Showing papers by "Sabine Sarnacki published in 2002"

Outcome of suprarenal localized masses diagnosed during the perinatal period

Abstract: BACKGROUND The growing use of abdominal ultrasonography during pregnancy and in the postnatal period is leading to the discovery of an increasing number of suprarenal masses. The optimal diagnosis and treatment of these masses has not yet been determined. METHODS The authors reviewed the files of patients with suprarenal masses detected prenatally or during the first 3 months of life, between 1986 and 1999, in the pediatric surgery and oncology departments of Paris hospitals. RESULTS Thirty masses were detected prenatally and 23 postnatally. In the latter group of patients, the diagnosis was based on ultrasound in 8 cases and on the palpation of a mass in 15 cases, 13 of which were neuroblastoma. At birth, the masses were cystic in 19 cases, solid in 17, and mixed in 13. Sensitivities of methyliodobenzylguanidine and urinary catecholamine assay were 70% and 52% respectively. Surgery was performed in 38 cases with a median age of 42 days. Histologic analysis showed 31 neuroblastomas, 1 adrenal hemorrhage, 2 necrotic masses, 1 bronchogenic cyst, and 3 sequestrations. All the patients were alive and disease free, at a follow-up ranging from 3 months to 13 years. CONCLUSIONS In this series, 58% of the suprarenal masses diagnosed perinatally were localized neuroblastoma with a favorable outcome. All other cases either regressed spontaneously or turned out to be benign lesion. Thus, the management of these masses must strike a compromise between aggressive treatment and a wait-and-see attitude. This requires appropriate initial assessment and a close follow-up in a specialized center. Cancer 2002;94:2474–80. © 2002 American Cancer Society. DOI 10.1002/cncr.10502

Changes in WT1 splicing are associated with a specific gene expression profile in Wilms' tumour

Abstract: Wilms' tumour (WT) or nephroblastoma is the most frequent kidney cancer in children. In a previous study, we reported alterations to WT1 transcription in 90% of WT tested, with decreased exon 5 +/- isoform ratio being the most frequent alteration (56% of WT). We now report an approach based on cDNA profiling of tumour pools to identify genes likely to be dysregulated in association with a decreased WT1 exon 5 +/- ratio. We compared the expression profiles of pools of tumours classified according to whether this isoform imbalance was present (five tumours) or not (four tumours), using Atlas Cancer cDNA expression arrays. Fourteen of 588 genes tested displayed specific up-regulation (CCND2, PCNA, N-MYC, E2F3, TOP2A, PAK1, DCC and PCDH2) or down-regulation (VEGF, IGFBP5, TIMP3, ARHB, C-FOS and CD9) in the pool of tumours with decreased exon 5 +/- ratio. These results were validated by RT-PCR analysis of four genes (CCND2, PCNA, VEGF and IGFBP5). We extended the analysis of VEGF expression to 51 tumours by real-time RT-PCR and ascertained differential expression of this gene associated with WT1 expression pattern. Moreover, our results suggest that the VEGF expression level may be of prognosis relevance for relapsed patients.

Indications and results of surgery in patients with Crohn's disease with onset under 10 years of age: a series of 18 patients.

Abstract: Dans le but d'etudier le mode de presentation et l'evolution de la maladie de Crohn (CD) avec un debut avant l'âge de 10 ans et d'apprecier les indications et les effets de la chirurgie, une etude retrospective a ete realisee chez 18 enfants. Cent vingt-deux enfants avec CD ont ete traites pendant une periode de 22 ans dans notre institution. Trente ont eu le premier signe de la maladie avant l'âge de 10 ans. Dix-huit parmi les trente ont eu une resection intestinale (IR). L'âge moyen au debut de CD etait de 6.4 ans et la duree moyenne du suivi etait de 11.1 ans. Les symptomes les plus communs etaient de douleurs abdominales (72%), des diarrhees (72%) et des retards de croissance (50%). Le colon et l'ileon etaient le plus frequemment concernes. La plupart de ces enfants (94,5%) recevaient un traitement medical intensif et/ou un traitement nutritionnel avant la chirurgie. Nous avons realise 26 resections intestinales et 14 autres operations chez ces 18 patients. L'indication pour IR etait l'obstruction intestinale chronique (13 cas) ou un non fonctionnement intestinal chronique (13 cas). Une amelioration de l'etat clinique etait obtenue chez 17 des 10 cas. IR est benefique chez les enfants pour lesquels le traitement medical est inefficace. Un intervalle libre apres la chirurgie peut entrainer un moment important pour ameliorer la croissance, le developpement de la puberte et pour retourner a une vie presque normale pour un long moment.