[Results of open radical nephrectomy in a series of 230 patients]. / Résultats de la néphrectomie élargie à ciel ouvert: à propos d'une série de 230 patients.

OBJECTIVE: To analyse the oncological results of open radical nephrectomy for renal cancer PATIENTS AND METHODS: Between December 1989 and June 2003, open radical nephrectomy for renal cancer was performed by the same operator in 230 patients. Cancers treated by partial nephrectomy were excluded from this study. The operation was performed via a lumbar incision in 95.7% of cases. Surveillance consisted of clinical examination and thoracic and abdominopelvic computed tomography twice a year for the first two years, then annually for 3 years, and then at 7 years and at 10 years in the absence of recurrence. RESULTS: With a median follow-up of 63.1 months, the 5-year and 10-year recurrence-free survival rates were 88.4% and 67.2%, respectively. Tumour size, tumour stage (TNM 1997), Fuhrman's nuclear grade, histological type of cancer, and the presence of suggestive signs were correlated with the recurrence-free survival rate. The 10-year recurrence-free survival rate was zero in patients with visceral or lymph node metastases at the time of diagnosis. The patient's age at the time of diagnosis and the presence of a paraneoplastic syndrome did not influence recurrence free survival in this series. CONCLUSIONS: This study confirms the data of the literature: tumour size, tumour stage, nuclear grade, histological type, and suggestive signs are prognostic factors of renal cancer. Radical nephrectomy ensures a global 5-year recurrence-free survival rate higher than 85%.

Progressive neuronal degeneration of childhood: prenatal diagnosis by MRI.

We report two cases in the same family of progressive neuronal degeneration of childhood--Alpers syndrome--with prenatal MRI findings in one case. The first infant presented at birth with severe microcephaly, then rapidly evolved to progressive encephalopathy with refractory epilepsy, leading to death at 10 months. Biochemical investigations including liver function tests were normal. CT and MRI showed severe diffuse brain atrophy. The diagnosis of progressive neuronal degeneration of childhood was made on the clinical and imaging data. The second pregnancy was marked by gradual decrease of fetal cerebral biometry and a prenatal MRI performed at 32 weeks showed diffuse cortical atrophy, as observed in the sibling. The infant died at 5 months. Neuropathological findings were consistent with Alpers syndrome.