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Biomedical subjects

D Sommelet

Publications and source records attributed to D Sommelet.

At least 55 records · Page 3Linked to original sources
PubMed1998

[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↗
PubMed1997

An excess of testicular germ cell tumors in Down's syndrome: three case reports and a review of the literature.

BACKGROUND: The incidence of specific solid tumors in Down's syndrome (DS) is not well established. Testicular germ cell tumors (TGCT) might be increased in this population. METHODS: The presence of TGCT among male subjects from the French department of Corrèze was recorded and literature on the subject reviewed. RESULTS: A total of 120 living children and adults with DS and 17 pregnancies (12 births and 5 therapeutic abortions) were examined over an 8-year period (1987-1994). Three TGCT were diagnosed. A seminoma and an embryonal carcinoma were observed in two young adults and an intratubular germ cell neoplasm in a 22-week-old fetus. Because testicular tumors occur at an incidence rate of 4 cases per 100,000 person-years in the general population, these observations suggest a clearly increased risk of developing TGCT in the DS population. In addition, a review of the literature also shows an excess of TGCT in this population. Cryptorchidism alone, which is prevalent in individuals with DS, cannot explain this significantly increased incidence of TCGT. The authors hypothesize that an excess of luteinizing hormone and follicle-stimulating hormone gonadotropins and overexpression of the Ets-2 gene through gene dosage effect could predispose patients with DS to the development of TGCT. CONCLUSIONS: Surveillance of the gonads of male patients with DS is recommended. A better understanding of the factors involved could also help to identify risk factors in the general population.

Adult↗
PubMed1997

Antioxidant micronutrients and childhood malignancy during oncological treatment.

Serum antioxidant vitamins A (retinol) and E (alpha-tocopherol), beta-carotene, zinc, and selenium, and cholesterol and related proteins for 170 children with newly diagnosed malignancy were measured at diagnosis and 6 months after initiation of treatment, and compared with those of 632 cancer-free controls. Incident cancer cases and controls were 1-16 years old and recruited between 1986 and 1989. At diagnosis, age- and sex-adjusted serum concentrations of retinol, beta-carotene, zinc, and alpha-tocopherol were significantly inversely associated with cancer. No significant decreases in mean values were observed at 6 month, except for the alpha-tocopherol-to-cholesterol ratio in patients with bone tumors and serum zinc in bone tumors and central nervous system malignancies. An increase during the period of treatment was found for retinol and selenium in leukemia patients. beta-carotene was maintained at the initial concentrations determined prior to therapy. These findings provide further information about micronutrient requirements in children with cancer.

Adolescent↗
PubMed1997

[Myelodysplastic syndromes in children, Groupe d'étude des myélodysplasies de l'enfant de la Société d'hématologie et d'immunologie pédiatrique (SHIP)].

Myelodysplastic syndromes (MDS) in children constitute a heterogeneous disorder, including 'primary' MDS and MDS associated with constitutional abnormalities. The Franco-American-British (FAB) cytological classification for adults can be applied for childhood in 50 to 100% of the cases. The transformation into acute myeloblastic leukemia often occurs, but stabilisation or spontaneous regression of the disease may also be observed. The therapeutic decision is difficult because there is no predictive factor of the course of the disease. Allogenic bone marrow transplantation is the best curative option when treatment is necessary.

Child↗
PubMed1997

Loss of chromosome 1p may have a prognostic value in localised neuroblastoma: results of the French NBL 90 Study. Neuroblastoma Study Group of the Société Française d'Oncologie Pédiatrique (SFOP).

Between March 1990 and December 1994, 316 consecutive children with localised neuroblastoma were registered in the French NBL 90 study. In addition to the assessment of a new chemotherapy regimen in unresectable neuroblastoma, we evaluated the prognostic significance of MYCN amplification and loss of the short arm of chromosome 1 (LOH1p). MYCN was found in 22/225 children (10%) and associated with unfavourable clinical features such as age at diagnosis > 1 year and large and unresectable tumours. LOH1p was observed in 9/91 patients (10%), of whom some had favourable prognostic factors such as age at diagnosis < 1 year (n = 4), INSS stage 1 or 2 (n = 3) and no MYCN amplification (n = 4). Overall survival (OS) and event-free survival (EFS) were, respectively, 56% and 22% (median follow-up: 36 months) for children with LOH1p compared with 97% and 94% for those without (log-rank = 10(-8)). All except 1 of the 5 children with MYCN amplification and LOH1p relapsed and ultimately died of the disease. Among the 4 with LOH1p and no MYCN amplification, recurrence occurred in 3 (2 local, 1 metastatic), all alive in second remission after salvage therapy (12-19 months after the relapse). In multivariate analysis, LOH1p was the strongest prognostic indicator for subsequent relapse. LOH1p appears more discriminant than MYCN amplification for predicting the risk of recurrence in children with localised neuroblastoma. However, its analysis was possible in only 30% of our patients and its final impact on survival should be confirmed in larger, prospective studies in order to stratify subsequent treatment.

Abdominal Neoplasms↗
PubMed1997

N-Myc gene amplification is a major prognostic factor in localized neuroblastoma: results of the French NBL 90 study. Neuroblastoma Study Group of the Société Francaise d'Oncologie Pédiatrique.

PURPOSE: To assess the relevance of N-Myc gene amplification (NMA) as a prognostic factor in localized neuroblastoma (NB) and to evaluate whether less intensive adjuvant treatment is advisable in infants without NMA. PATIENTS AND METHODS: Assessment of NBs included clinical and imaging data to allow tumor-node-metastasis (TNM) staging, biologic determinations (N-Myc gene analysis), and standard histology and work-up to eliminate metastatic spread (metaiodobenzylguanidine [MIBG] scintigraphy and extensive bone marrow staging). Resectability was defined according to imaging findings. Chemotherapy was indicated in children older than 1 year at diagnosis who had postoperative residual disease or lymph node (LN) involvement, in infants with NMA, or as primary treatment in children with an unresectable NB, including dumbbell tumors. Radiotherapy was recommended in children older than 1 who presented with persistent gross residual disease at the end of therapy. RESULTS: Between 1990 and 1994, 316 consecutive children who presented with a localized NB were registered in the NBL 90 study. The median age was 12 months, and 42 patients had dumbbell tumors (13%). NMA was found in 22 of 225 assessable children (10%) and correlated with adverse prognostic indicators such as age older than 1 year, an abdominal primary tumor, a large tumor (T3), and unresectability. Among 186 children who had primary excision, five died of surgery-related complications. Primary chemotherapy was given to 130 patients, which allowed removal of the tumor in all but four. The 5-year overall survival (OS) and event-free survival (EFS) rates were, respectively, 91% and 84% with a median follow-up time of 36 months. The outcome of infants and older children was similar (P = .2). EFS of patients with resectable tumors was slightly better than with unresectable primary tumors (EFS, 89% v 78%; P = .02). In dumbbell NBs, neurologic recovery was achieved in 74% of cases that presented with symptoms, and initial laminectomy was avoided in 75% of children. In a univariate analysis, large tumors, high neuron-specific enolase (NSE) and lactate dehydrogenase (LDH) levels, positive LNs, macroscopic residue, and NMA adversely influenced outcome. In the multivariate analysis, NMA was the most powerful unfavorable predictive indicator: OS and EFS rates for these children were 36% and 32%, compared with 98% and 90% in nonamplified tumors (P < .001). CONCLUSION: Our data confirm the overall good prognosis of localized NBs, even when unresectable. NMA is the most relevant adverse prognostic factor in localized NBs, and more intensive treatment should be investigated in these patients. Prospective studies of other biologic factors are warranted to tailor therapy more accurately. The EFS of children who underwent primary surgery was excellent, and further justifies elimination of adjuvant treatment provided they have no NMA. Despite the elimination of postoperative therapy, infants with non-NMA tumors have an excellent outcome, which suggests that initial chemotherapy can be further reduced in case of unresectable NBs.

Adolescent↗
PubMed1997

Assessment of serum antioxidant micronutrients and biochemical indicators of nutritional status in children with cancer in search of prognostic factors.

Serum vitamins A (retinol) and E (alpha-tocopherol), beta-carotene, zinc and selenium, and related proteins for 157 children with newly diagnosed and histologically proven malignancy were compared with those of 632 cancer-free controls. Incident cancer cases and controls were 1-16 years of age and recruited in 1986-1989. Age and sex-adjusted serum concentrations of beta-carotene and alpha-tocopherol were significantly inversely associated with cancer. No significant association with cancer was observed for serum values of selenium. Although low levels of antioxidants might in part be involved through a causality link, the reported decreased peripheral nutrient levels are considered rather as an impairment of the body's defence system, occurring during the cancer-related metabolic and nutritional disturbances and inflammation processes. The cancer cases group was followed-up and examined 6 months after diagnosis. Among the 157 subjects, 24 had died and 133 were reported to be alive. No substantial difference for any antioxidant or chemistry variable at onset was observed as a function of clinical outcome and health status.

Adolescent↗
PubMed1997

[Pediatric joint ultrasonography. Aspects of synovial pathology].

Until recently, few articular indications for sonographic imaging have been widely accepted. However, concerns about imaging costs are expanding its role in musculo-skeletal pathology. Valuable diagnosis insight are readily obtained with recently available high frequency transducers. Ultrasound may be utilised following clinical examination as an efficient tool in the diagnosis and follow-up of various synovial disorders. Dynamic studies of articular structures as they move, is a significant advantage over other diagnosis modalities. Ultrasound has proved to be useful in daily practice but still requires protracted learning curve for the operator before he becomes proficient with this method.

Child↗
PubMed1997

[An exceptional variety of medulloblastoma: melanotic medulloblastoma].

We report a new case of melanotic medulloblastoma of the vermis in a 3 1/2 year old boy. This tumor showed a typical histological appearance with pseudoepithelial pigmented structures immunoreactive for S100 protein and vimentin. The tumor did not recur after total surgical removal and post operative radiation. However, after a 10 year follow-up, imaging demonstrated that a second tumor occurred in the left cerebellar hemisphere, which, on histological examination, was a typical glioblastoma. Hypothesis concerning the histogenesis of the second tumor, as well as a causal association with radiation therapy and possible contribution of growth hormone therapy are discussed.

Cerebellar Neoplasms↗
PubMed1995

[Favorable outcome of orbital nasal sinus mucormycosis complicating the induction treatment of acute lymphoblastic leukemia].

BACKGROUND: Most cases of mucormycosis occur in immunosuppressed children. Intracranial extension is lethal and must be prevented with early specific treatment. CASE REPORT: A 42 month-old boy was admitted suffering from acute lymphoblastic leukemia. Edema of the left eyelid developed on the sixth day of induction chemotherapy. Mucormycosis was suspected because of gradual extension of infection to nasal ala and periorbital area with fever, edema of nasal turbinates and nasal black secretions. Chemotherapy was discontinued and the patient was given intravenous amphotericin B (1.0 mg/kg/day) and heparin associated with G.CSF. Improvement was only temporary and scan examination performed on day 17 showed involvement of the orbit, eye and wall of the maxillary sinus; cultures of secretions were positive for staphylococcus and Absidia corymbifera. Remission of leukemia was obtained a few days later permitting surgical resection of involved tissues on day 30. A relapse of mucormycosis was observed six weeks later despite prolonged administration of amphotericin B requiring extended resection of necrotic areas and replacement of amphotericin B by its liposomal form (Ambisome). Bone marrow relapse of leukemia required further chemotherapy. The patient is in good condition 30 months after the initial symptoms. CONCLUSION: Our patient seems to be the first with prolonged remission of facial mucormycosis and acute leukemia despite relapse of both diseases. This favorable outcome could be due to the use of Ambisome.

Amphotericin B↗
PubMed1995

[Inflammatory myofibroblastic tumor of the lung with endobronchial, infiltrating, multifocal and recurrent form].

Pulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor, plasma cell granuloma) was reported most often as a single peripheral mass, successfully cured by surgery. A 14-year-old girl presented with a large left pulmonary mass involving and obliterating the main bronchus; there were angioinvasion and infiltration of mediastinum, hilar lymph nodes and pleura. Multiple, often tiny nodules were seen in the right lung. At microscopic examination, there were lymphocytic and plasmacytic infiltrates and borderline myofibroblastic proliferation with focal nuclear anaplasia. Smaller lesions were similar to organizing pneumonia. Disease was progressive in the remaining right lung after surgical resection and a two-month treatment with corticoids. The patient was then treated with chemotherapy. She was alive and well (twenty-month follow-up).

Adolescent↗
PubMed1994

Childhood anaplastic large cell lymphoma Ki-1/CD30: clinicopathologic features of 19 cases.

The authors report their cumulative experience of 19 children with what was previously called malignant histiocytosis (MH) but is now considered a true lymphoma and termed anaplastic large cell lymphoma (ALCL). The median age at diagnosis was 10 years and 6 months (range 2 y, 11 m, to 15 y). There were 13 males and 6 females. Most cases presented with fever, wasting and enlarged, often tender, lymph nodes. Other features were: fleeting cutaneous rashes in 7 cases; spontaneous regression of lymph nodes and skin lesions were seen in 5 patients. Bone marrow involvement was present in 3 cases, pulmonary infiltrate in 5, kidneys in 2, and central nervous system in none. The morphology of lymph node involvement was consistent with so-called MH, a description originally applied to sinusoïdal infiltration by large "histiocytic" cells. The coexpression of lymphoid activation antigens Ki-1/CD 30 (18/19), epithelial membrane antigen EMA (18/19) and interleukin-2 receptor/CD 25 (10/10) was the unifying immunopathologic feature of the neoplasm. Lineage antigens were not identifiable in 8/19 instances (null phenotype), while 10/19 expressed a T-cell phenotype. None of the tumors expressed histiocytic markers. After variable, but intensive, combination chemotherapy, 15 children out of 18 evaluable achieved complete remission (CR). Among all patients, thirteen are still alive in CR (ten in first CR) with a median follow-up of 5 years. This evaluation in the pediatric age group reinforces that so-called MH is a lymphoid neoplasm, a conceptual change that could lead to improved understanding and therapy.

Adolescent↗