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Biomedical subjects

F Flamant

Publications and source records attributed to F Flamant.

At least 55 records · Page 3Linked to original sources
PubMed1992

Packaging cells for avian leukosis virus-based vectors with various host ranges.

Using our previously described Haydée semipackaging cell line (F. L. Cosset, C. Legras, Y. Chebloune, P. Savatier, P. Thoraval, J. L. Thomas, J. Samarut, V. M. Nigon, and G. Verdier, J. Virol. 64:1070-1078, 1990) which produces avian leukosis virus gag and pol proteins, we have constructed packaging cells with subgroups B, C, and E envelope specificities. This allows us to produce helper-free avian leukosis virus particles carrying the lacZ reporter gene and the A, B, C, or E subgroup specificities. Titers of the recombinant lacZ virus are shown to be dependent upon the type of the env subgroup and the target avian cell.

Animals↗
PubMed1992

Identifying genes involved in the variability of genetic fatness in the growing chicken.

A precise knowledge of the genome involved in the expression of a quantitative trait could provide a useful tool in breeding programs; molecular genetic methods are capable of yielding this kind of information. An experimental procedure is presented here for identifying genes whose expression is related to weight variability of abdominal adipose tissue in the growing chicken. Quantitative traits are the result of metabolic pathways exhibiting some major regulation stages that are controlled genetically. These steps involve genes that may act as "major genes". With regard to chicken fat metabolism, most fatty acids are synthesized in the liver and incorporated into very low density lipoprotein (VLDL) particles before their secretion into the plasma. Accordingly, the present study focused on the expression of liver genes. The mRNA of lipogenic enzymes (acetyl-coenzyme-A carboxylase, fatty acid synthase, malic enzyme, and delta 9-desaturase) were analyzed. Also studied were apoprotein (apo)A1, apoVLDL-II, and apoB mRNA from 9-wk-old male chickens from two lines selected for high and low abdominal fat pads. Significant differences for apoA1 mRNA levels occurred between fat and lean birds. Moreover, the total quantity of mRNA provided an accurate estimation of the abdominal fat pad (r = .74 with P < .05).

Adipose Tissue↗
PubMed1992

[Granulosa cell tumors of the ovary in children and adolescents. Multicenter retrospective study in 40 patients aged 7 months to 22 years].

BACKGROUND: Juvenile granulosa cell tumors (JGCT) of the ovary are rare in children. The over-all outcome after surgery is relatively good, but the indication and type of complementary treatment for severe forms are still unclear. POPULATION: A retrospective survey of the majority of patients with JGCT of the ovary admitted between 1965 and 1990 to 11 French oncologic pediatric centers was carried out. Medical records including surgical and histological data, were analyzed and each tumor was retrospectively classified by the same pathologist according to the Wollner classification. RESULTS: There were 40 patients aged 7 months to 22 years (mean: 6 years); 28 were less than 10 year old at diagnosis. Three had enchondromatosis (Ollier's disease). At diagnosis, all patients presented with an abdominal tumor, 23 had developed manifestations of precocious pseudopuberty, 2 had signs of virilization after a normal puberty and 2 had secondary amenorrhea. Surgery was always the primary treatment: unilateral ovariectomy in 35 cases, bilateral in 4 and biopsy alone in 1 case. There were 21 stage I, 1 stage II, 16 stage III and 2 stage IV cases. 13 patients received combined chemotherapy and 2 abdominal radiotherapy. 34 patients were alive and disease-free 10 months to 26 years after surgery and 6 died. All 23 patients with precocious pseudopuberty had a favorable outcome. CONCLUSIONS: This study confirms earlier reports. Unilateral ovariectomy is the first-choice therapy. There is no evidence that tumors complicated by rupture and hemoperitoneum require chemotherapy. Combined chemotherapy does not appear to improve the prognosis for the rare malignant forms. The factors of good prognosis are age less than 10 years and the presence of precocious pseudopuberty.

Adolescent↗
PubMed1992

[Fibromatosis and fibrodysplasia ossificans progressiva. An avoidable diagnostic error].

BACKGROUND: Myositis ossificans progressiva is a rare progressive disease of connective tissue and muscle whose early diagnosis, before the lesions become ossified, can be difficult. The congenital malformations that accompany the disease may help in this diagnosis. CASE REPORT: A 12 year-old boy developed a localized swelling in the right trapezius muscle. Muscle biopsy showed fibromatosis lesions. Surgical excision was incomplete and despite chemotherapy, new lesions appeared periodically in the cervical and dorsal regions where they became ossified. Bilateral hallux valgus was noticed at that time and used to rectify the diagnosis as myositis ossificans progressiva. CONCLUSION: Many children suffering from myositis ossificans progressiva have congenital malformations, most commonly of big toes and thumbs. These anomalies are important for distinguishing myositis ossificans progressiva from other inflammatory diseases of muscle.

Child↗
PubMed1992

[Primary germinal tumors of the nervous system in children and adolescents. A retrospective study of 35 cases from 1975 to 1987].

Thirty-five cases of primary intracranial germ cell tumor treated at the Institut Gustave-Roussy between 1975 and 1987 were reviewed. There were 23 males and 12 females; the median age was 12 years (range: 3 months to 17 years). Tumors originated in the pineal region in 15 cases, in the suprasellar region in 15 cases, and in both region in 5 cases. The diagnosis was confirmed histologically in 20 cases (14 germinomas and 6 non germinomatous germ cell tumors), and cytologically in 5 cases. There were 11 secreting tumors (alpha-foetoprotein or chorionic gonadotrophin) without histological diagnosis in 6 cases. Four patients were treated with a presumptive diagnosis. Surgical resection was performed in 6 cases. Thirty-two patients were irradiated on the tumor site; 17 received a craniospinal irradiation and 7 a cranial irradiation. Six patients received chemotherapy. Twenty-five patients are alive with a median follow-up of 4 years. Prognosis is better in germinomas than in non germinomatous germ cell tumors (survival rates = 88 and 50% respectively). Chemotherapy is presently being evaluated in the treatment of germ cell tumors with the aim of using lower doses of radiotherapy and decreasing its late effects in germinomas and of improving the prognosis of other histological types.

Adolescent↗
PubMed1992

[Neonatal malignant tumors. Apropos of 75 cases].

From 1975 to 1986, 75 neonates were treated at the Institute Gustave-Roussy for a malignant solid tumor, comprising 1.7% of the overall oncopediatric population treated during the same period of time. Of these 75 patients, 47 (62%) presented with neuroblastomas, 15 (20%) with mesenchymal tumors, 9 (12%) with germ cell tumors and 4 with various other types of tumors. Treatment consisted of surgery in 63 patients, chemotherapy in 43 and radiotherapy in 18. The 5 year crude survival rate was 77%. Neuroblastomas did the best with a crude survival rate of 89%. The young age of the patients resulted in two types of problems: immediate tolerance and long term sequelae. The therapeutic regimen should take into account the good prognosis of the majority of these tumors in order to decrease delayed effects.

Abdominal Neoplasms↗
PubMed1991

Prognostic factors of alveolar rhabdomyosarcoma in childhood. An International Society of Pediatric Oncology study.

Pretreatment characteristics of 57 patients with alveolar rhabdomyosarcoma (RMS) registered by the International Society of Pediatric Oncology (SIOP) between January 1975 and December 1983 were identified to perform a prognostic factor study. Living status and disease-free survival (DFS) time were assessed for all patients on January 1, 1988. By multi-variate analyses, lymph node involvement (P = 0.0003) and tumor invasiveness (P = 0.007) were identified as the most significant covariates correlated with survival. A model including N stage (P = 0.001) and age (P = 0.03) was selected for predicting DFS rates. Children between 5 and 10 years of age had better DFS rates than those younger than 5 years of age or those older than 10 years of age. The authors suggest a prognostic classification that could allow treatment to be adjusted according to clinical staging.

Adolescent↗
PubMed1991

Prognostic factors in 951 nonmetastatic rhabdomyosarcoma in children: a report from the International Rhabdomyosarcoma Workshop.

A total of 951 newly diagnosed patients with nonmetastatic rhabdomyosarcoma (RMS) under 21 years of age from four different cooperative study groups were analyzed to identify the most important pretreatment tumor characteristics in predicting survival. The patient characteristics considered were tumor invasiveness (T), tumor size, status of locoregional lymph nodes, and primary site. The cooperative groups and studies analyzed were: SIOP [RMS-75 (1975-1984)], the Intergroup Rhabdomyosarcoma Study [IRS-II (1978-1982)], Federal Republic of Germany [CWS-81 (1981-1986)] and Italy [RMS-79 (1979-1986), each study involving a different therapeutic protocol. Patient characteristics identified as having a significant relationship to survival by univariate analysis were (favorable features given in brackets): tumor invasiveness [T1], tumor size [less than or equal to 5 cm], status of locoregional lymph nodes [clinically negative], and primary site [orbit and genitourinary non-bladder prostate (GU-non-BP) sites]. T2 tumors tended to have large size (greater than 5 cm), to be associated with positive nodes, and to have a different distribution by primary site than T1 tumors. A multivariate Cox regression analysis of the pooled data identified tumor invasiveness (T), primary site, and the interaction between T and primary site as significant predictive factors for survival. The prognosis of orbital tumors was consistently favorable regardless of T status, whereas the prognosis for "other sites" was consistently unfavorable. International cooperation in this study has permitted the identification of good and poor risk patient subgroups, permitting the focusing of therapeutic protocols to particular risk groups and setting the stage for common future therapeutic protocols for rare subgroups of patients.

Child↗
PubMed1991

Rhabdomyosarcoma of the larynx in children: a series of five patients treated in the Institut Gustave Roussy (Villejuif, France).

Rhabdomyosarcoma of the larynx is a malignant disease rarely seen in children. We present five cases (3 males, 2 females) observed among 126 patients with rhabdomyosarcoma of the head and neck (excluding the orbit) treated in the Institut Gustave-Roussy between 1955 and 1981. The patients were aged between 5 1/2 years and 13 1/2 years at the time of diagnosis. The presenting symptoms were dysphonia and/or dyspnea; tracheotomy was required in two cases. All five patients received chemotherapy consisting of vincristine, cyclophosphamide, actinomycin D + procarbazine or doxorubicin. Four patients also received radiotherapy (45 Gy during 5 weeks). Laryngectomy was not performed. Treatment sequelae included regional hypoplasia, huskiness, and subclinical thyroid insufficiency; two patients developed a benign thyroid adenoma. One patient presented a bilateral carotid stenosis subsequent to radiotherapy performed on "wide fields" portal designed to provided prophylactic treatment on uninvolved lymph nodes. All the patients are alive and relapse-free with a follow-up of between 13 and 17 years. This small series of RMS of the larynx shows that patients can be cured without laryngectomy, although long-term sequelae associated with radiotherapy are observed.

Adolescent↗
PubMed1991

Pancreatoblastoma: response to chemotherapy.

Two cases of pancreatoblastoma in children are reported here. Only biopsies were made at laparotomy as surgical resection by duodeno-pancreatectomy was not possible. In both children a dramatic response was observed with chemotherapy: doxorubicin plus cisplatin for one, cyclophosphamide, actinomycin D, bleomycin, vinblastine sulfate, and cisplatin for the other. After completion of the chemotherapy the first patient had a local resection; then he had radiotherapy. He is alive in first remission 40 months after the end of the treatment. In the second patient, regional recurrence occurred 8 months after chemotherapy was ended. A transient second remission was obtained with ifosfamide plus etoposide alternating with epirubicin plus vincristine. The patient died 36 months after the diagnosis. Therefore, these two cases suggest that chemotherapy may be proposed before any attempt at surgical excision. Nevertheless, early consolidation by radical resection or irradiation must be considered.

Adenocarcinoma↗
PubMed1991

Twenty years experience of interstitial iridium brachytherapy in the management of soft tissue sarcomas.

From February 1968 to February 1988, 50 patients above 10 years of age with a soft tissue sarcoma were treated with interstitial brachytherapy, combined with a wide excision. After pathologic review, 48 were included in the final analysis. A pathological grading was made possible in 41, which showed a majority of high grades (2 + 3 = 86%). Patients presented mainly with small (less than 5 cm: 36) or mid-size lesions (greater than 5 cm: 12). The tumor was located in the limbs (32), trunk (9), and head and neck (7). Four patients had metastases at the time of treatment. Brachytherapy was part of the initial treatment in 22 cases, and of a salvage procedure after previous excision(s) combined or not with another form of treatment in 26. A uniform technique of iridium 192 wires after-loaded in plastic tubing was used. Sixty Gy median doses were delivered with brachytherapy alone (44) or combined with external beam (4). Sixteen patients also received an adjuvant chemotherapy. Follow up ranged from 16 months to 20 years (median 82 months). At the time of analysis, two patients (4%) only had failed in the irradiated volume, but the marginal failures rate (14:31%) was unexpectedly high. Seven of the patients who failed (43%) were salvaged by a second similar procedure. The 5-year survival was 62% in non-previously treated patients and 56.5% in previously treated ones (pNS). By multivariate analysis, only the tumor location appeared predictive of LF (p less than 0.01), which in turn was strongly correlated with the metastatic outcome (p less than 0.01). Necroses were observed in 17 cases (35%) and associated with a benign course in most of them. High dose brachytherapy combined with conservative surgery is highly effective in small and mid-size soft tissue sarcomas located in the extremities and head and neck, whereas in trunk and in recurrent tumors, the adjunction of large fields external radiotherapy and/or possibly polychemotherapy appears necessary.

Adolescent↗
PubMed1991

Long-term follow-up of ifosfamide renal toxicity in children treated for malignant mesenchymal tumors: an International Society of Pediatric Oncology report.

The renal function of 74 children with malignant mesenchymal tumors in complete remission and who have received the same ifosfamide chemotherapy protocol (International Society of Pediatric Oncology Malignant Mesenchymal Tumor Study 84 [SIOP MMT 84]) were studied 1 year after the completion of treatment. Total cumulative doses were 36 or 60 g/m2 of ifosfamide (six or 10 cycles of ifosfamide, vincristine, and dactinomycin [IVA]). None of them had received cisplatin chemotherapy. Ages ranged from 4 months to 17 years; 58 patients were males and 42 females. The most common primary tumor site was the head and neck. Renal function was investigated by measuring plasma and urinary electrolytes, glucosuria, proteinuria, aminoaciduria, urinary pH, osmolarity, creatinine clearance, phosphate tubular reabsorption, beta 2 microglobulinuria, and lysozymuria. Fifty-eight patients (78%) had normal renal tests, whereas 16 patients (22%) had renal abnormalities. Two subsets of patients were identified from this latter group: the first included four patients (5% of the total population) who developed major toxicity resulting in Fanconi's syndrome (TDFS); and the second group included five patients with elevated beta 2 microglobulinuria and low phosphate reabsorption. The remaining seven patients had isolated beta 2 microglobulinuria. Severe toxicity was correlated with the higher cumulative dose of 60 g/m2 of ifosfamide, a younger age (less than 2 1/2 years old), and a predominance of vesicoprostatic tumor involvement. This low percentage (5%) of TDFS must be evaluated with respect to the efficacy of ifosfamide in the treatment of mesenchymal tumors in children.

Acute Kidney Injury↗
PubMed1990

Diagnostic and therapeutic problems of soft tissue tumors other than rhabdomyosarcoma in infants under 1 year of age: a clinicopathological study of 34 cases treated at the Institut Gustave-Roussy.

Thirty-four cases of soft tissue tumors (STT) other than rhabdomyosarcoma in infants under 1 year of age were seen in our institution between 1955 and 1985. All were diagnosed initially as malignant tumors except for three cases of fibromatosis, and, thus, they received therapy appropriate at that time. During a recent pathologic review, four were seen to have had hemangioma, six hemangiopericytoma, one hamartoma, seven fibromatosis, eight fibrosarcoma, and eight unclassified sarcomas. Of these 34 cases, the initial histological diagnosis was confirmed in only 17. Sixteen patients were believed to have received inappropriately aggressive therapy; indeed, four of these cases diagnosed prior to 1975 had been benign. Major long-term side effects were seen in 7/16 treated patients (six radiotherapy, one surgery), who, in retrospect, should have received less aggressive treatment. The reasons for these initial errors in diagnosis included doubtful histologic features associated in some cases with alarming clinical presentation. We believe that difficulties still remain in distinguishing benign from malignant STT in this age group by histological examination. Therefore, we urge extreme caution in using aggressive therapy before the diagnosis is certain.

Age Factors↗
PubMed1990

Poor prognosis infantile fibrosarcoma with pathologic features of malignant fibrous histiocytoma after local recurrence.

In a retrospective study of infants under 1 year of age treated at our institution over a 30-year period for soft tissue tumors, eight fibrosarcomas (FS) were seen, six of which were congenital. Therapy consisted of local excision (n = 3), radiotherapy (n = 1), surgery + radiotherapy (n = 1), surgery + chemotherapy (n = 1), and surgery + chemotherapy + radiotherapy (n = 2). Among these eight patients, four are alive in first complete remission (CR) with 13, 17, 23, and 27 years of follow-up. Of the remaining four patients, two had local recurrences and are still alive in CR after re-excision of the tumor, while the other two had both local and distant relapses and died. Interestingly, in the two patients who developed distant metastases, the pathologic pattern was that of malignant fibrous histiocytoma (MFH) at the time of local recurrence. To our knowledge, no similar cases of transitions between infantile FS known for its favorable outcome and MFH have been reported in this age group. The relevance of such transitions is difficult to assess. However, given the known metastatic potential of MFH, we believe that chemotherapy regimens currently used in the management of childhood soft tissue sarcomas should be used in similar cases.

Child, Preschool↗
PubMed1990

ApoVLDLII gene transcription in immature cockerels without estradiol stimulation.

1. Previous studies have led to the conclusion that the gene encoding the apo very low density lipoprotein II (ApoVLDLII) is under full estrogen control. However, by using a sensitive hybridization technique we found a weak expression of this gene in some males in the absence of hormone. 2. As the vitellogenin II gene, which is also estrogen dependent, remains inactive in these animals it is likely that they carry a deregulated allele of the ApoVLDLII gene. 3. The highest level of ApoVLDLII mRNA is found in genetically fat males suggesting a possible involvement of the ApoVLDLII gene in fatness determinism.

Animals↗