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Biomedical subjects

D Ranchere-Vince

Publications and source records attributed to D Ranchere-Vince.

10 recordsLinked to original sources

[Inflammatory myofibroblastic pseudotumor of the tongue].

INTRODUCTION: Inflammatory myofibroblastic tumor (IMT) is a new entity recently described, first in the lung. IMT are found throughout the body. CASE: We report a very rare tongue localization in a 22-year-old white woman. After a first diagnosis of fibrosarcoma, a new examination of the slide and immunohistochemical examination led to the definitive diagnosis of inflammatory myofibroblastic tumor of the tongue and not fibrosarcoma. DISCUSSION: IMT are rarely encountered in the head and neck region. Only 2 cases of IMT of the tongue have been reported in the world literature. Diagnosis of IMT is immunohistochemical. Spindle cells were reacted for smooth-muscle actin and vimentin. Treatment is surgical. The contribution of radiotherapy is not clear because of the small population of patients studied.

Actins↗

Does nephroblastomatosis influence the natural history and relapse rate in Wilms' tumour? A single centre experience over 11 years.

The presence of multifocal or diffuse nephrogenic rests (NRs) in one or both kidneys is termed nephroblastomatosis (Nbm). Nbm may be a predisposing factor for Wilms' tumour (WT). The aim of this retrospective study was to evaluate the impact of Nbm on the outcome of WT in children. We assessed the outcome of 81 children with Wilms tumours and practical implications of Nbm in the treatment and follow-up. All the pathology slides have been reviewed in 1997. 63 had WT without Nbm (group A) and 18 had WT associated with Nbm (group B). There was no statistical difference between the two groups according to the age at diagnosis and histology. Clinical abnormalities were more frequent in group B (33 versus 8%). There was no statistical difference between the percentage of stage IV in both groups, but bilaterality (stage V) was present only in the group B. Relapse was observed in 20/81 patients (25%): 11 (17%) in group A and 9 (50%) in group B. Mean delay of relapse was longer (25 months) in group B than in group A (10 months). For the whole population, with a median follow-up of 9 years, the event-free survival (EFS) and the overall survival (OS) probabilities were respectively 74%+/-10 and 83%+/-9 at 120 months. The difference in EFS between groups A (82+/-9%) and B (38%+/-29) was significant (P=0.004). The discovery of Nbm in the non-tumoral part of the kidney with WT can be an adverse factor and in particular favours the subsequent development of a new Wilms tumour. It justifies separate follow-up guidelines.

Adolescent↗

[Solid pseudopapillary tumors of the pancreas. Pathology report of 13 cases].

OBJECTIVES: Solid pseudopapillary tumors of the pancreas are exceptional. The aims of our study were to reevaluate the mode of presentation of these tumors and to analyze the role of pathological examination in diagnostic assessment and prognostic evaluation. PATIENTS: We report the clinical, radiological and pathological findings in a retrospective series of 13 patients in whom a diagnosis of solid pseudopapillary tumor of the pancreas was made between 1983 and 1997. There were 12 females (median age: 22.5 years) and one male, aged 73. RESULTS: The tumor was discovered incidentally (3 cases) or because of nonspecific digestive symptoms (10 cases). Biological data were uninformative. The tumor was pancreatic in 12 cases and duodenal in 1. In all cases, imaging techniques showed an heterogeneous lesion with no or poor vascularization. A cystic component was identified in 4 cases. Surgical resection was performed in all cases. Pathological examination showed an encapsulated tumor in 8 cases, a non-encapsulated but well-limited lesion in 3 cases and an infiltrative tumor in 2 cases. At the time of diagnosis, multiple liver metastases were present in 1 case. Mean duration of follow-up was 24 months (range: 3-168). At last follow-up, all patients, including the patient with synchronous metastatic disease, were alive, without local recurrence. CONCLUSION: Our study confirms that most cases of solid pseudopapillary tumors of the pancreas present with a suggestive clinical picture, including their occurrence in young women and their good prognosis after surgical resection. However, our results also underline the occurrence of cases presenting with unusual features, including old age, male sex, extra-pancreatic localization and malignant evolution. Histopathological examination is essential for the establishment of the diagnosis but morphological data are of little prognostic value.

Adolescent↗

Structure of the supernumerary ring and giant rod chromosomes in adipose tissue tumors.

Supernumerary ring or giant rod marker chromosomes are a characteristic of well-differentiated liposarcomas (WDLPS) and atypical lipomas (ALP) and are often observed as the sole cytogenetic abnormality, but are rare in lipomas. Using a combination of different methods, we extensively investigated the structure and composition of rings and giant rods in a series of 17 WDLPS-ALP samples and three intra- or intermuscular lipomas (IMLP), revealing a unique combination of particular features strikingly related to these tumors. Although the rings and rods displayed in vitro and in vivo stability, the presence of alpha-satellites could not be detected on these supernumerary structures. Comparative genomic hybridization analysis, in combination with fluorescence in situ hybridization, identified the chromosomal regions contributing to the formation of these chromosomes: in WDLPS-ALP, all carried amplifications of 12q 14-15 and the MDM2 gene, with variable other noncontiguous regions. In the three IMLP, the rings consistently carried amplifications of 12q15-21 and 1q21, but increased copies of MDM2 were found in only one case. Other genes located more proximal in 12q14-15 were amplified in several WDLPS-ALP, but showed a normal copy number in IMLP. Furthermore, the immunohistochemical expression of the MDM2 protein was detected in most (12/14) WDLPS-ALP, in 1-30% of the cells, but never in IMLP. These supernumerary chromosomes represent a peculiar kind of amplification structure, midway between double minute chromosomes and homogeneously staining regions, but the mechanisms underlying the formation of these structures remain obscure.

Aged↗

Rhabdoid tumor of the kidney associated with a tumor of the posterior fossa.

We report a case of a malignant rhabdoid tumor of the kidney (MRTK) associated with a cerebellar tumor. Diagnosis was confirmed before neoadjuvant chemotherapy by a percutaneous fine-needle biopsy of the abdominal tumor. The clinical and radiologic features of this rare association of childhood neoplasms are reviewed.

Biopsy, Needle↗

[Standards, options and recommendations (SOR) for clinical care of rhabdomyosarcoma (RMS) and other soft tissue sarcoma in children. Federation of the French Cancer Centers. French Society of Pediatric Oncology].

CONTEXT: The "Standards, Options and Recommendations" (SOR) project, started in 1993, is a collaboration between the Federation of the French Cancer Centres (FNCLCC), the 20 French Cancer Centres and specialists from French Public Universities, General Hospitals and Private Clinics. For pediatric issues, this project is a collaboration between the FNCLCC and the French Society of Pediatric Oncology (SFOP). The main objective is the development of clinical practice guidelines to improve the quality of health care and outcomes for cancer patients. The methodology is based on literature review and critical appraisal by a multidisciplinary group of experts, with feedback from specialists in cancer care delivery. OBJECTIVES: To develop a clinical practice guideline according to the definitions of Standards, Options and Recommendations for the clinical care of rhabdomyosarcoma and other soft tissue sarcoma in children and adolescents. METHODS: Data have been identified by literature search using Medline (1985-may 1997) and experts group personal references lists. The main criteria considered were incidence, risk factors, prognostic factors and efficacy of cancer treatment. Once the guideline was defined, the document was submitted for review to 14 national and international independent reviewers, and to the medical committees of the 20 French Cancer Centres and, in particular the 4 which have expertise in pediatric cancer management, for agreement. RESULTS: The main recommendations for rhabdomyosarcoma management are: 1/ diagnosis is based on appropriate clinical and radiological findings; 2/ pathological and immunohistochemical studies are essential to confirm the diagnosis; 3/ surgery must be performed by an experienced surgeon. Surgery and radiotherapy must be as conservative as possible; 4/ therapeutic strategies for rhabdomyosarcoma depend on location and extends and are based on chemotherapy, surgery and radiotherapy. Inclusion of patients in SFOP, SIOP and IRS clinical trials is recommended; 5/ treatment of metastatic rhabdomyosarcoma is based on intensive chemotherapy, and surgery with or without radiotherapy; 6/ the management of non-rhabdomyosarcoma is based on the likelihood of sensitivity to chemotherapy; 7/ at the present time, there are no clear data on which to base guidelines for timing and duration of follow-up studies in these conditions.

Child↗

False positive MIBG scan.

The fortuitous discovery of a left posterior cervico-mediastinal mass during hospitalization for hemolytic anemia in an 18 month old baby is reported. Metaiodobenzylguanidine (MIBG) uptake suggested a sympathetic tissue origin of the mass despite negative catecholamines excretion, but histopathologic examination revealed a juvenile capillary angioma within a normal sympathic ganglion.

3-Iodobenzylguanidine↗