Showing papers by "Sabine Sarnacki published in 2008"

Laparoscopic Resection of Abdominal Neuroblastoma

Abstract: Background Since indications for laparoscopic adrenalectomy have progressively expanded to pediatric surgery, preliminary reports have studied the laparoscopic approach for abdominal neuroblastoma (NB). We aimed to report on the indications and the results of laparoscopic resection in a large series of abdominal NBs.

Pitfalls in the Diagnosis and Management of Obstructive Uterovaginal Duplication: A Series of 32 Cases

Abstract: Objectives Obstructive uterovaginal duplication is rare and frequently misdiagnosed. The aims of this study were to review all the patients managed for this malformation in our institution, evaluate their long-term outcomes, and discuss the embryologic origin of this malformation. Methods From 1984 to 2007, we treated 32 patients for obstructive uterovaginal duplication in our institution. We separated them in 2 groups (prepubertal [n = 8] and pubertal [n = 24]) and analyzed their clinical and radiologic presentations and their treatments. Patients >18 years of age (n = 22) were recontacted. Results For the prepubertal group, the median age at diagnosis was 6 months. Postnatal ultrasound showed an absent ipsilateral kidney in 6 case subjects, although 4 patients had a prenatal diagnosis of ipsilateral multicystic dysplastic kidney. This renal anomaly was associated with a pelvic sonolucent mass in 3 case subjects, allowing us to prenatally suspect the diagnosis. All of the patients were cured by vaginal approach. For the pubertal group, the median delay of diagnosis after menarche was 9 months. Among patients managed in an emergency setting (n = 11), there were 9 misdiagnoses with inappropriate abdominal surgeries, including 3 hysterectomies of the obstructed hemiuterus. Concerning long-term results, 5 patients were attempting to have children, with successful pregnancies for 4 of them. One patient suffered from infertility attributable to contralateral isthmic stenosis after a hysterectomy of the obstructed hemiuterus. Conclusions Obstructive uterovaginal duplication is a benign pathology when properly managed. Therefore, management of abdominal pain in peripubertal girls should include systematic abdominal and gynecologic examinations completed by radiologic pelvic and renal evaluation. Surgical treatment should be performed by vaginal approach to avoid infertility. Concerning the origin of the malformation, the high association of prenatal dysplastic kidneys and postnatal absent kidneys suggests anomalies of both wolffian and mullerian duct development, contrasting with the classic definition of this malformation.

Immature cryopreserved ovary restores puberty and fertility in mice without alteration of epigenetic marks.

Abstract: Background Progress in oncology could improve survival rate in children, but would probably lead to impaired fertility and puberty. In pre-pubertal girls, the only therapeutic option is the cryopreservation of one ovary. Three births have been reported after reimplantation of cryopreserved mature ovary. Conversely, reimplantation of ovary preserved before puberty (defined as immature ovary) has never been performed in humans. Methodology/Principal Findings In order to analyze ovarian function, we performed transplantation using fresh or cryopreserved immature grafts in pre-pubertal or adult mice. Puberty as well as cyclic hormonal activity was restored. All follicle populations were present although a significant reduction in follicle density was observed with or without cryopreservation. Although fertility was restored, the graft is of limited life span. Because ex vivo ovary manipulation and cryopreservation procedure, the status of genomic imprinting was investigated. Methylation status of the H19 and Lit1 Imprinting Control Regions in kidney, muscle and tongue of offsprings from grafted mice does not show significant alteration when compared to those of unoperated mice. Conclusions/Significance These results demonstrate that immature ovarian grafting can restore spontaneous puberty and fertility. However, these data suggest that follicle depletion leads to premature ovarian failure. This study addresses the very important epigenetics issue, and provides valuable information to the study of ovarian transplantation suggesting that these procedures do not perturb normal epigenetics marks. These results are highly relevant to the reimplantation question of immature cortex in women.

Preoperative Wilms tumor rupture: a retrospective study of 57 patients.

Abstract: BACKGROUND. According to current International Society of Pediatric Oncology (SIOP) Wilms recommendations, all preoperative tumor ruptures should be classified as stage IIIc. However, to the authors' knowledge, the definition and diagnostic criteria of preoperative rupture have not been defined clearly. METHODS. The authors performed a retrospective analysis of 57 children with clinical and/or radiologic (computed tomography [CT]) signs of preoperative tumor rupture of a series of 250 patients enrolled in Wilms SIOP protocols at their institution. RESULTS. Clinical and radiologic signs of preoperative rupture were observed in 39 patients and 55 patients, respectively. The site of rupture on imaging was retroperitoneal only in 48 patients and both retroperitoneal and intraperitoneal in 7 patients. Surgery was performed after chemotherapy in 55 of 57 patients. Peritoneal disease recurrence occurred in 3 of 57 patients, including 2 patients with stage III tumors who had initial intraperitoneal rupture and 1 patient with a stage I tumor. Among the 48 patients who had radiologic signs of retroperitoneal-only rupture, the final pathologic stage was stage III in 22 patients, stage II in 9 patients, and stage I in 17 patients, and no abdominal disease recurrence was observed, although only 23 of 48 patients received flank radiotherapy. The 5-year local control rate was significantly higher in patients who had retroperitoneal-only rupture compared with patients who had intraperitoneal rupture (100% vs 83.3%; standard error, ±15.2%; P = .0015). CONCLUSIONS. The use of CT scans significantly increased the number of patients who could be classified with “tumor rupture.” Intraperitoneal rupture was diagnosed accurately with CT and was associated with a significant risk of peritoneal disease recurrence. In contrast, patients who have radiologic signs of localized retroperitoneal-only rupture at diagnosis most likely should not be upstaged, and their treatment may be determined according to pathologic stage only. Cancer 2008. © 2008 American Cancer Society.

Transcription factors involved in pancreas development are expressed in paediatric solid pseudopapillary tumours.

Abstract: Aims: Solid pseudopapillary tumours (SPT) are rare pancreatic tumours, especially in children. The origin of this benign tumour remains unknown. Mutations of β-catenin, a gene essential for pancreatic development, are constantly found, leading to delocalization of immunohistochemical signals from the cytoplasm to the nuclei of tumour cells. The aim was to report clinical and histological data of eight children with SPT and explore the immunohistochemical expression of pancreatic duodenal homeobox (PDX) 1 and Sox9, known to be crucial for pancreatic development and linked to the β-catenin cascade. Methods and results: Eight children with features suggestive of SPT underwent surgical resection. Tumours displayed typical histological appearances. One was incompletely resected and recurred. Immunolabelling revealed nuclear location of β-catenin in all cases and strong cytoplasmic but no nuclear expression of PDX1 or Sox9 in all but one case. Conclusions: The clinical behaviour of SPT in the paediatric population is similar to its adult counterpart. Complete surgical resection is essential. PDX1 and Sox9 proteins are exclusively expressed in the cytoplasmic compartment in SPT, suggesting overexpression of the corresponding genes linked to β-catenin mutations. These findings favour the hypothesis that SPT originates from transformation of normally quiescent pancreatic stem cells.

Prenatal diagnosis of dumbbell neuroblastoma.

Abstract: A neuroblastoma that develops in the sympathetic nodes can infiltrate the intervertebral foramina and invade the spinal canal, leading to spinal cord and nerve root compression and neurological impairment. Dumbbell neuroblastomas are now considered to be unresectable tumors and preoperative chemotherapy is recommended. We report the prenatal diagnosis of a dumbbell neuroblastoma successfully managed through premature delivery followed by immediate chemotherapy. We suggest that delivering prematurely in such cases is only of benefit if chemotherapy can be administered under favorable conditions. Chemotherapy should proceed immediately after delivery in order to reduce the size of the tumoral mass and its effects on the spine.

SFCP-030 – Chirurgie viscérale – Volvulus du grêle de diagnostic périnatal : deux entités, deux pronostics

Abstract: Objectif Les volvulus du grele de diagnostic perinatal peuvent etre separes en deux entites anatomiques distinctes : les volvulus complets sur anomalie de la rotation mesenterique et les volvulus segmentaires. Nous avons revu 22 cas de volvulus de diagnostic perinatal et compare l’evolution en fonction du type anatomique. Materiel et methode Dix presentaient un volvulus segmentaire (VS) et 12 un volvulus complet sur anomalie de la rotation mesenterique (VC). Pour ces deux groupes, les conditions du diagnostic, le type de l’intervention chirurgicale et l’evolution post operatoire ont ete compares (test non parametrique de Wilcoxon). Resultats Le diagnostic a ete realise en antenatal dans un cas dans le groupe VC et dans 9 cas dans le groupe VS. Le terme moyen de naissance etait de 39 SA et de 36 SA respectivement. L’echographie realisee en post natal chez 9 patients du groupe VC retrouvait une image typique (whirlpool) alors qu’elle n’apportait aucun argument en faveur du volvulus chez les 5 enfants du groupe VS ayant eu cet examen. Les interventions ont ete realisees a une mediane de 6 jours dans le groupe VC (0 – 23 jours) et toujours dans les premieres 24 heures pour le groupe VS (p = 0,002). Dans les cas du VC il n’a pas ete retrouve de souffrance digestive majeure et aucune resection intestinale n’a ete realisee. Dans le groupe VS, on notait 4 cas de necroses segmentaires et 5 peritonites et tous ont eu une resection digestive (12,5 cm en moyenne) avec la realisation d’une ileostomie dans 3 cas. La mediane de reprise du transit a ete de 2 jours dans le groupe VC, sans nutrition parenterale, alors qu’elle etait de 7 jours (2 – 17 jours) dans le groupe VS (p Conclusion Malgre une resection intestinale limitee, le pronostic du VS apparait ici plus pejoratif que celui du VC. L’existence d’un obstacle digestif evoluant depuis la periode antenatale dans les VS pourrait induire une souffrance du systeme nerveux enterique qui n’est pas observe dans les VC de survenue neonatale, ou l’obstacle est de survenue recente. Cette notion doit etre prise en compte dans l’information qui est faite aux familles.