<br />

most from the effects of local spread (urinary<br />

obstruction, uremia, secondary<br />

infection, etc.) {863,2900}. Metastases<br />

often occurred late in the course of the<br />

disease, mainly in lungs {215,2900}. The<br />

stage of the disease at diagnosis is the<br />

best predictor of survival.<br />

142 Tumours of the urinary system

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